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CHAPTER 112: Sudden Infant Death Syndrome and Apparent Life-Threatening Event
TABLE 111-8 Signs and Symptoms of Neonatal Sepsis
Temperature instability
Central nervous system dysfunction
Respiratory distress
Feeding disturbance
Jaundice
Rashes
Fever, hypothermia
Lethargy, irritability, seizures
Apnea, tachypnea, grunting
Vomiting, poor feeding, gastric distention, diarrhea
The bacterial causes of neonatal sepsis reflect the organisms that colonize the female genital tract and nasal mucosa of caregivers. In general,
the two groups of pathogens most frequently encountered are grampositive cocci, such as β-hemolytic streptococci, and enteric organisms,
such as Escherichia coli and Klebsiella species, and Haemophilus influenzae. Listeria monocytogenes also causes sepsis and meningitis in neonates. Viral infections comprise another common cause of fever and are
most likely due to enteroviruses (coxsackievirus and echovirus) acquired
at the time of delivery, or respiratory syncytial virus and influenza A virus acquired postnatally.
The clinical investigation for neonatal sepsis is similar to that of an
older infant except that the threshold for a full sepsis workup, including
CSF analyses, is lower. All neonates should be admitted and treated with
empiric IV antibiotics. Initial treatment of a neonate with suspected
bacterial septicemia or meningitis usually includes ampicillin (50
milligrams/kg to cover group B Streptococcus and Listeria) and an
aminoglycoside (gentamicin, 2.5 milligrams/kg, to cover E. coli and
other gram-negative organisms). When gram-negative meningitis is
strongly suspected, gentamicin is usually replaced with cefotaxime or
ceftazidime, which have better central nervous system penetration.
An infant with a maternal history of herpes or suspicious CSF findings
(predominance of lymphocytes and erythrocytes in a nontraumatic lumbar puncture) and all infants who are ill appearing should also receive IV
acyclovir.12
NEUROLOGIC COMPLAINTS
■ ABNORMAL MOVEMENTS AND SEIZURES
Seizures are covered in detail in Chapter 129, Seizures and Status Epilepticus in Children. It is important to distinguish benign sleep myoclonus
in infancy and the normal startle reflex from actual seizures. The former
consists of rhythmic myoclonic jerks observed when the infant is drowsy
or in quiet sleep and can be suppressed upon touching and or waking the
infant. Tetany due to hypocalcemia associated with congenital syndromes, such as DiGeorge, must also be distinguished from seizure activity. Recognition of seizures in the newborn period is important,
because their management and outcome are different than at any other
age. Newborns are more likely to present with subtle manifestations,
such as eye deviation, tongue thrusting, eyelid fluttering, apnea, pedaling
movements, or arching, rather than generalized activity. Seizures usually
indicate a severe underlying structural or metabolic problem and are
rarely idiopathic.
Acknowledgment: The authors gratefully acknowledge the contributions
of Tonia J. Brousseau, the lead author of this chapter in the previous edition.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
112
745
Sudden Infant Death
Syndrome and Apparent
Life-Threatening Event
Carol D. Berkowitz
Ilene Claudius
Joel S. Tieder
SUDDEN INFANT DEATH SYNDROME
Sudden infant death syndrome (SIDS) is the unexpected death of infants
<1 year old for which no pathologic cause can be determined by a thorough history, physical examination, postmortem examination, and environmental investigation. SIDS is a diagnosis of exclusion. The syndrome
has been a leading cause of death of infants between 1 month and 1 year
of age. In the past, between 5000 and 10,000 infants (1 to 2 per 1000 live
births) succumbed yearly to SIDS. With recent changes in infant sleep position, the number of deaths has decreased to about 3000, or 0.8 deaths
per 1000 infants. In addition, the recognition of other risk factors, such as
bed-sharing and parental smoking, have altered the way sudden unexpected deaths during infancy are categorized, and the number of SIDS
cases may be, in part, related to this change in diagnostic criteria.1 Another term that is applicable to these infants is sudden unexplained infant
death, which includes cases of SIDS.
■ PATHOPHYSIOLOGY
The first 6 months of life represents a unique critical period of vulnerability. Potential external stressors include prone sleeping, soft or adult
bedding, bed sharing, and minor infection, often with respiratory syncytial virus (RSV).
Autopsies of some SIDS victims have shown pathologic changes, including smooth muscle thickening in small pulmonary arteries, right ventricular hypertrophy, hematopoiesis in the liver, increase in periadrenal
brown fat, adrenal medullary hyperplasia, and abnormalities of the carotid body. Other markers reported with some regularity include brainstem
gliosis and increased neuronal apoptosis in the brainstem and hippocampus. These were thought to be indicative of long-standing hypoxemia and,
in the early 1990s, attention focused on an association between SIDS and
sleeping in the prone position.5,6 Epidemiologic studies indicated that the
incidence of SIDS was lower in countries where infants sleep supine or in
the side-down position, and that a reduction in the incidence of SIDS followed a reduction in prone sleeping.5,6 Two mechanisms linking SIDS to
prone sleeping are noted. With prone sleeping, infants will assume a facedown position and may experience apnea, particularly in response to a
cold stimulus on the face (diving reflex). In clinical trials, rather, prone infants were found to rebreathe expired air and experience hypercarbia.7 In
addition, infants normally dissipate heat through their head, and prone
sleeping may inhibit heat loss, thereby exacerbating hyperthermia, another noted risk factor for SIDS.8 Because of these observations related to the
prone position, the American Academy of Pediatrics has recommended a
supine sleeping position for normal infants since 1992.
■ EPIDEMIOLOGIC FACTORS
Victims range in age from 1 month to 1 year, with a peak incidence between 2 and 4 months of age. SIDS is rare in the first month of life, probably because neonates have a better anaerobic capacity for survival and
may be able to raise their PaO2 over 20 mm Hg with a gasp. SIDS-like
presentation in the first month of life should raise concern for an underlying condition such as congenital heart disease, nonaccidental
trauma, or metabolic disease. There are a disproportionate number of
SIDS deaths in lower socioeconomic groups, although this is true for
deaths in infancy from all causes. Mothers of SIDS victims are frequently
<20 years old, unwed, smoke, use drugs, and have made few prenatal and
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postpartum visits. Prenatal and postnatal maternal smoking increases
the incidence of SIDS. SIDS is more likely to occur during the winter
months and when the infant is asleep. Thirty percent to 50% of SIDS patients have some acute infection, usually of the upper respiratory tract, at
the time of the event. Infection with RSV has been associated with apnea,
particularly in premature infants and those with an antecedent history of
apnea. Otitis media and gastroenteritis also have been associated with
SIDS. Infected infants tend to be older than noninfected infants, and
males outnumber females in the infected group by 2:1. The sex ratio is
equal in the infants that are healthy at the time of the event.9
A disproportionate number of infants succumb to SIDS while with a
babysitter.10 Many of these infants are found in the prone position. For
some infants, this is the first time they have been placed in the prone position, and investigators have proposed that these infants have poor
strength and tone in their neck muscles. Several studies have reported an
increased incidence of co-sleeping or sleeping on an inappropriate surface among SIDS victims.11,12 In one study, <10% of infants who had
died from SIDS had been supine on an appropriate surface at the time of
death.12 Recent well-controlled trials suggest an increased risk of SIDS
when infants <12 weeks old bed-share with parents.13 The risk is further
increased with sleeping on a couch where the infant can become wedged
between cushions.14 Table 112-1 lists common risk factors recognized
for SIDS.
Familial cases of SIDS raise the possibility of abuse. The presence of traumatic head injury, bruises, long-bone fractures, rib fractures, internal hemorrhages, evidence of physical neglect, or blood around the nares suggests
abuse.15 A history inconsistent with the usual events surrounding a SIDS
death also may raise the suspicion of abuse. Some infants with abusive head
trauma may present with nonspecific symptoms, including apnea.16
■ CLINICAL FEATURES AND MANAGEMENT
Generally, SIDS victims present in one of two ways: not amenable to resuscitation or potentially responsive to resuscitation measures. Infants
with rigor mortis, livedo reticularis, pH <6, and a significantly reduced
core temperature in the absence of a history of environmental hypothermia should not be resuscitated. On the other hand, the warm infant
with apnea and no pulse may benefit from attempts at resuscitation.
Regardless of the presentation of SIDS, obtain a thorough history and
perform a complete physical examination. Important questions include:
complete description of the circumstances, caretakers, recent illness, prenatal and birth history, maternal and family history of miscarriages or
other infant deaths, and family history of metabolic disease. For epidemiologic information, documentation of sleep position, sleep location,
when and by whom the infant was last seen alive, when and in what position the infant was found, and whether or not bed-sharing was involved
is helpful to the coroner. Examination of the infant may be unrevealing or
may show subtle though relevant signs of trauma, such as facial bruising
or petechiae, raising suspicion of inflicted trauma. Physical data, including rectal temperature, blood in the nose or the mouth, presence of pete-
TABLE 112-1 Risk Factors for Sudden Infant Death Syndrome (SIDS)
Extrinsic Risk Factors
Intrinsic Risk Factors
Prone or side sleeping
Bedclothes over head
Sleeping on sofa or soft furniture
High ambient temperature
Soft bedding
Bed sharing
Postnatal smoke exposure
Prenatal smoke, alcohol, or drug exposure
Prematurity
Family history of SIDS
Male gender
Race
Native American
African American
Maori
Aboriginal Australian
Poverty
Reproduced with permission from Kinney HC, Thach BT: The sudden infant death syndrome. N Engl J Med 361: 795, 2009.
chiae on the face or conjunctiva, apparent injuries, and presence of rigor
mortis or lividity, will help the coroner in determining an approximate
time of death and determining the likelihood of suffocation or abuse.
The management of a nonresuscitative SIDS infant and the infant’s
family is challenging for the entire team. The emergency provider is confronted by a distraught caregiver who often has found the baby cold,
blue, and lifeless only hours after having fed the infant. Frequently, valiant, albeit unsuccessful, efforts are carried out in the ED, or the infant is
revived briefly, only to succumb after several hours in the intensive care
unit. The major responsibility of the physician is then to notify, counsel,
and educate the family. Frequently, the family wants to spend time with
the deceased infant. In general, the infant’s body should not be manipulated or photographed after death has been declared unless permission is granted by the coroner. If the family wants a hand or footprint,
inkless pads must be used, and this must be documented in the medical
record. Do not remove any lines or tubes placed during attempted resuscitation. If the presence of tubing is disconcerting to the family, tubes
may be cut at the skin to appear less obvious. Unless directed otherwise
by the coroner, the family should be allowed to hold the deceased infant
in a private setting that allows discrete monitoring of the family.
In most jurisdictions, victims of sudden and unexplained deaths must
be reported as soon as possible to the coroner’s office. As treating physician, complete the form reporting the death, but do not sign the official death certificate, as the cause of death will not be evident until
the coroner’s investigation is finished. If blood samples were drawn,
put the samples on hold in the laboratory for later access by the coroner.
Once death has been pronounced, the physician does not have jurisdiction to perform postmortem sampling or radiography unless directed to
do so by the coroner.
A home scene investigation is often conducted. Some jurisdictions
have infant death teams that fully evaluate the circumstances surrounding the unexpected death of young infants. If the physician believes the
infant is a victim of SIDS, the family should be so advised but told that
the final confirmation awaits the autopsy report. Involving the primary
care provider, who may follow up on the autopsy and remain in contact
with the family, is of paramount importance. The hospital chaplain or
social worker may provide additional support, and a chaplain consult
may be especially needed in cases in which the laws regarding autopsy
before burial are at odds with the family’s religious doctrine. For infants
requiring a perimortem baptism, this is ideally done by a chaplain but
can be done by a medical provider if no chaplain is available. Most communities have organizations for parents of SIDS victims, and information about these organizations can be obtained from First Candle (1800-221-SIDS, www.firstcandle.org). Parents also may be referred to
Web sites such as http://www.sidsfamilies.com. Some states also require
notification of organ procurement agencies.
APPARENT LIFE-THREATENING EVENT
Apparent life-threatening event (ALTE) is an episode that is frightening to a caregiver and involves some combination of apnea, color
change (cyanosis, pallor, or plethora), change in muscle tone (limp or
stiff), choking or gagging.17 The peak incidence is between 1 week and
2 months of age, with the majority of ALTEs occurring before 10 weeks
of age.18 The male to female ratio is 2:1.19 Known risk factors for ALTE
include RSV infection, prematurity, recent anesthesia, and known gastroesophageal reflux or airway/maxillofacial anomalies.
■ PATHOPHYSIOLOGY
ALTE is a symptom, not a diagnosis. The term ALTE is defined by subjective symptoms and requires some level of interpretation as presented by
nonmedical caregivers. For example, without a respiratory monitor, quantifying change in color or change in respiratory effort is challenging, even
for medical providers. Many of the symptoms of an ALTE may represent
normal physiologic phenomena of newborns, such as periodic breathing,
perioral cyanosis and acrocyanosis, and reflux, and ascertaining the temporal sequence of the constellation of symptoms is important.
CHAPTER 112: Sudden Infant Death Syndrome and Apparent Life-Threatening Event
Apnea is typically characterized as central, obstructive, or mixed. Apneic pauses of >20 seconds or those associated with changes in color,
tone, or heart rate are considered pathologic. Central apnea implies a
disruption in the central respiratory centers resulting in a cessation of respiratory effort. There is no attempt to breathe. Normal periodic breathing
is characterized by pauses of several seconds in respiration, between normal to rapid periods of breathing. Infants with obstructive apnea appear
to be attempting to breathe through an occluded airway, with paradoxical
movements of the chest and abdomen and a dip of 3% or greater in oxygen saturation.20 Many parents describe components of both central and
obstructive apnea, indicating a mixed picture. Apnea of prematurity is a
disorder in the control of breathing in premature infants, occurring in up
to 25% of this group.21 Its frequency decreases with increasing maturity,
and it is usually outgrown by 37 weeks’ postconceptual age but occasionally persists a few weeks past term.22 An ALTE occurring in a premature
infant who has reached 37 weeks’ postconceptual age and is without a
recent history of apnea of prematurity should be investigated with the
assumption that there is a new underlying etiology.
The diagnostic criteria for ALTE include changes that may be seen as
part of normal newborn behavior, benign conditions, or serious pathology, as well as with ALTE. Changes in infant skin color are often difficult
for caretakers to characterize and may represent true perioral or central
cyanosis, plethora, or pallor. Cyanosis becomes apparent when at least 5
grams/100 mL of blood is deoxygenated. Because young infants are often
polycythemic, this threshold is more easily met in this age group, and cyanosis may be observed in normal newborns, although it is typically perioral or distal in its distribution (acrocyanosis). Normal infant polycythemia
can also lead to a ruddy appearance (plethoric), which, in the crying infant,
may be misinterpreted by lay caregivers as cyanosis or “purple” coloring.
Pallor is characteristic of the vasovagal response and can be seen in association with gastroesophageal reflux disease (GERD).
In neonates, changes in muscle tone are difficult to describe, because
baseline neurologic status is variable due to immaturity. Seizures uncommonly present as stereotypical tonic-clonic activity and are more likely to
present with altered consciousness or intermittent high and low tone. In
addition, infants may exhibit changes in tone (either decreased or increased) in the postictal state that may be observed by caretakers and lead
to concern for ALTE. Changes in tone may also be secondary to hypoxia
resulting from apnea. Stiffening and arching behavior have been well described in infants with severe GERD (Sandifer syndrome). Choking, gagging, and coughing are also common symptoms in infants and may be
due to gastroesophageal reflux, overfeeding, or incoordination of the normal suck-swallow-breath sequence that can be exacerbated by congestion
from an upper respiratory infection (URI). Rarely, congenital anatomic
malformations, such as tracheoesophageal fistula, may lead to choking,
gagging, or coughing with feeds.
Some respiratory infections, such as pertussis and RSV, may be associated with a number of observed changes that are part of the ALTE diagnostic criteria: coughing (which may be staccato in pertussis), gagging
(from thick mucus secretions in RSV), color changes (including true cyanosis), and stiffening or loss of tone are well described. Apnea has also
been described in the setting of both pertussis and RSV, and may occur
in the absence of significant congestion, nasal discharge, cough, or work
of breathing, particularly in neonates.
■ DIFFERENTIAL DIAGNOSIS
Table 112-2 lists common, uncommon, and rare diagnoses assigned to
patients presenting with ALTE. More common processes are discussed
independently below.
Gastroesophageal Reflux Disease As a final diagnosis, GERD is among
the most common and the most controversial of the potential sources for
ALTE. GERD is the involuntary passage of gastric contents into the esophagus, and it occurs daily in children during the first year of life. This form
of reflux, commonly characterized by frequent “spitting up,” is entirely
normal and should be considered physiologic. Pathologic GERD, however, is defined as regurgitation of gastric contents into the esophagus with
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TABLE 112-2 Reported Final Diagnoses for Patients Presenting
with Apparent Life-Threatening Event
Common Diagnoses
Seizure/febrile seizure
Gastroesophageal reflux
Respiratory tract infection
(upper or lower tract)
Misinterpretation of benign
process such as periodic
breathing
Vomiting/choking episode
Less Common
Diagnoses
Rare Reported
Diagnoses
Pertussis
Inflicted injury
Poisoning
Serious bacterial
infection
Arrhythmia or other
cardiac process
Electrolyte abnormality
(including glucose)
Anemia
Breath-holding spell
Metabolic disease
Anatomic maxillofacial
obstruction
accompanying symptoms and complications. It is recommended that
GERD be diagnosed with a pH probe or treated empirically if indicated.
Given the temporal correlation between peak age for ALTE and that of
GERD, and the fact that reflux of gastric contents into the hypopharynx
can trigger laryngospasm, a diagnosis of GERD provides an easy explanation for an ALTE. However, researchers have been unable to demonstrate
a temporal relationship between episodes of GERD on pH probe and
ALTEs or apneic events,26,27 and the literature on the topic is mixed. Some
studies have shown a decrease in repeated brief apneas after initiating appropriate medications in patients with GERD.28 Other authors have provided evidence that reflux may be either unrelated,29 secondary to apnea,26
or even protective in stimulating a patient out of succumbing to an apneic
episode.30
Bronchiolitis A number of studies have reported an increased risk of
both central and obstructive apnea during respiratory infections in infants, especially RSV bronchiolitis. Although the exact mechanism is unclear, dysregulation of mucosal immune responses and sensorineural
stimulation have been postulated.31,32 One polygraphic study demonstrated bronchiolitis-related episodes of central apnea in infants during
quiet sleep.33 Obstructive apnea may also occur when infants choke on
secretions from the disease. Diagnosis can be difficult, as apnea may be
the first presenting symptom of bronchiolitis. Apnea on presentation
is a risk factor for recurrent apnea with bronchiolitis, as is younger age,
lower temperature, higher PCO2, or radiographic signs of atelectasis.34,35
Most studies have been performed on clinical or RSV-proven bronchiolitis, but recently the same association was found with Metapneumovirus-associated bronchiolitis.36 A recent paper by Wilwerth et al. found a
2.7% rate of apnea among infants admitted with bronchiolitis. All patients
with apnea in this study were <1 month of age if full-term or 48 weeks’
postconceptual age if premature, or had a history of apnea upon presentation to the ED.35 Use of over-the-counter cough and cold medications
may contribute to the increased risk of apnea during a respiratory illness,
and these medications have been withdrawn from the market.
Seizures Seizures have been identified in 4% to 7% of infants with ALTE,
and may be obvious or subtle when associated with apnea. In younger
infants, apnea may be the sole manifestation of a seizure.37 Interictal
electroencephalograms may be normal, making the diagnosis challenging. One study showed the potential for severe hypoxemia during infantile seizures.38 Rarely, seizures are secondary to underlying causes such
as congenital brain malformation, metabolic disorders, electrolyte abnormalities, perinatally acquired brain injury, or intracranial bleeding (including nonaccidental trauma), and these possibilities must be considered.
One study of infants presenting with an ALTE caused by a seizure found
that 11% were eventually diagnosed as child abuse, 3.6% developed chronic epilepsy, and 3% developed developmental delay. This paper concluded
that, although an inpatient workup is not mandatory, close follow-up must
be ensured.39
Nonaccidental Trauma/Poisoning Child abuse is reported in 2.3% to 2.5%
of infants presenting with ALTE.40,41 Suffocation, inflicted head injury,
and poisoning are among the most concerning possible causes of an
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SECTION 12: Pediatrics
ALTE. Truman reported a high risk of future death or recurrent ALTEs
among children presenting with fresh blood from the nose/mouth, and an
increased risk of moderate to high suspicion for nonaccidental trauma in
infants >6 months of age with ALTE.42 Southall performed covert video
surveillance in a highly selective population of recurrent ALTE patients
considered suspicious for abuse, which confirmed abusive behavior in 33
of 39 cases. Interestingly, 29% of the siblings of this group had died unexpectedly. They also found an association between intentional suffocation
and bleeding from the nose and mouth, as well as high rates of marital dissatisfaction and personality disorders among the parents. Several of the reported cases suggested a type of Munchausen by proxy directed toward a
perceived inattentive coparent.43 On dilated retinal examination, a separate study noted retinal hemorrhages in 1.4% of ALTE patients presenting
to a single center.40 Higher numbers have been reported in patients undergoing inpatient workups after an ALTE.44 Poisoning as a cause of inflicted
ALTE is also a concern, and may be intentional or unintentional. Intentional poisonings frequently involve narcotics, benzodiazepines, or phenothiazines in an attempt to quiet or sedate a fussy infant.45 Unintentional
poisonings may involve inappropriate dosing of medications, or mixing of
over-the-counter cough and cold preparations containing ingredients with
similar activity. A recent study of 596 children with ALTE reported clinically significant true positives on the toxicology screen in 8.4%, 4.7% of
whom were positive for an over-the-counter cough and cold preparation.46
Pertussis Bordetella pertussis, or “whooping cough,” causes a respiratory
infection that persists in spite of vaccination due to waning immunity in
older individuals, vaccine failures, and vaccine refusal. Infants <6 months
old are particularly susceptible because the initial immunization series begins at 2 months old and is only partially effective, particularly until the series is completed at 6 months old. Classically, the disease starts with URI
symptoms (catarrhal phase) and progresses to paroxysmal coughing (paroxysmal phase) over 3 to 6 weeks. However, the classic presentation is often absent or blunted in infants, in whom the disease may present with
isolated apnea. In children <2 years of age, apnea occurs in 0.5% to 12.0%
of cases47,48 and is most common in those <3 months of age. Complications of pertussis include respiratory failure, pneumonia, airway obstruction, seizures, encephalitis, and apnea. Infants <12 months old have the
highest complication rates, and most are infants <2 months old.49
Serious Bacterial Infections Serious bacterial infections (SBI) must be
considered in all febrile infants with an ALTE (see Chapter 113, Fever
and Serious Bacterial Illness). Reported rates range from 0% to 8.2%, and
even in the infant presenting with an afebrile ALTE, the risk of bacteremia, meningitis, or urinary tract infection should be considered.
Concern is greatest in the infant <60 days old, who may manifest few
other symptoms to indicate the possibility of SBI. A study of 112 infants
<60 days old with ALTE who underwent testing for SBI identified three
cases of bacteremia and one urinary tract infection, as well as one case of
pertussis. This constituted 2.7% of the sample, and prematurity and hypothermia were found to confer additional risk.50
Breath-Holding Spells Breath-holding spells occur in 4% to 5% of children <8 years of age, and entail a cessation of respiration at the end of expiration, usually in response to pain, anger, or fear. Spells typically last <1
minute and may be accompanied by cyanosis, pallor, syncope, and seizures. Cyanotic breath-holding spells have not been associated with any
underlying medical condition; there is some literature supporting the
finding that pallid spells are associated with pronounced QT dispersion
and may have a cardiac etiology. These are generally easily recognized in
older children, although they may be diagnosed in infants as young as 6
months of age: one study found that in 15% of children with breath-holding spells, the age of onset was <6 months.51 Although breath-holding
should be a diagnosis of exclusion in younger patients, it is likely that
some ALTEs are early manifestations of breath-holding spells.
■ APPROACH TO THE APPARENT LIFE-THREATENING
EVENT PATIENT IN THE ED
Typically, ALTE patients can easily be categorized into one of three discrete
groups. The first group consists of those for whom a proximate cause for the
ALTE is clear from the history or physical examination. Fever in a neonate
with signs suggestive of sepsis, a cough classic for pertussis or bronchiolitis,
or a seizure witnessed by a member of the health care team and confirmed
as similar to the presenting complaint by the caretaker are examples of this
type of patient. The second group comprises infants for whom the diagnosis
is not immediately clear, but who appear unstable. The third and largest
group consists of well-appearing infants with a concerning history, but a
physical examination that is either normal or noncontributory.
Stable Patients with a Clear Diagnosis Manage stable infants with a
clear diagnosis according to the identified disease, but only if the diagnosis is objectively confirmed (e.g., positive RSV testing in the patient with clinical bronchiolitis), and modify disease treatment because
apnea confers clear risks to good patient outcome. For example, an episode of apnea is a risk factor for further apneic events in bronchiolitis.
Even a single episode of apnea in an infant with pertussis is concerning,
and hospitalization must be considered. In general, the discharge disposition should be dictated by the physician’s concern that a life-threatening event will recur, including the duration and severity of the ALTE, the
resuscitation required, single versus multiple events, the severity of other
symptoms, and the follow-up available to the child.
Unstable Patients without a Clear Diagnosis For unstable patients without a clear diagnosis, the priority is stabilization, which may require assisted ventilation for the infant with persistent ventilatory compromise, or for
an infant with frequent apnea requiring monitoring and stimulation in the
TABLE 112-3 Important Historical Questions in the Apparent
Life-Threatening Event (ALTE) Patient
Prematurity (before 37 wk)
Past
medical
Prior hospitalization, surgery, ED visits
history
History of prior apnea
Prior respiratory difficulties (snoring, stridor)
Prior feeding difficulties (choking, gagging, coughing with feeds)
Immunization status (pertussis)
Prior history of urinary tract infection
Family
History of sudden infant death syndrome or sudden death
history
Cardiac arrhythmias or congenital heart disease
Seizure disorder
Metabolic disease
Event
Duration of event
history
Resuscitation required (e.g., stimulation, mouth-to-mouth, chest
compressions)
Temporal relationship with feeding, sleep, crying, vomiting, choking,
gagging
Color (cyanosis, pallor)
Change in tone (including seizure activity)
Central vs. obstructive pattern of apnea (i.e., apparent respiratory effort)
Number of ALTEs experienced within 24 h of presentation
Episodic vs. sustained change in mental status (syncope, postictal
phase, irritability, obtundation)
Review of Respiratory symptoms or other intercurrent illness
systems
Period of fasting (e.g., recent onset of sleeping through night)
Medication use, medications in the home or used by breastfeeding
parent
Possible trauma
Social
Possibility of follow-up
history
Comfort level of parents
Parental concern for abuse
Parental psychiatric issues or marital stress (e.g., absentee parent)
Infectious exposure (pertussis, respiratory syncytial virus, upper respiratory infection, lower respiratory tract infection)
CHAPTER 112: Sudden Infant Death Syndrome and Apparent Life-Threatening Event
ED. In such a situation, head injury, sepsis, metabolic or electrolyte disorder, poisoning, pertussis with complications, and bronchiolitis (in the neonate or ex-preemie) are the most likely possibilities. The disposition of
this group is clearly hospitalization, and persistently unstable infants may
require an intensive care setting.
Stable Patients without a Clear Diagnosis Stable patients lacking a clear
diagnosis represent the largest group of ALTE patients and the greatest
diagnostic conundrum. This group includes infants who, by history,
have had an ALTE but appear well in the ED. They may have URI symptoms or nonspecific examination findings, but nothing to render them
unstable or suggest an obvious diagnosis. Specific information that
should be obtained in the history is outlined in Table 112-3.
Several experts have offered algorithms, but there is not a clear, evidence-based pathway that works for all patients. A potential algorithm
suggested by one expert in this field is listed in Figure 112-1.20 The following are considerations for testing, disposition, and follow-up.
Infectious Workup Many providers will perform a full “rule out sepsis” or
SBI evaluation for the afebrile infant <2 months old presenting with
ALTE (complete blood count, urinalysis and cultures of blood, urine,
and cerebrospinal fluid). Multiple single center studies,50,52,53 with vari-
749
ability in case definitions and patient populations, reported sufficiently
conflicted outcomes, making it difficult to calculate the diagnostic yield
of routine testing for SBI in patients with ALTE. Clearly, toxic or illappearing infants and those with localizing symptoms and signs of infection should receive appropriate SBI testing. Urine should be a consideration in all ALTE patients, even in the absence of a fever, unless an
alternative diagnosis is likely. Chest radiograph should be similarly contemplated, although in absence of any respiratory findings, false positives
can be common.
Other Laboratory Tests Obtain a careful history and perform a physical examination; further laboratory tests should be directed by specific signs and
symptoms in the well-appearing child with ALTE.55 Urinary toxicology
screens should be considered in all ALTE infants in whom another diagnosis is not strongly likely.
Further Testing GI reflux55 is a common diagnosis in all infants that may
or may not be the proximate cause of an ALTE. A careful history and
physical examination should indicate which patients require additional
evaluation, diagnostic imaging, or monitoring in the vast majority of
cases. Maintain a high index of suspicion for an occult condition in
children <2 months old.
Thorough history from primary
witness and physical exam
Was the event consistent with obstructive symptoms?
No
Associated with normal color, tone, and mental status?
Yes
Yes
Characteristic of choking on regurgitated milk,
mucus from an URI, or breath holding spell?
No
Yes
No
Is the exam focal?
No
Yes
Is the exam (including the tone) normal?
Have you observed an organized and reassuring feed?
No
Consider a screening protocol of blood gas, CBC,
electrolytes, glucose, EKG, and
fundoscopic exam or head CT
No
Admit for observation periods and initiate focused work-up and
treatment plan based on presumptive diagnosis
Yes
Do you and the family feel reassured that event is
unlikely to recur and/or consistent with a generally
benign process?
Yes
Are there any features that could
be c/w shaken baby?
Yes
Obtain head CT.
Positive?
Obtain head CT.
Positive?
Yes Yes
No
Treat
Involve CPS
No
Yes
Are there any features that
could be c/w shaken baby?
No
No
The diagnosis is consistent with a persistent
unstable airway or abnormal respiratory control?
No
Advise patient and treat
as indicated
Review stimulation techniques,
CPR techniques, and SIDS
risk factors
No
Yes
Patient required resuscitation or
parents unusually concerned?
Yes
Discuss with the family the option of a home monitor
Ensure follow-up
FIGURE 112-1. Algorithm for the approach to the apparent life-threatening event patient. c/w = consistent with; CBC = complete blood count; CPS = child protective services; SIDS, sudden infant death syndrome; URI = upper respiratory infection. (Reproduced with permission from DeWolfe CC: Apparent life-threatening event: a review.
Pediatr Clin North Am 52: 1127, 2005.)
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SECTION 12: Pediatrics
TABLE 112-4 Reasons for Admission for Apparent
Life-Threatening Event (ALTE)
Consider admission
<48 wk postconceptual age (prematurity)
Ill appearing
Concerning findings on physical examination
Bronchiolitis or pertussis with apnea
Nonaccidental trauma suspected
More than one event in past 24 h or multiple ALTEs
Abnormalities in past medical history
Prolonged central apnea, >20 s
ALTE requiring resuscitation
Poor follow-up
Family history of sudden infant death syndrome
■ DISPOSITION AND FOLLOW-UP
Traditionally, 83% of ALTE patients have been admitted for inpatient
workup and monitoring.57 Several authors have attempted to identify
high- and low-risk subsets of infants based on the likelihood of a subsequent ALTE or adverse outcome.59–61 A conservative, but rational, approach is presented in Table 112-4.
Cough and cold preparations are contraindicated in infancy even
when it appears that an infant has choked on mucus. Reassure the family that the ALTE is not a precursor or related to SIDS. CPR instruction,
if feasible, may provide additional reassurance to caregivers. Finally,
communication with the primary care provider regarding the event and
recommendations for further evaluation and subspecialist referral
should be made. Routine use of empiric histamine-2 blockers or proton
pump inhibitors for gastroesophageal reflux is not recommended. If the
events persist or there is continued evidence of GERD, then referral to a
gastroenterologist may be indicated. Those with a family history of SIDS
or recurrent ALTE may need referral to a pulmonologist for a sleep evaluation or home monitoring. Infants presenting with seizure in the first
year of life should be admitted to the hospital for inpatient evaluation
and neurology consultation, although febrile seizures in infants >6
months of age can be safely discharged without further workup (see
Chapter 129, Seizures and Status Epilepticus in Children).
LONG-TERM OUTCOME
The greatest fear for the ED physician is to discharge home an infant who
subsequently succumbs to SIDS or occult illness. Although ALTE is not
considered a risk factor for SIDS, a very small portion of ALTE patients
may still be at risk. One small study found a subsequent mortality rate of
10% among patients requiring cardiopulmonary resuscitation for ALTE,
and a 28% rate in such infants after multiple ALTE episodes.62 Another
study reported a 1.9% death rate in infants with ALTE requiring CPR,
much higher than the population risk of 0.8%.63 SIDS victims on monitors
were found to have longer duration central apneic episodes before succumbing to a SIDS death.64 This has led to a common practice of recommending sleep studies or prolonged monitoring upon discharge for
infants with multiple ALTEs or those requiring significant CPR. Currently, monitors are only recommended for infants with one or more severe
ALTEs, symptomatic preterm infants, siblings of two or more SIDS victims, and infants with certain diseases such as central hypoventilation.
Acknowledgments: The authors would like to thank Denise Bertone,
RN, and James Ribe, MD, of the Los Angeles Coroner’s Office for their
assistance in preparing this chapter.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
113
Fever and Serious
Bacterial Illness
Vincent J. Wang
FEVER
The focus of this chapter is the management of a neonate, infant, or child
(Table 113-1) with acute fever who is at risk for serious bacterial illness
(SBI). SBI is bacterial infection in neonates, infants, and young children with high morbidity and mortality if not properly treated.
■ CLINICAL FEATURES
Any elevation in temperature above normal is considered a fever, but the
threshold for concerning fever varies with the age group and is related to
the ability of signs and symptoms to identify the underlying cause of fever. Acute fever is fever for <7 days. In the neonate or infant <2 to 3
months of age, the threshold for concerning fever is 38°C (100.4°F); in
infants and children 3 to 36 months old, the threshold is 39°C (102.2°F).
In children >36 months old, the definition of significant fever is not fixed
because most children >36 months old should demonstrate signs or
symptoms of the underlying cause. In children with developmental delay
or mental retardation, with limited ability to demonstrate specific signs
and symptoms, the cause of fever may be difficult to determine, and
more testing is often necessary.
Oral temperatures are generally 0.6°C (1°F) lower than rectal temperatures, and axillary temperatures are 0.6°C (1°F) lower than oral temperatures. Temperatures taken with infrared thermometers that scan the
tympanic membrane are of variable reliability and reproducibility.2 Body
temperature normally varies from morning to evening with the body’s
circadian rhythm. The degree of variation, which is greater in young
women and small children, is approximately 1.1°C (2°F).
■ TREATMENT
Fever is treated with acetaminophen or ibuprofen. The dosage of acetaminophen is 15 milligrams/kg/dose (maximum daily dose, 80 milligrams/kg) every 4 to 6 hours, up to five times/day. Acetaminophen can
be given PO or PR. The dosage of ibuprofen is 10 milligrams/kg/dose
(maximum daily dose, 40 milligrams/kg) every 6 to 8 hours.
SERIOUS BACTERIAL ILLNESS
Infants ≤3 months of age, and especially neonates, are relatively immunosuppressed. Neonates and young infants demonstrate decreased opsonin activity, decreased macrophage and neutrophil function, and bone
marrow exhaustion.3 Infants and children demonstrate a poor immunoglobulin G antibody response to encapsulated bacteria until 24
months of age. Immune development is a continuum and improves as
the child matures. Therefore, the age of the patient and the virulence of
the bacteria are considerations for the evaluation of fever in children and
the identification of SBI. The most common maniffestations of SBI in
children are discussed: urinary tract infection (UTI), bacteremia and
sepsis, pneumonia and sinusitis, and meningitis.
TABLE 113-1 Pediatric Age Definitions
Neonate
Birth to 28 d of age*
Infant
29 d to 1 y of age
Child
>1 y of age
*For preterm neonates, calculate age from calculated date for term birth, not actual preterm
birth date.
CHAPTER 113: Fever and Serious Bacterial Illness
■ URINARY TRACT INFECTION
Overall, the most common SBI is UTI with or without pyelonephritis (see
Chapter 126, Urinary Tract Infection in Infants and Children). Among
young children presenting to EDs with fever and no obvious source of infection, between 3% and 8% have UTI.4 The overall incidence of UTI is 5%
in children between 2 months and 2 years old.5 For children between 1 and
2 years of age, the incidence of UTI in girls is up to 8%, but in uncircumcised boys, it is 1.9%. Uncircumcised boys have a rate of UTI 5 to 20 times
greater than circumcised boys. The presence of fever ≥39°C (102.2°F)
and a urine suggestive of infection indicate renal parenchymal involvement, or pyelonephritis. After 2 years of age, UTI remains a frequent bacterial cause of fever in girls but is more commonly associated with urinary
symptoms.
Escherichia coli and other gram-negative rod bacteria are the most
common causative organisms, although gram-positive organisms comprise a significant minority in older boys and in children with underlying
medical conditions such as neurogenic bladder. UTIs may not produce
symptoms other than fever, so routinely obtain a urinalysis and culture
in the evaluation of the febrile neonate or infant without other source.
The ideally obtained urine specimen for a child in diapers has traditionally been by urethral catheterization or suprapubic aspiration. In children
with labial adhesions or phimosis, a bag collection specimen may be preferred as a screening test. However, if the urinalysis is positive, obtain a
urine specimen for culture by bladder catheterization or clean-catch midstream method before antibiotic therapy, as bag collection methods produce frequent false positive cultures from skin contamination.
The initial diagnosis of UTI is made with chemical strip testing or a
microscope urinalysis (see Table 126-3). Chemical testing detects leukocyte esterase or nitrites. A positive test for leukocyte esterase has a
sensitivity of 67% to 85% for UTI, whereas a positive test for nitrites has
a specificity of 95% to 99% for UTI. A positive test for leukocytes on microscope urinalysis testing is a urine white blood cell count (WBC) 5 to
10/high power field, and has a sensitivity of 51% to 91%. The identification of bacteria on Gram stain has a sensitivity and specificity of 80% to
97% and 87% to 99%, respectively. Where Gram stain or microscopy
testing is not readily available, the chemical test compares favorably
with microscopy testing.
Before beginning antibiotic treatment, obtain an appropriate urine sample for culture and susceptibility testing. Consider blood and cerebrospinal
fluid (CSF) testing in children suspected to have a UTI. Approximately 5%
to 10% of febrile infants with UTI will have bacteremia.6,7 UTIs can be associated with bacteremia in up to 30% of infants between 4 weeks and 8
weeks of age.8
One study reported that 13% (15 of 117) of infants <3 months of age
with a febrile UTI admitted to the hospital had a sterile pleocytosis of the
CSF thought to be due to systemic release of inflammatory mediators or
low bacterial virulence in the subarachnoid space.6,7,9,10 Less than 1% of
febrile infants with UTI will have a bacterial meningitis, but concomitant
infection of the urine and CSF has been reported.
■ BACTEREMIA AND SEPSIS
Most studies of febrile infants ≤3 months old cite a bacteremia/sepsis
incidence of 2% to 3%. The most common causes of bacteremia and
meningitis in this age group are E. coli, Group B Streptococcus, and Listeria monocytogenes. Ill-appearing neonates or those identified at high
risk because of laboratory testing have an incidence of SBI of 13% to
21%.11 Overall, however, viral infections are the most frequent cause of
fever in infants ≤3 months old.
Before the widespread use of the pneumococcal conjugate vaccine, in
febrile infants and children between 3 and 36 months old, high fever,
WBC >15,000/mm3, and absolute neutrophil count >10,000/mm3 were
independent predictors of occult bacteremia. The presence of any of
these factors increased the incidence of bacteremia to 8% to 17%.
Administration of the Haemophilus influenzae type b vaccine and
the heptavalent pneumococcal conjugate vaccine have decreased the
occult bacteremia rate of well-appearing, febrile children 3 to 36
751
months of age from approximately 2% to 3% to 0.5% to 0.7% (data are
based on surveillance data by the Centers for Disease Control and Prevention).12 The Centers for Disease Control and Prevention reports a
76% reduction in invasive infections from S. pneumoniae when comparing 2005 with 1998 data in the U.S. The incidence of SBI in children 2 to
6 months old, who were incompletely or not immunized, was noted to
decrease because the widespread use of the vaccine resulted in herd immunity.13 In 2009, a decavalent pneumococcal conjugate vaccine was released in Europe and is expected to further decrease the incidence of
pneumococcal disease. Given these declines, and the fact that 80% of
pneumococcal bacteremia resolves spontaneously, the traditional standards for routine evaluation of the febrile infant 3 to 36 months old are
being reevaluated as the prevalence of occult bacteremia decreases.14
■ PNEUMONIA AND SINUSITIS
Pneumonia and sinusitis are common bacterial infections of childhood,
frequently associated with or following upper respiratory tract symptoms
(see Chapter 116, The Nose and Sinuses, Chapter 117, The Mouth and
Throat, and Chapter 121, Pneumonia in Infants and Children). Pneumonia occurs in all age groups, with the most common causative agents being the same as those for bacteremia or meningitis in each age group.
Sinusitis is uncommon in children <3 years of age because sinus formation is incomplete.
Plain chest radiographs remain the gold standard for diagnosis of
pneumonia. In neonates and young infants, routine chest radiographs are
not necessary unless the patient has specific physical examination findings suggestive of pneumonia, such as respiratory distress, rales, grunting,
significant tachypnea, or hypoxemia.6,15 In older children with chronic
medical problems, such as cystic fibrosis, congenital heart disease, or cancer, consider pneumonia in the differential diagnosis of fever and upper
respiratory tract symptoms, even if there are no signs of lower tract infection. In one study, a WBC count ≥20,000/mm3 was associated with occult
pneumonia in 19% of patients without focal findings.16 Without predisposing conditions or abnormal test results, the decision to obtain a chest
radiograph can otherwise be made clinically. Pneumonia in a febrile but
otherwise asymptomatic child is unlikely.
■ MENINGITIS
Most studies of febrile infants <3 months old cite a bacterial meningitis incidence of 1%. The most common organisms are the same as those
for bacteremia/sepsis: E. coli, Group B Streptococci, and L. monocytogenes. For children >3 months old, the most common organisms are S.
pneumoniae, Neisseria meningitidis, and Staphylococcus aureus, with a
lower incidence of S. pneumoniae meningitis since routine vaccinations
with the conjugate vaccine. The diagnosis of meningitis is made by obtaining CSF by lumbar puncture (see Chapter 143B, Pediatric Procedures: Lumbar Puncture in Children).
There is a wide overlap in CSF and peripheral blood findings, so it is often difficult to distinguish between viral and bacterial meningitis. CSF
WBC >30 cells/mm3 in the neonate and >10 cells/mm3 in children >1
month old suggest meningitis (see Chapter 143B, Pediatric Procedures:
Lumbar Puncture in Children, for further discussion). Risk factors for bacterial meningitis in children 29 days to 18 years old include (1) positive
CSF Gram stain, (2) CSF absolute neutrophil count ≥1000 cells/microliter,
(3) CSF protein ≥80 milligrams/dL, (4) peripheral blood absolute neutrophil count ≥10,000 cells/microliter, and (5) history of seizure before or at
time of presentation (Table 113-2).17 A negative bacterial meningitis
score does not exclude some treatable causes of meningitis/encephalitis
such as herpesvirus or Lyme disease.
Because of the serious morbidity of missed meningitis, even with
the application of the bacterial meningitis score, it is best to admit
those <2 months old with any degree of pleocytosis, admit all children
who appear ill, and administer appropriate antibiotics in the ED. Infants with aseptic meningitis generally should be hospitalized and ensured adequate long-term follow-up, because they are at greater risk
for dehydration and subsequent neurologic and learning disabilities.
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SECTION 12: Pediatrics
TABLE 113-2 Bacterial Meningitis Score for Infants
>2 Months Old and Well Appearing
Risk Factor*
CSF ANC
≥1000 cells/microliter
CSF protein
≥80 milligrams/dL
Peripheral blood ANC
≥10,000 cells/microliter
CSF: serum glucose
Not reliable for decision making because infrequently drawn
Seizure
Before or after presentation
CSF Gram stain
Positive Gram stain 61% sensitive, 99% specific for
bacterial meningitis
Abbreviations: ANC = absolute neutrophil count; CSF = cerebrospinal fluid.
*Any one factor = high risk for bacterial meningitis. Very low risk of bacterial meningitis if
infant lacks all high-risk criteria (NPV 100%; any one factor positive has sensitivity 98.3% and
61.5% for bacterial meningitis).
Reproduced with permission from Nigrovic LE, Kuppermann N, Macias CG, et al: Clinical
prediction rule for identifying children with cerebrospinal fluid pleocytosis at very low risk of
bacterial meningitis. JAMA 297: 52, 2007.
For those with CSF pleocytosis and likelihood of viral meningitis,
even with a negative bacterial meningitis score, if the child is to be discharged from the ED, it is wise to administer a long-acting parenteral
antibiotic, (ceftriaxone, 100 milligrams/kg) and ensure follow-up in 24
hours.17
GENERAL TREATMENT AND DISPOSITION
PRINCIPLES BASED UPON AGE
The clinical challenge is to distinguish the cause of fever: a benign viral
infection, SBI, or a noninfectious illness. Most causes are due to viral infections, but bacterial infections are not infrequent. The significance of
fever depends on multiple factors. If the physical examination identifies
the source of infection, evaluation, testing, and treatment are dictated by
the presumptive diagnosis. If the physical examination does not identify a source of infection causing fever, decision making is based first
upon age and then by height of fever. There are no absolute rules in the
evaluation and management of fever, but the guidelines in Table 113-3
are suggested for the management of neonates, infants, and children
who are well appearing, have had all relevant immunizations, and
have no obvious cause for the fever. Detailed discussion of evidencebased information is provided below under Decision Rules for Assessment of Fever in Neonates and Young Infants. Any ill-appearing infant
or child should have a complete sepsis evaluation performed and should
be admitted for parenteral antibiotic therapy.
FEVER EVALUATION IN NEONATES
AND INFANTS ≤3 MONTHS OF AGE
■ CLINICAL FEATURES
In infants ≤3 months of age, review the birth history, including the
length of the gestation, the use of peripartum antibiotics in the mother
or infant during labor or delivery, and the presence of neonatal complications, such as fever, tachypnea, or jaundice. For preterm infants, count
the age by estimated postconception date, and not by the actual delivery
date for the first 90 days of life. Signs and symptoms of SBI are typically
nonspecific in this age group. For example, vomiting and diarrhea accompany many problems, including gastroenteritis, otitis media, UTIs,
and meningitis. Alternatively, crying may be either a manifestation of
SBI or a benign condition of infancy (colic or hunger).
Undress infants completely. Assess age-appropriate normal vital
signs. Tachypnea or hypoxemia may be a clue to lower respiratory tract
infection. Inconsolable crying, or increased irritability when handled, is
frequently seen in infants with meningitis. Although fullness of the an-
terior fontanelle may be noted in some infants with meningitis, other
signs of meningeal irritation, such as nuchal rigidity, are usually absent.
Perform a head-to-toe evaluation to identify a focus of infection, such as
an inflamed tympanic membrane or evidence of cellulitis.
Treatment of neonates and infants ≤3 months old with a focal source
for fever is controversial. In a small study of tympanocentesis-confirmed
acute otitis media, there were no cases of bacteremia or meningitis, but
UTI in 9%.18 There are no other studies that have identified the incidence of bacteremia, meningitis, or UTI in children ≤3 months old who
have a focal source such as cellulitis, otitis media, or other identified bacterial infections. Therefore, even with an identified source, caution is
urged, especially in children ≤3 months old, and laboratory testing is
necessary to detect occult infection.
■ DECISION RULES FOR ASSESSMENT OF FEVER
IN NEONATES AND YOUNG INFANTS
Clinical assessment of the severity of illness in neonates and young infants is difficult. The three most commonly applied outpatient criteria
for the management of fever in well-appearing neonates and young infants are the Rochester Criteria, the Philadelphia Protocol, and the Boston Criteria (Table 113-4). All three have limitations for clinical
decision making. A comparison of these decision rules is difficult because of differences in inclusion criteria, laboratory testing, and clinical
implications for decision making. In addition, the routine administration of antibiotics to pregnant women who test Group B Streptococcus
positive from vaginal cultures and improved immunization practices
have changed the incidence of SBI, making it difficult to extrapolate
these three decision rules to current practice.
The Rochester Criteria state that in well-appearing neonates and infants ≤60 days old, without prior or peripartum illness and with a normal
complete blood count (CBC), a negative urinalysis and negative chest radiograph (if indicated) are sufficient to exclude SBI.19 However, the
Rochester Criteria miss 1% of patients with SBI and do not include
lumbar puncture as one of the diagnostic tests. The Rochester Criteria
are the least sensitive of the three guidelines for SBI.
The Philadelphia Protocol20 (Table 113-4) includes the results of lumbar puncture in clinical decision making and includes young infants 29
to 56 days old. The sensitivity of the low-risk criteria for excluding SBI
(neonatal bacteremia, UTI, or meningitis) is 98%, specificity 44%, PPV
is 14%, and NPV is 99.7%. The temperature criterion for fever was
38.2°C (100.8°F), not 38°C (100.4°F). The incidence of meningitis in this
cohort was 1.2%. Utilizing the Philadelphia Protocol, all patients with
meningitis were identified. One patient out of 288 patients, who had otherwise met low-risk criteria, was identified by an elevated CSF WBC
alone. In addition, all cases of bacteremia and UTI were identified by the
Philadelphia Protocol.
The Boston criteria (Table 113-4) attempted to identify young infants
at lower risk of SBI and safely treat them as outpatients with empiric
ceftriaxone.21 The Boston Criteria included infants 28 to 89 days of age
and accepted a peripheral WBC up to 20,000/mm3 as normal. Lumbar
puncture was performed in all patients, and all patients with meningitis
were excluded. In those who met low-risk criteria, <1% of patients had a
missed SBI, and none had complications after empiric treatment with
ceftriaxone.
Subsequent studies applying the Rochester, Philadelphia, and Boston decision rules missed SBI in neonates 0 to 30 days old.9,20,22,23 The
safest course for 0- to 30-day-old infants is sepsis testing, admission,
and empiric antibiotic treatment (Table 113-3).
The recognition of occult SBI in well-appearing neonates and infants
<3 months of age is difficult. No single clinical variable or diagnostic test
can correctly, or reliably, identify SBI in this age group. In addition, as
noted above with the Rochester, Philadelphia, and Boston decision rules,
these rules differ in their inclusion criteria. Combinations of variables
can be helpful. A study from Boston used the Classification and Regression Tree binary recursive partitioning program to choose statistically
significant cutoff points for predictive variables for SBI.24 In order of
greatest statistical significance to predict SBI were positive urinalysis,
CHAPTER 113: Fever and Serious Bacterial Illness
753
TABLE 113-3 Suggested Guidelines for the Evaluation and Management of Neonates, Infants, and Children with Fever,
Who Are Well Appearing, Have Had All Relevant Immunizations, and No Clinical Source for Fever
Age Group
Evaluation
Treatment
Neonate, 0–28 d* of age, ≥38°C (100.4°F) CBC and blood culture.
SBI incidence of ill appearing: 13%–21%; if and
not ill appearing: <5%
Urinalysis and urine culture.
and
CSF cell count, Gram stain, and culture.
Chest x-ray is optional, if no respiratory symptoms.
Stool testing if diarrhea is present.
Infant 29–56 d* of age, ≥38.2°C (100.8°F) Same as for neonates.
(Philadelphia Protocol)
SBI incidence of ill appearing: 13%–21%; if
not ill appearing: <5%
Infants 57 d* to 6 mo* of age, ≥38°C
(100.4°F)
Non-UTI SBI incidence is estimated to be
negligible
UTI is 3%–8%
Infants 57 d to 6 mo* of age ≥39°C
(102.2°F)
SBI incidence is estimated <1%; non-UTI
SBI incidence is estimated to be negligible.
UTI is 3%–8%
Infants/children 6–36 mo of age
Non-UTI SBI incidence is <0.4%
UTI in girls ≤8%
UTI in boys (<12 mo) ≤2%
Uncircumcised boys (1–2 y) remains 2%
Children >36 mo and older
Urinalysis and urine culture alone.
or
For conservative management, treat infants 57–90 d using
Philadelphia Protocol above.
Urinalysis and urine culture alone.
or
Urinalysis and urine culture in addition to CBC and blood
culture.
Urinalysis and urine culture.
Girls 6–24 mo.
Boys 6–12 mo.
Uncircumcised boys 12–24 mo.
No further workup is routinely necessary.
Admit.
and
Parenteral antibiotic therapy with ampicillin, 50 milligrams/
kg, and cefotaxime, 50 milligrams/kg, or gentamicin, 2.5
milligrams/kg.
Discharge if:
WBC ≤15,000/mm3 and ≥5000/mm3 and <20% band forms.
Urinalysis negative.
CSF WBC <10 cells/mm3.
Negative chest x-ray or fecal leukocytes if applicable.
Admit if any of above criteria are not met and treat with parenteral ceftriaxone, 50 milligrams/kg with normal CSF, 100
milligrams/kg with signs of meningitis.
Discharge if negative.
Treat for UTI with cefixime, 8 milligrams/kg/d daily or
divided twice a day, or cefpodoxime, 10 milligrams/kg/d
divided twice a day, or cefdinir, 14 milligrams/kg/d divided
every 12–24 h for 7–10 d as outpatient.
Admit and treat with the parenteral ceftriaxone if fails conservative criteria for discharge.
Discharge if negative.
Treat for UTI as above.
If WBC ≥15,000/mm3, consider treatment with ceftriaxone,
50 milligrams/kg IV/IM, and follow-up in 24 h.
If WBC ≥20,000/mm3, consider chest x-ray and CSF testing†.
Discharge if negative.
Treat for UTI as above as outpatient.
Discharge and treat with acetaminophen, 15 milligrams/kg
PO/PR every 4 h, or ibuprofen, 10 milligrams/kg PO every 6
h as needed.
Abbreviations: CBC = complete blood count; CSF = cerebrospinal fluid; SBI = serious bacterial illness; UTI = urinary tract infection; WBC = white blood cell count.
*For preterm infants, count age by estimated postconception date, and not by actual delivery date for the first 90 days of life.
†Meningismus
is difficult to discern in infants <6 months of age, and especially in infants <2 months of age. Therefore, we recommend routine CSF testing in infants <2 months of age, but
selective CSF testing in infants 2–6 months of age. There is no absolute cutoff point for prediction of meningitis with a peripheral WBC count.
WBC >20,000/mm3, temperature >39.6°C (103.3°F), WBC <4100/mm3,
and age <13 days old. No variable, or cutoff point, was 100% sensitive or
specific, and the number of meningitis cases was too small to lead to inclusion of results of lumbar puncture in the decision rule. This study
challenges the previous study protocols by introducing clinical and diagnostic test variables that were determined to be statistically significant by
the Classification and Regression Tree program. Although this is not
standard of care, this added information may help determine different
thresholds for fever evaluation in the neonate, or for admission and antibiotic therapy.
■ SPECIAL SITUATIONS: NEONATES AND
INFANTS <3 MONTHS OF AGE WITH
FEVER AND ASSOCIATED VIRAL ILLNESS
Neonates and infants with bronchiolitis have a significant incidence of
UTI.25 Similarly, neonates and infants with enterovirus and parainfluen-
za virus also have a significant incidence of SBI (UTI and bacteremia).11
ED evaluation should therefore also include a minimum of urinalysis
and urine culture in patients with bronchiolitis and urinalysis, urine culture, CBC, and blood culture in patients with suspected or proven enterovirus or parainfluenza infection.
■ TREATMENT, DISPOSITION, AND FOLLOW-UP
There appears to be no “community standard of practice” regarding the
need for hospitalization for infants 3 months of age and younger. Some
physicians hospitalize all febrile infants <3 months old, and others hospitalize selectively. Some have attributed this difference in management
to a difference in bias, with physicians in private practice having a bias
toward wellness (the infant is basically healthy) and EPs having a bias toward illness (worst-case scenario approach).27 Because the differentiation between sick and well infants <28 days of age is difficult, with
varying reports of missed SBI, all such febrile infants should have sepsis
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SECTION 12: Pediatrics
TABLE 113-4 Comparison of Rochester Criteria, Philadelphia Protocol, and Boston Low-Risk
Criteria for Assessment of Fever in Well-Appearing Neonates and Infants*
Low-Risk Criteria for Serious
Rochester Criteria
Bacterial Infection*
Fever
Age
Past medical history
Physical examination
Laboratory values
Blood count
Urinalysis
Stool
Lumbar puncture and
cerebrospinal fluid findings
Chest radiograph
Comments
Philadelphia Protocol
Boston Criteria
T ≥38°C (100.4°F).
≤60 d.
Term infant ≥37 wk gestation.
No perinatal or postnatal antibiotics.
No treatment for jaundice.
No chronic illnesses or admissions.
Not hospitalized longer than mother.
Well appearing.
Unremarkable examination.
T ≥38.2°C (100.8°F)
29–56 d
No immunodeficiency syndrome
T ≥38°C (100.4°F)
28–89 d
No immunizations within 48 h
No recent antibiotics
Same
Same
WBC ≥5000, ≤15,000/mm3.
Absolute band count ≤1500/mm3.
WBC ≤10 per high power field.
WBC ≤5 per high power field.
None.
WBC ≤15,000/mm3
Band to neutrophil ratio ≤0.2
WBC ≤10 per high power field
—
WBC ≤8 per high power field
WBC ≤20,000/mm3
WBC ≤10 per high power field
—
WBC ≤10 per high power field
Negative Gram stain
None.
Negative
Negative if obtained
Excluded lumbar puncture, so number of
Sensitivity of low-risk criteria for SBI 5 of low-risk neonates and infants had SBI (8 bacmissed meningitis cases is unknown. UTIs
98%; specificity 44%; PPV 14%; NPV teremia, 8 UTI, 10 bacterial gastroenteritis); 96%
missed in those with negative urinalysis. The 99.7%
sensitive to ceftriaxone
least sensitive of the low-risk criteria.
Abbreviations: SBI = serious bacterial illness; T = temperature; UTI = urinary tract infection; WBC = white blood cell count.
*Any single deviation from the criteria is interpreted as failure of low-risk criteria.
Reproduced with permission from Jaskiewicz JA, McCarthy CA, Richardson AC, et al: Febrile infants at low risk for serious bacterial infection—an appraisal of the Rochester criteria and implications for management. Pediatrics 94: 390, 1994; Baker MD, Bell LM, Avner JR: Outpatient management without antibiotics of fever in selected patients. N Engl J Med 329: 1437, 1993; and
Baskin MN, O’Rourke EJ, Fleisher GR: Outpatient treatment of febrile infants 28 to 89 days of age with intramuscular administration of ceftriaxone. J Pediatr 120: 22, 1992.
evaluations, including CBC, blood culture, urinalysis, urine culture, CSF
cell count and culture, and admission for parenteral antibiotics. Infants
who are ill appearing or fail to meet the low-risk criteria (Table 113-4)
should be admitted and administered parenteral antibiotics. Ampicillin
(50 milligrams/kg/dose every 8 hours) and a cefotaxime (50 milligrams/
kg/dose every 8 hours) is a common antibiotic regimen (or ampicillin
and gentamicin, 2.5 milligrams/kg/dose), but choose antibiotics based
on regional susceptibility patterns for Group B Streptococcus, E. coli, and
L. monocytogenes. Do not give ceftriaxone to infants <1 month old because it may displace bilirubin and worsen hyperbilirubinemia.
The decision to discharge a febrile infant home must be made after careful clinical and appropriate laboratory assessment, and after ensuring the
reliability of follow-up. Utilization of the Rochester Criteria, the Philadelphia Protocol, or the Boston Criteria may be considered. No missed cases
of meningitis have been described with the Philadelphia Protocol and the
Boston Criteria. The Philadelphia Protocol is recommended.
If low-risk criteria are met for the Philadelphia Protocol, the patient
may be discharged home without antibiotic administration, with evaluation in 24 hours. Additional factors for outpatient management are a
reliable caretaker with a telephone and an infant who can maintain hydration. Any child who is ill appearing should be admitted and given
parenteral antibiotics, regardless of the age.
Baskin and colleagues28 proposed parenteral ceftriaxone and 24-hour
observation (with negative cultures at 24 hours) for febrile infants between 2 and 4 weeks of age who were low risk for SBI by the Boston Criteria. Utilization of the data from viral testing may also influence the
decision to discharge to home or admit for observation.
Antibiotics are administered for clinically evident bacterial disease,
such as pneumonia, meningitis, otitis media, cellulitis, and septic arthritis. Specific management is outlined in Chapter 114, Ear and Mastoid
Disorders in Infants and Children, Chapter 121, Pneumonia in Infants
and Children, Chapter 133, Musculoskeletal Disorders in Children, and
Chapter 134, Rashes in Infants and Children.
INFANTS 3 TO 36 MONTHS OLD
■ CLINICAL FEATURES
Clinical assessment is reliable for older infants and young children. Viral
illnesses, including respiratory infections and gastroenteritis, account for
most febrile illnesses and usually have system-specific symptoms, such as
vomiting, diarrhea, rhinorrhea, cough, or rash. Characteristics to note are
willingness of patients to make eye contact, playfulness and positive response to interactions, negative response to noxious stimuli, alertness, and
ease of consolation. Toxic infants will not respond appropriately.
Otitis media is generally caused by S. pneumoniae or nontypeable H.
influenzae. Although pneumonia is commonly of viral etiology, it is difficult to distinguish bacterial from viral causes. In older infants or young
children with UTI, fever is usually the only presenting sign, but a history
of foul-smelling urine or crying with urination may be noted. Cellulitis
is clinically apparent. Abscess may be associated with these patients as
well. Bacterial pharyngitis is unlikely under the age of 3 years old.
Nuchal rigidity and Kernig or Brudzinski signs may be absent in children with meningitis even up to the age of 2 years old. A bulging fontanelle, vomiting, irritability that increases when the infant is held,
inconsolability, or a complex febrile seizure may be the only signs suggestive of meningitis. Infants with aseptic meningitis generally should
be hospitalized and ensured adequate long-term follow-up because
they are at greater risk for dehydration and subsequent neurologic
and learning disabilities.
CHAPTER 114: Ear and Mastoid Disorders in Infants and Children
Petechiae noted on physical examination are concerning for infection
by N. meningitidis. However, most children with fever and petechiae will
have a viral cause, such as adenovirus, whereas purpura fulminans (see
Figure 245-19), hypotension, lethargy, and meningismus predict meningococcemia. For the well-appearing child with fever and petechiae, there
are no good predictors for SBI. Ultimately, time may be the best diagnostic test, with a brief observation period or admission warranted for cases
that are indeterminate.
■ DIAGNOSIS
The American Academy of Pediatrics practice guidelines advocate
testing for UTI in all girls and in all uncircumcised boys <2 years old
if no apparent focus of infection is present.5 Circumcised boys should
be tested if <1 year of age.
Many would advocate that empiric blood testing and treatment for
these patients is no longer necessary. Although reports of decreased bacteremia incidence have been published, no studies to determine predictors of bacteremia, outcome of empiric antibiotic use, reduction of
complications, or outcome of nontreatment protocols have been published since the introduction of the pneumococcal conjugate vaccine. In
addition, no practice parameters have established a current standard of
care, and the American Academy of Pediatrics has not renewed their
practice guideline since 1993. The physician is therefore left with evidence of decreased incidence of bacteremia, but no studies or practice
parameters supporting a selective testing or nontesting protocol.
Given the decrease in invasive pneumococcal disease, and the limited
predictive value of an elevated WBC for bacteremia,29 three options exist.
The first option is following the previous practice parameters and obtaining
a CBC and blood culture on all children between 3 and 36 months old with
a fever >39°C (102.2°F), and then treating with ceftriaxone only if the WBC
>15,000/mm3. Because of the low PPV of an elevated WBC, the second option is obtaining a blood culture and waiting for results before beginning
empiric treatment. The third option is to assume that the incidence of pneumococcal disease has decreased so substantially that laboratory evaluation
in a well-appearing child is unnecessary, and discharge with expectant follow-up with their primary care provider is adequate. Variants on the first
and second options may be to evaluate children between 2 and 6 months of
age because these children have not received the first three vaccine series.
755
For organisms other than S. pneumoniae, N. meningitidis, or methicillin-resistant S. aureus, more conservative management may be
warranted.
Any child who appears ill or toxic should be admitted to the hospital.
Likewise, children who are thought to be at risk for a SBI and do not have
reliable follow-up or the ability to return to the hospital should also be
admitted for inpatient management.
FEVER IN CHILDREN >36 MONTHS OF AGE
Children >36 months old are easier to evaluate, and their complaints are
usually more specific. Children with infections such as UTI, meningitis,
pneumonia, pharyngitis, and otitis media are more likely to complain of
symptoms typical for these diagnoses. Pharyngitis due to Group A Streptococcus becomes more common in this age group, especially in the
school-aged child. However, infectious mononucleosis also becomes more
prevalent in this age group, and may mimic the signs and symptoms of
Group A streptococcal pharyngitis. Treatment for Group A streptococcal
infection is amoxicillin (40 to 50 milligrams/kg divided twice a day or three
times a day for 10 days), penicillin G benzathine (50,000 units/kg IM single
dose, up to 900,000 units IM for older pediatric patients), or azithromycin
(10 milligrams/kg daily for 3 days) for penicillin-allergic patients.
Kawasaki disease (see Chapter 134, Rashes in Infants and Children)
also typically presents in children <5 years of age. Patients usually have
high fevers for ≥5 days, strawberry-appearing tongue, conjunctivitis and
iritis, red mucous membranes in the mouth and dry cracked lips, and
swollen lymph nodes. Peeling of the skin in the hands, feet, and genital
area may also occur in the later phases. Variants of Kawasaki disease also
occur, with fewer of these classic signs. Untreated, patients with Kawasaki disease may develop life-threatening coronary aneurysms. Treatment
for Kawasaki disease involves aspirin and IV immunoglobulin.
Acknowledgment: The author would like to thank Dr. Carol D. Berkowitz
who authored this chapter for previous editions.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
■ TREATMENT, DISPOSITION, AND FOLLOW-UP
Any child who appears ill or toxic, is unable to maintain oral hydration,
or has inadequate follow-up after discharge, should be admitted for IV
hydration and/or parenteral antibiotic therapy. Choices for antibiotics
depend on the organism and the regional susceptibilities.
For recognized bacterial infections, use appropriate antibiotics based
on the type of infection and regional and national standards.
■ POSITIVE BLOOD CULTURES
Recall all children with positive blood cultures.
In the case of positive S. pneumoniae cultures:
• If the child is receiving appropriate antibiotics, is clinically well, and afebrile, the child should complete the course of therapy.
• If afebrile and clinically well but not receiving antibiotics, opinions differ regarding the need for additional blood cultures and antibiotic
therapy. In general, neither are necessary unless the child has
developed a specific focus of infection.
The febrile child should receive a complete sepsis evaluation (CBC,
urinalysis, CSF indices, and blood, urine, and CSF cell count and cultures). For the persistently febrile patient who is well appearing and has
a normal evaluation, admission is usual, although empiric treatment
with ceftriaxone and follow-up as an outpatient may be considered.
For any patient who is ill appearing, obtain the sepsis evaluation and
admit for parenteral antibiotics.
Children with cultures positive for N. meningitidis or methicillinresistant S. aureus should be admitted for parenteral antibiotic therapy.
CHAPTER
114
Ear and Mastoid Disorders
in Infants and Children
David M. Spiro
Donald H. Arnold
OTITIS MEDIA
Otitis media is the most common outpatient pediatric diagnosis. There
are approximately 25 million office visits for this condition in the U.S.,
with direct costs greater than $5 billion per year.1 Otitis media is inflammation of the space of the middle ear. Otitis media is a general term that
has been used to describe multiple disorders of the middle ear, including
acute otitis media, chronic otitis media, and otitis media with effusion.
This chapter discusses acute otitis media, otitis media with effusion, otitis externa, and acute mastoiditis.
■ ACUTE OTITIS MEDIA
Epidemiology Otitis media accounts for 13% of all visits (including children and adults) to U.S. EDs.2 Accurate data on the true incidence of
acute otitis media are lacking, as previous surveys have not made the distinction between acute otitis media, otitis media with effusion, and eustachian tube dysfunction. There is considerable overlap between presenting
756
SECTION 12: Pediatrics
signs and symptoms of upper respiratory illnesses and acute otitis media,
especially in the preverbal child.
The peak incidence of otitis media is between 6 and 18 months of age.3
In the U.S., up to 50% of children will have had at least one episode of
acute otitis media by the age of 1 year.1 The incidence is higher in children
who are Native Americans, Eskimos, males, day care attendees, exposed
to tobacco smoke, born with craniofacial anomalies, prone position
sleepers, pacifier users, diagnosed with their first episode of acute otitis
media at <6 months, or born with immunodeficiency syndromes.1,3 The
incidence is lower in infants who are breastfed.1
Acute otitis media spontaneously resolves in most cases.4 Acute otitis
media and the development of otitis media with effusion may be natural
occurrences as the anatomy and immune system develop in the pediatric
population. Environmental insults, such as tobacco smoke, lack of
breastfeeding, and exposure to children in day care who frequently receive antibiotics, may lead to episodes that would not otherwise occur.
Prevention of the disease burden is possible through the use of newer
vaccines such as the contemporary pneumococcal vaccine that includes
protection from seven serotypes of Streptococcus pneumoniae.5
Pathophysiology The middle ear is a laterally compressed cavity within
the temporal bone bounded by the tympanic membrane laterally and the
eustachian tube medially. In the healthy state, this space is aerated and
contains the ossicular chain, which serves to transmit sound energy to
the inner ear. In children, as compared with adults, the eustachian tube
is shorter and more horizontally oriented. This orientation is the anatomic rationale for the increased incidence of disease seen in children.
An upper respiratory tract infection can obstruct the eustachian tube
and disrupt its function of aerating the middle ear. Thus, an obstructed
eustachian tube prevents equilibration of air pressure between the middle ear and the atmosphere and creates conditions favorable to the development of sterile or purulent effusions.
Bacteria are the most common cause of acute otitis media and can be
isolated from middle ear fluid in a majority of cases. Bacteria originate
from the nasopharynx and enter the middle ear space via the eustachian
tube. The most common pathogens in the postpneumococcal vaccine
era are S. pneumoniae (31%) and nontypeable Haemophilus influenzae
(56%), followed by Moraxella catarrhalis.6 These data were obtained in
the postpneumococcal vaccine era and represent a change from before
this vaccination program was initiated. Of importance is a major change
in the increased prevalence of β-lactamase organisms such as M. catarrhalis (almost 100%) and nontypeable H. influenzae (35% to 40%).1 Neonatal acute otitis media is uncommon. Most effusions of the middle ear in
this age are sterile and develop in the in utero environment.
Clinical Features The classic signs and symptoms of acute otitis media
are ear pain and fever. Often, the young child presents to the ED with a
history of upper respiratory symptoms that are associated with acute otitis media. In the preverbal child, a fussy, irritable, or nonconsolable infant may be the presenting symptoms.
Diagnosis Acute otitis media and otitis media with effusion are processes that occur along the same continuum, and otitis media with effusion
is usually a result of acute otitis media. The relationship between the two
disorders often results in variations in the definition of acute otitis media
over time and diagnostic uncertainty when these patients present to the
ED. The most important step in reducing unnecessary antibiotics for
acute otitis media is to establish a proper diagnosis, as antibiotic
treatment is not recommended for otitis media with effusion.
The definition of acute otitis media requires three equally important
components (Table 114-1).7
1. Acute onset (<48 hours) of signs and symptoms, and
2. Middle ear effusion (MEE), and
3. Signs and symptoms of middle ear inflammation.
The first step in establishing the presence or absence of physical findings of the middle ear is a properly conducted otoscopic evaluation. The
two most common types of otoscopic heads are the round and rectangular types. The round head, primarily used by otolaryngologists, has a
TABLE 114-1 Diagnostic Elements, Method, and
Criteria for Acute Otitis Media
Diagnostic
Element
Acute onset
Middle ear
effusion
Middle ear
inflammation
Method
Criteria
History of illness
Pneumatic otoscopy
or
Otorrhea
or
Tympanometry
Otoscopy
<48 h
Bulging of the TM
Limited TM mobility
Air fluid level behind TM
At least one of the following: fever,
otalgia, irritability in infant, red TM not
due to crying or fever
Abbreviation: TM = tympanic membrane.
Reproduced with permission from Subcommittee on Management of Acute Otitis Media:
Diagnosis and management of acute otitis media. Pediatrics 113: 1451, 2004.
glass back that can swivel out for direct, nonmagnified visualization and
for foreign body and cerumen removal. The rectangular heads are plastic
backs and are more commonly used in the ED. Neither head is superior
to the other. Use of a bright light source, clean otoscope head, and a
properly fitting speculum are key steps to accurately assess the tympanic
membrane.
Consider the age of the child to obtain proper, gentle immobilization
of the head. Discuss immobilization with the family before the otoscopic
examination. Many experienced parents feel comfortable holding the
child in their arms with the child’s head rested against the shoulder or
chest of the adult provider. In infants and younger children, having the
patient in the supine position with the examiner controlling the head of
the child with the parents holding the arms is often preferred. If the
child’s head is uncontrolled, an accurate examination of the tympanic
membrane may not be possible.
A small amount of cerumen may not inhibit tympanic membrane visualization, but impacted cerumen must be removed. Techniques for removal of cerumen include use of a soft speculum or gentle irrigation of
the canal with warm water. Both procedures can cause pain or traumatic
perforation of the tympanic membrane. Docusate, a ceruminolytic
agent, is an alternative.8 Break open a capsule of docusate, and then
instill 1 mL of the capsule contents into the ear canal. Let it remain for
15 minutes, then irrigate with saline or water. Although no technique
is always successful or risk free, cerumen removal is necessary to properly visualize the tympanic membrane and assess mobility to confirm the
presence or absence of an effusion in the middle ear space.
Assess the presence or absence of discharge; the position or contour
(normal, bulging, retracted), color (pink, grey, red, yellow), the degree of
translucency (translucent, opaque, nonopaque), and mobility (normal,
increased, decreased, absent) of the tympanic membrane. The normal
eardrum is translucent and pearly gray (Figure 114-1). The tympanic
membrane may become reddened with crying or with high fever, so the
examination must be performed in a manner that is gentle and nonthreatening. The result of an examination in an angry or crying child is a
red tympanic membrane, leaving the quandary whether the erythema is a
result of crying or inflammation. The eardrum should be freely mobile in
response to positive and negative pressure by the pneumatoscope, but retracted tympanic membranes have reduced mobility. The tympanic
membrane of acute otitis media is usually opaque, pale yellow, red, and
often bulging, and bony landmarks (long and short process of the
malleus) are not easily discernible (Figure 114-2). The loss or decrease in
mobility of the tympanic membrane implies the presence of an MEE.9
Aspiration of the middle ear is the most definitive method of verifying
the presence and type of MEE and infecting organism. But this is rarely
practiced in the ED. Middle ear aspiration may be beneficial in (1) children with sepsis, (2) children with immunodeficiency disorders, (3) neo-
CHAPTER 114: Ear and Mastoid Disorders in Infants and Children
Anterior
superior
Anterior
inferior
Posterior
superior
Posterior
inferior
A
757
B
FIGURE 114-1. Normal right tympanic membrane in 6-year-old child. (Courtesy
of Dr. Shelagh Cofer, Department of Otolaryngology, Mayo Clinic.)
nates, (4) children with persistent symptoms of acute otitis media after
>48 to 72 hours on antimicrobial therapy, or (5) purulent otitis media
with an extremely painful, bulging tympanic membrane. Diagnostic
tympanocentesis is performed by inserting a 2.5- to 3.0-in. 18-gauge spinal needle or catheter over a needle attached to a syringe through the anterior inferior or posterior inferior quadrant of the tympanic membrane
(Figure 114-3). Tympanocentesis should always be performed with continual observation of the tympanic membrane and needle through the
otoscope. The aspirate should be cultured in blood culture broth, on
blood and chocolate agar plates, and sent for Gram stain.
The differential diagnosis for acute otalgia is listed in Table 114-2.
Foreign body in the external ear canal may present with otalgia. This di-
FIGURE 114-2. Acute otitis media in a 3-year-old child with an outward bulge of
the tympanic membrane and an exudative process in the middle ear space. (Courtesy of Dr. Shelagh Cofer, Department of Otolaryngology, Mayo Clinic.)
FIGURE 114-3. A and B. Tympanocentesis. Take care to not damage the ossicles
of the middle ear during tympanocentesis. Apply topical benzocaine for local anesthesia before the procedure. Procedural sedation may be necessary. (Reproduced
with permission from Reichman EF, Simon RR: Emergency Medicine Procedures.
Copyright © 2004 The McGraw-Hill Companies, Inc. All rights reserved. Section 12,
Otolaryngologic Procedures; Chapter 143, Tympanocentesis; Figure 143-1.)
agnosis is usually confirmed with a good otoscopic examination. However, blood in the ear canal may obscure a foreign body. Otitis externa is
more likely with a history of exposure to water. Pain may occur with gentle movement of the pinna. An edematous or foul-smelling external canal helps confirm the diagnosis.
Treatment Consensus guidelines strongly recommend the treatment of
pain associated with acute otitis media.7 Prescribing only an antibiotic for
the treatment of acute otitis media is inappropriate care, as antibiotics
are not analgesic medications. There is a paucity of literature addressing
management of ear pain.2 For centuries, home remedies, such as various
oils (e.g., olive oil) and impregnated wool, have been used to treat otalgia.
One study found that an herbal extract may be beneficial and that use of an
antibiotic did not improve pain scores.10 Two studies have demonstrated
the short-term effectiveness of topical analgesics such as benzocaine/antiTABLE 114-2 Differential Diagnosis of Acute Otalgia
Common
Acute otitis media
Serous otitis media
Foreign body in the external ear canal
Otitis externa
Less common
Accidental trauma (e.g., perforation of tympanic membrane)
Oral cavity diseases (referred pain)
Cholesteatoma
Peritonsillar abscess
Rare
Mastoiditis
Brain abscess
Lemierre syndrome
Herpes zoster oticus (Ramsay Hunt syndrome)
Rhabdomyosarcoma of the ear or temporal bone
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SECTION 12: Pediatrics
TABLE 114-3 Treatment of Otalgia in Acute Otitis Media
Medication
Dosage
Systemic
Acetaminophen 15 milligrams/kg PO/PR
every 4 h, as needed
Ibuprofen
10 milligrams/kg PO every
6 h, as needed
Hydrocodone
0.2 milligram/kg PO every
6–8 h, as needed
Topical
Antipyrine/
2–4 drops in the ear every
benzocaine
1–2 h, as needed for pain
Lignocaine
Comments
May reduce fever primarily.
May reduce fever, take with
food.
Consider bedtime dosing or
for severe otalgia.
Insert moist cotton into
external canal after drops
applied.
pyrine and lignocaine.11,12 Topical analgesics are contraindicated with a
known perforated tympanic membrane.
The primary systemic analgesics and antipyretics used to treat acute
otitis media are ibuprofen and acetaminophen. In one randomized trial
comparing ibuprofen, acetaminophen, and placebo, only ibuprofen was
found to be superior to placebo.13 Opioid medications may be used at
night during the sleeping hours, although no studies have demonstrated
the effectiveness of this medication class for the treatment of acute otitis
media. Table 114-3 lists medications used to treat otalgia associated with
acute otitis media.
Millions of antibiotic prescriptions are written annually in the U.S.
for the treatment of acute otitis media despite evidence that this condition primarily resolves spontaneously. Unfortunately, the widespread
use of antibiotics is strongly associated with antimicrobial resistance,
which is a significant public health concern.14 A recent consensus
guideline recommends an observation option, defined as withholding
immediate, systemic antibiotics.7 An observational approach for acute
otitis media has been used successfully in areas of Europe with similar
rates of mastoiditis compared to the U.S.15 Mastoiditis is the primary
suppurative complication of acute otitis media. Interestingly, the majority of cases of mastoiditis are associated with antimicrobial use before presentation.16
There are eight published trials evaluating the use of an observational
approach for acute otitis media, two of which have been evaluated in the
setting of the ED.17,18 The first such trial enrolled children in a randomized controlled trial who were clinically diagnosed with acute otitis media using a consensus guideline.17 Children were either prescribed a
wait-and-see antibiotic prescription (WASP) or told to fill an antibiotic
prescription immediately. There were no significant differences between
groups in regard to otalgia, fever, otorrhea, or unscheduled medical visits. Approximately two of every three families were found to fill the antibiotic prescription. Fever and otalgia were the primary reasons for
filling the prescription. The second trial in the ED setting prospectively
compared two observational models in the pediatric ED.18 Patients were
randomly assigned to receive a WASP or observation without a prescription. Observational therapy was well accepted by both groups, which has
been a consistent theme in previously published trials. Adherence to primary observation without a written prescription was better than when a
WASP was given at the time of visit.
Acute otitis media is immediately treated with an antibiotic for infants under the age of 6 months or when a wait-and-see is inappropriate (Table 114-4). High-dose amoxicillin, 80 to 90 milligrams/kg PO
per day divided into two daily doses for 5 to 7 days, is the first-line recommended antibiotic for the treatment of uncomplicated acute otitis
media.7 The higher dose achieves concentrations in the middle ear that
exceed the minimum inhibitory concentration for highly resistant forms
of S. pneumoniae, the most common bacteria found in acute otitis media.
Amoxicillin-clavulanate may be chosen if high-dose amoxicillin fails in
order to provide coverage against β-lactamase–producing M. catarrhalis
TABLE 114-4 Indications for Immediate Antibiotic
Use for Acute Otitis Media
Age <6 mo (consider another source for fever if <2 mo of age)
Child ill appearing (e.g., shock, poorly responsive, floppy, drowsy)
Clinician suspects another bacterial illness (e.g., urinary tract infection)
Recurrent acute otitis media (within 2–4 wk)
Immunocompromised
Uncertain access to medical care
Craniofacial anomalies
and nontypeable H. influenzae, although the addition of clavulanic acid
increases the likelihood of vomiting and diarrhea. IM ceftriaxone for
three daily doses may be considered if children cannot tolerate oral medications. Children with a known allergy to the penicillin class may consider the use of a macrolide agent, such as azithromycin. A 10-day course
of antimicrobials has been recommended for decades without any evidence to support an exact duration of therapy. Shortened treatment regimens (5 to 7 days) may reduce resistance to antibiotics and reduce side
effects by decreasing total drug exposure. Fever and ear pain should be
expected for 24 to 48 hours after an ED evaluation. If symptoms persist
72 hours after antibiotic therapy has been initiated, reevaluation is needed. Routine, scheduled visits are not recommended for uncomplicated
acute otitis media if symptoms have resolved. Figure 114-4 illustrates a
proposed management scheme for acute otitis media.
■ OTITIS MEDIA WITH EFFUSION
Otitis media with effusion is defined as fluid in the middle ear space
without clinical signs of inflammation or acute symptoms of illness
(Figure 114-5). Otitis media with effusion usually follows an episode of
acute otitis media, and both are processes of the same disease continuum. Approximately 2 million episodes of otitis media with effusion are
diagnosed annually in the U.S., with an estimated annual cost of $4 billion.19 Otitis media with effusion may persist for weeks to months after
an episode of acute otitis media. Pneumatic otoscopy is strongly recommended as the primary method to diagnose otitis media with effusion.
Close to 90% of episodes of otitis media with effusion resolve spontaneously after an acute otitis media episode is diagnosed.4 The American
Academy of Pediatrics 2004 consensus guideline recommends watchful
waiting without immediate use of antibiotics for children with uncomplicated otitis media with effusion.19 Children with permanent hearing
loss, craniofacial anomalies, or underlying speech delays may receive immediate antibiotics or have close outpatient follow-up by the primary
care clinician. Long-term benefits of antihistamines, decongestants, corticosteroids, and antibiotics are unproven and not routinely recommended.7 Mild to moderate conductive hearing loss in the range of 10 to
20 dB is the most prevalent complication of otitis media with effusion.
Hearing and language testing is recommended if otitis media with effusion lasts >3 months.19
■ OTITIS EXTERNA
Otitis externa is inflammation of the external ear canal, auricle, or outer
surface of the tympanic membrane. Otitis externa is most often caused
by infection, but local trauma can also produce otitis externa. The peak
prevalence occurs between 7 and 12 years of life, and it is rarely diagnosed before the age of 3 years old.20
Pathophysiology The external canal wall is layered with thin cartilage
and lined with skin. The epidermal skin layer of the canal is contiguous
with the outermost layer of the tympanic membrane. Flora of the external ear canal are similar to those of normal skin and include Staphylococcus epidermidis and α-hemolytic Streptococcus species. Otitis externa is
usually caused by colonization of invasive organisms, the most common
of which are Pseudomonas aeruginosa, S. epidermidis, and S. aureus,
which often coexist.21 Otitis externa may occur when protective features
CHAPTER 114: Ear and Mastoid Disorders in Infants and Children
759
Acute Otitis Media
(all three must be present)
1. Acute onset (<48 hours)
2. Middle ear effusion
3. Middle ear inflammation
Age: <6 months
Antibiotics immediately
Analgesics
Age: >6 months
Yes
High-Risk Factors
(any one suffices to exclude)
1. Ill appearance
2. Documented AOM within 1 week
3. Concurrent antibiotic treatment
4. Limited access to medical care
5. Other bacterial infections
6. Immunocompromise
No
Wait-and-see
antibiotic prescription
Analgesics
Symptoms worsen
or persist
(48–72 hours)
Yes
FIGURE 114-4. Proposed management of acute otitis media
(AOM).
of the ear canal are compromised. The most common cause is due to hyperhydration and maceration of the epithelial tissue, often induced when
a child is submerged during swimming. Occasionally, otitis externa can
occur with mechanical debridement of the epithelial layer from trauma
(e.g., cotton swab inserted in the external canal).
Clinical Features Otitis externa may cause a sense of ear fullness or itching in the early stages of disease. Pain usually develops associated with a
FIGURE 114-5. Well-appearing toddler with middle ear effusion. Note slight bulging of tympanic membrane. (Courtesy of Dr. Shelagh Cofer, Department of Otolaryngology, Mayo Clinic.)
Antibiotic treatment
Analgesics
No
Follow-up as needed
Analgesics
purulent and sometimes cheesy, foul-smelling discharge. As otitis externa progresses, pain can be induced with any range of motion of the temporomandibular joint. On physical examination, gentle tugging of the
auricle or inward pressure of the tragus can induce discomfort. In the severe form of otitis externa, further anterior spread can cause tenderness
of the surrounding lymphoid and subcutaneous tissue. Rarely, posterior
spread can involve the mastoid or can cause osteomyelitis of the skull.
Diagnosis A diligent otoscopic examination is needed to make the diagnosis of otitis externa. Placing the speculum of the otoscope into the external ear canal may induce pain. Visualization of the external canal
during otoscopy may reveal purulent discharge or edema of the canal
surface, and visualization of the tympanic membrane may be difficult or
impossible. Otoscopic inspection may reveal a normal or erythematous
tympanic membrane. Pneumatic otoscopy should reveal adequate mobility of the tympanic membrane to exclude acute otitis media. Acute
otitis media with perforation may be difficult to distinguish from otitis
externa, as both can present with pus in the external ear canal. Check
carefully to identify the presence or absence of a foreign body. Malignant otitis externa is osteomyelitis of the ear canal and should be suspected with the presence of fever >38.9°C (102°F), if pain is severe, or
with the presence of facial paralysis or meningeal signs.
Treatment Treatment of otitis externa includes pain relief, eradication of
infection, and prevention of future infections. Oral analgesics, such as
ibuprofen, are used to reduce pain. Primary therapy is directed toward
cleaning and drying the external ear canal. Dry mopping with a cottontipped wire applicator may be sufficient and curative in mild cases. Mild
otitis externa may also be treated with acidifying agents, such as 2% acetic acid drops applied topically three times daily. These medications
may be painful upon application and are contraindicated in the presence of a suspected tympanic membrane perforation.
For uncomplicated otitis externa, routine cultures are not needed. Topical fluoroquinolone drops, such as ofloxacin or ciprofloxacin, instilled
760
SECTION 12: Pediatrics
into the ear canal twice a day, are the standard treatment. Polymyxin B/
neomycin/hydrocortisone preparations have been traditionally recommended as first-line therapy, with reported cure rates that range from 80%
to 90%.22 However, neomycin hypersensitivity is common, and other topical medications are available. When instilling antibiotic drops, the child
should lie down with the affected ear upward. When filling the ear with the
topical agent, the pinna can be gently moved back and forth to improve delivery throughout the entire external canal. The child should remain in this
position for 5 minutes after application.
If the external canal is extremely edematous, place a Pope ear wick to
improve delivery of topical treatments. The wick should be kept dry and
removed in 3 days. Children who fail therapy should be reexamined in 48
to 72 hours, and the clinician should consider other diagnoses. Parenteral
therapy may be required in patients with spreading infection outside the
anatomic boundaries of the external canal. In this case, obtain cultures of
the external canal, and administer antipseudomonal (ceftazidime, 50 milligrams/kg IV every 8 hours) and penicillinase-resistant (methicillin, 50
milligrams/kg/dose every 6 hours) antibiotics (Table 114-5).
Preventing Otitis Externa Patients should avoid swimming as the canal
heals. After a recent infection or with repeated infections, earplugs
should be used while swimming. The routine use of cotton swabs applied
to the external canal is discouraged, as this may cause further trauma to
the lining of the canal wall. During at-risk periods (e.g., swimming season), daily prophylaxis with acidifying and drying drops (e.g., vinegar
and isopropyl alcohol as 1:1 solution) may prevent infection.
■ ACUTE MASTOIDITIS
Mastoiditis is infection or inflammation of the mastoid process. At birth,
the mastoid consists of one cell called the antrum. By 3 years of age, most
children develop mastoid air cells, which form a honeycomb appearance.
The incidence rate of mastoiditis in the U.S. ranges from 1.2 to 2.0 cases
per 100,000 person-years.15 This incidence is similar in the Netherlands,
which has a low rate of antibiotic prescriptions for acute otitis media.15
The prevalence is similar to acute otitis media, with a peak age of 12 to
36 months.
Mastoiditis is subclassified into two forms: classic and latent. Classic
disease is acute mastoiditis that follows acute otitis media. Latent disease
is chronic and may have subtle findings. Latent disease has been attributed to partial antibiotic treatment of acute otitis media. For the purpose
of this chapter, only classic, or acute, mastoiditis is discussed.
Pathophysiology Acute mastoiditis develops when inflammation of the
middle ear spreads into the cells of the mastoid through the aditus ad antrum. This happens when bacteria in the middle ear space cannot be resorbed properly or when the eustachian tube is obstructed. This process
can induce destruction of the mastoid bone and periosteum. This local
inflammatory process can extend into the cranial cavity and cause local
destruction of the bone. Extension of the local disease can lead to brain
abscess. Meningitis can occur through direct extension of disease or secondary to hematogenous spread. A rare complication of acute otitis media and mastoiditis is otitic hydrocephalus, which is due to a thrombosis
of the transverse sinus of the dura. Otitic hydrocephalus can present with
vomiting and headache associated with acute otitis media. Diagnosis of
otitic hydrocephalus can be made by CT or MRI.
Clinical Features Risk factors for disease include recurrent acute otitis
media, immunocompromise, or the presence of a cholesteatoma (Figure
114-6). The vast majority of acute mastoiditis occurs as a result of, or simultaneous with, acute otitis media. Therefore, the otoscopic examination
will demonstrate a bulging and erythematous tympanic membrane. Mastoiditis is not likely if middle ear examination is normal. For children <1
year of age, symptoms may be nonspecific and may include disruption of
sleep, irritability, or decreased intake of liquids. Acute mastoiditis can
most often be diagnosed clinically at the bedside. In addition to abnormal
tympanic membrane findings, erythema, tenderness, and edema over the
mastoid may be appreciated. This process may induce a classic protrusion
of the auricle as seen in Figures 114-7 and 114-8. Cranial nerve involvement may be seen with advanced disease, which may cause palsies of the
VI (abducens) or VII (facial) nerves.
Diagnosis Once mastoiditis is suspected, early consultation with a pediatric otolaryngologist is recommended. The diagnosis is often suspected
clinically and confirmed by CT of the mastoid. CT imaging has a high
sensitivity (~90% to 100%) to confirm fluid in the middle ear and mastoid spaces. CT imaging is also useful to determine spread of the disease
into the intracranial space. Complete blood count and erythrocyte sedimentation rate are nonspecific tests and rarely change outcome. Recommended lab studies include cultures of the blood and middle ear fluid if
the child is febrile.
Treatment Currently, the majority of cases are treated conservatively
with antibiotics. Antibiotic therapy should be directed toward the most
common bacteria, which are the same three organisms associated with
acute otitis media, in addition to S. aureus, S. pyogenes, and P. aeruginosa. A recent review of 86 children in Israel found that S. pneumoniae
TABLE 114-5 Common Agents for the Treatment of Otitis Externa
Medication
Dosage
Comments
Ibuprofen
10 milligrams/kg PO every
6 h, as needed
2–4 drops three to four
times a day
May reduce fever, take with
food
Inexpensive; effective for
mild otitis externa and for
prevention during high-risk
periods; can sting with
application
Inexpensive; can sting with
application; neomycin
allergy is common
Expensive; ofloxacin is only
agent approved if the
tympanic membrane is
perforated
2% acetic acid
(VoSoL)
Neomycin, polymyxin 2–4 drops four times a day
B, hydrocortisone
Fluoroquinolones
2–4 drops twice a day
FIGURE 114-6. Cholesteatoma. A cholesteatoma is seen in this ear. Primary
acquired cholesteatomas are thought to arise from gradual invagination of the pars
flaccida, usually secondary to trauma. Note the yellow epithelial debris from the
cholesteatoma in the area of the pars flaccida. Often there is an effusion and debris,
which can distort the anatomy on otoscopy. (Courtesy of C. Bruce MacDonald, MD.
Reproduced with permission from Knoop et al: Atlas of Emergency Medicine, 2nd
ed. Jauch et al: Chapter 5, Ear, Nose, and Throat Conditions, Figure 5-9.)
CHAPTER 115: Eye Problems in Infants and Children
761
after discharge from the inpatient setting. For uncomplicated disease,
simple myringotomy or placement of tympanostomy tubes is usually
performed by otolaryngology. Mastoidectomy is performed if clinical
improvement does not occur with antibiotics or if extra-mastoid spread
of disease has been confirmed.
Acknowledgments: The authors gratefully acknowledge the contributions of Drs. Kimberly Quayle, Susan Fuchs, and David M. Jaffe, the authors of this chapter in the previous edition. We are also appreciative of
Dr. Shelagh Cofer for her otoscopic pictures and Dr. Larry Stack for his
photograph of mastoiditis.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
115
FIGURE 114-7. Note protrusion of the auricle. (Courtesy of Dr. Lawrence B. Stack,
Department of Emergency Medicine, Vanderbilt University.)
and P. aeruginosa were the most common organisms identified.23 If
acute mastoiditis is diagnosed after antibiotic treatment for acute otitis
media, mastoid cultures may be sterile.24 Inpatient admission is warranted, and IV antibiotics (ampicillin-sulbactam, 100 milligrams/kg IV every
6 hours) are recommended until the physical signs have diminished. Parenteral therapy is followed by an oral course of antibiotics for 2 weeks
Eye Problems in
Infants and Children
Thomas A. Mayer
Katherine Fullerton
Bill Bosley
Pediatric ophthalmologic problems are a common, yet challenging issue
for all emergency physicians. The history often comes from the parents,
particularly in preverbal children, and it may even be difficult for older
children to fully articulate their symptoms. The child needs to be calmed
and reassured sufficiently to allow for a complete and thorough examination. It is important to comfort parents as well as the child. This chapter includes a review of eye examination techniques, and illnesses and
injuries specific to the care of children. Important emergency pediatric
eye problems are discussed. Because the care of pediatric and adult trauma to the eye and its surrounding structures are similar, only those areas
of difference are discussed in this chapter. Further discussion of eye
emergencies is provided in Chapter 236, Eye Emergencies.
EYE ANATOMY
Eye anatomy is presented in Figures 115-1, 115-2, 115-3, and 115-4.
EYE EXAMINATION IN A CHILD
FIGURE 114-8. Note protrusion of the auricle. (Courtesy of Dr. Shelagh Cofer,
Department of Otolaryngology, Mayo Clinic.)
Perform a general survey of the child to note any obvious abnormalities—
rash, soft tissue changes, matter on the lashes, injection of the conjunctiva, drainage from the eye, corneal or lens opacities, any misalignment of
the eyes, or ptosis. Newborns may appear cross-eyed during the first
month of life.
Visual acuity (VA) is the vital sign of the eye, and it should be the first
objective measurement obtained after the history. The one exception to
obtaining VA first is a chemical exposure, which requires immediate copious irrigation with normal saline. Obtaining VA in a child will depend
on the child’s age and level of development. The Snellen, Allen, and
Rosenbaum charts check distance VA. If the child knows letters of the alphabet (typically 4 to 6 years of age), the standard Snellen eye chart may
be used; if the child knows numbers, the Snellen number chart may be
used. When using the Snellen charts, check acuity at a distance of 20 ft.
Document VA for the lowest line on which four or more characters were
correctly identified. One can also chart “20/30 minus 3,” which indicates
the patient incorrectly identified three characters in the 20/30 line. For
children 3 to 5 years of age, an Allen picture chart or the single tumbling
“E” chart may be used. These charts check distance VA and should be located at a distance of 10 ft from the patient. If this is not possible, use the
762
SECTION 12: Pediatrics
Superior
punctum
Frontal bone
A
Lacrimal
gland
Levator muscle
Caruncle
Superior rectus muscle
Pupil
Sclera
Medial
canthus
Septum
Optic nerve
Superior
canaliculus
Lateral
canthus
Limbus
Nasolacrimal
sac
Septum
Iris
Cilia
Inferior oblique muscle
Nasolacrimal
duct
Maxilla
B
Orbital septum
Lens
Inferior
punctum
Inferior
canaliculus
Ethmoidal labyrinth
Common
canaliculus
Rectus muscles
Optic nerve
Globe
Orbital fat
Bony margins
of orbit (lamina
papyracea)
Sphenoid sinus
Optic foramen
FIGURE 115-3. Anatomic diagram of the eye and the adnexa.
Temporal
lobe
FIGURE 115-1. A and B. Orbital anatomy. (Reproduced with permission from
Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006, McGrawHill, New York, Figure 8-13.)
Rosenbaum near vision card or the Allen reduced picture cards, held at
16 in. (41 cm) distance from the eyes to check VA. Record which chart
was utilized and the distance at which it was tested.
By 3 to 6 months, a baby should be able to fixate and follow an object
at 1 to 2 ft away. For children from approximately 3 months to 3 years of
age, a brightly colored object, light source, or moving toy can be used to
attract their attention and see if they follow it with both eyes, then each
eye individually, with the opposite eye occluded.
Check acuity in the nonaffected eye first to get an idea of what to expect
from the involved eye. Unless a history of amblyopia is elicited, any twoline or greater discrepancy between eyes should be referred for follow-up.
If the child holds his or her hand to cover the eye not being tested, make
sure to use the palm and not the fingers, so as not to “peek” or put pressure on the globe. It is better to cover the eye not being tested with a solid
occluding device. If a child wears glasses for distance vision, then test VA
with prescription lenses. If they are not available, then use a pinhole occluder. If the child is unable to read the top line of an eye chart, then hold
up one or more fingers and ask how many fingers are visible. If the child
is unable to count fingers, then move your hand from side to side and ask
if the child sees motion. Document these responses with the distance your
Retina
Bulbar
conjuctiva
Choroid
Sclera
Tarsal
conjuctiva
Iris
Eyelid
Levator palpebrae
superioris
Superior oblique
Superior rectus
Medial rectus
Cornea
Optic disc
Trochlea
Filtration
angle
Ciliary
body
Optic nerve
Inferior
oblique
Lateral rectus
Vitreous
Lens
Inferior rectus
FIGURE 115-2. Extraocular muscles of the eye.
FIGURE 115-4. Horizontal cross-sectional diagram of the eye.
CHAPTER 115: Eye Problems in Infants and Children
hand is from the patient (i.e., “count fingers at 2 ft” or “hand motion at 3
ft”). If the child is unable to see motion, then shine a bright light into the
eye and document “light perception” or “no light perception.”
EXAMINATION OF PUPILS AND FUNDUS
Depending on the age and personality of the child, it may be possible to
examine the child sitting on a parent’s lap. Infants, toddlers, mentally
disabled, and uncooperative children may need to be swaddled in a
sheet. Some patients may require an eyelid retractor to examine the eye.
One can use a standard vein retractor or an eyelid speculum.
Document pupil diameter in ambient light. The full-term newborn
cornea is closer to its adult size than most other organs. The horizontal
diameter ranges from 9.0 to 10.5 mm versus 10.5 to 13.0 mm in an adult.1
When checking pupil size, have the child focus on a distant object to exclude accommodative miosis and record whether the size was obtained
in bright, ambient, or dark illumination. If the difference between pupils
is 0.5 mm or greater, then anisocoria exists. This may be “physiologic
anisocoria” with no clinical significance. A greater size discrepancy in a
dark room when compared with a light room indicates physiologic
anisocoria. Note if both pupils look black in ambient light or if leukocoria is present (white appearance of pupil). Leukocoria may be indicative
of problems with the cornea, lens, or anterior or posterior chamber.
Next check the direct and consensual light reflexes. If the child is old
enough to cooperate, have him or her look at a distant object in the
room, such as a brightly colored picture attached to a wall, or have a
parent hold up two fingers while standing at the end of the room and
direct the child’s attention to the parent. Look at the patient’s right eye
and shine a light source into the right pupil. If the right pupil constricts,
this is a normal direct response. Remove the light from the right eye and
focus your attention on the left pupil. Again shine the light into the right
eye and observe the left pupillary response. If the left pupil constricts,
this is a normal consensual response. Next swing the light over to the
left pupil. The left pupil should remain constricted in response to the direct light exposure. If the left pupil dilates in response to direct light, a
positive relative afferent pupillary defect is present. Repeat the procedure with the left eye and document your observations. Some children
will have a small amount of pupillary movement (dilation and contraction) in the consensual eye when the light is swung from the direct eye
over to the consensual eye. This is pupillary escape and can be a normal response.
A bright light source is recommended for children about 5 years or
older. In younger children, a bright light will only cause the child to shut
his or her eyes.
Continuing with the examination, in a dimly lit room, look at the pupils through the direct ophthalmoscope; position yourself at the patient’s
eye level about 1 to 2 ft away from the patient. Note the presence or absence of a red reflex. This may be the extent of the funduscopic examination you will be able to obtain from infants and toddlers. Document the
presence or absence of the red reflex for each eye.
With older children, perform the same funduscopic examination that
you would in an adult.
SPECIFIC PEDIATRIC EYE PROBLEMS
In the following sections, specific eye problems in children are discussed.
Eye injuries and eye emergencies also commonly seen in adults are discussed in Chapter 236, Eye Emergencies.
■ STRABISMUS AND AMBLYOPIA
Strabismus is ocular misalignment. Knowledge of preexisting strabismus
is important to the ED physician to differentiate between congenital and
childhood strabismus and acquired emergent causes of strabismus. Terminology used to describe strabismus is listed in Table 115-1. Normal
newborns may have transient misalignment that usually improves by 3
to 4 months of age as the strength of extraocular muscles improves.1
763
TABLE 115-1 Strabismus Terminology
Term
Description
Esotropia
Exotropia
Hypertropia
Hypotropia
Esophoria
Exophoria
Inward eye deviation
Outward eye deviation
Upward eye deviation
Downward eye deviation
Inward eye deviation when other eye is covered
Outward eye deviation when other eye is covered
Amblyopia is a loss of VA not correctable by glasses in an otherwise
healthy eye. If children have unilateral visual impairment, their brain
will “choose” the image presented by the eye with better vision. If amblyopia is not corrected by about age 10 years old, the brain eventually suppresses visual information presented by the impaired eye, leading to
permanent vision loss. Amblyopia can be caused by unequal refractive
error or from unilateral vision loss caused by entities such as cataracts or
corneal injury. Amblyopia can both cause and be caused by strabismus.
Causes of Strabismus Congenital and childhood strabismus <6 years of
age is only rarely caused by severe neurologic or systemic disease. However,
when new ocular misalignment is reported, or the patient has other symptoms, such as sunsetting of the eyes (upgaze deficit), nausea, vomiting, or
lethargy, determine whether other pathology exists, such as monocular visual impairment, orbital fractures, cellulitis, primary or metastatic tumors,
meningitis, infiltrative processes, or increased intracranial pressure.
Emergent causes of strabismus may lead to entrapped extraocular muscles or cranial nerve palsies. To define which cranial nerve is involved, the
practitioner must be familiar with the innervation of the extraocular muscles. Cranial nerve VI innervates the ipsilateral lateral rectus muscle, and a
palsy will lead to esotropia (inward or nasal deviation of the eye at rest).
Cranial nerve IV innervates the ipsilateral superior oblique muscle, and a
palsy will cause hypertropia. Cranial nerve III innervates all other extraocular muscles, and a palsy will lead to exotropia, hyper- or hypotropia (depending on which branch is affected), and ptosis.
Diagnosis To identify strabismus, several simple tests may be performed
in addition to the normal eye examination. The physician may begin
with a general inspection to determine whether the ocular globes appear
to be in alignment. Some children may have epicanthal folds that lead to
pseudostrabismus or the appearance of ocular misalignment when the
eyes are in fact in proper alignment (Figure 115-5).
Hold a penlight several feet from the child and observe the reflection
of the light on each cornea. In normal ocular alignment, the light reflection will appear on the same position of each eye. Finally, a cover test can
be performed to diagnosis strabismus. During the cover test, have the
child fixate on a distant object. Then cover one of the child’s eyes. If the
uncovered eye then moves, it can be assumed that it was not properly fixated on the object and strabismus is present. When the eyes are uncovered, they will revert to their original misalignment. Misalignment can
further be described as a “tropia,” or constant misalignment, or a “phoria,” or intermittent misalignment (Table 115-1). If a “phoria” is suspected, both eyes must be tested. With a “phoria,” the eyes will revert to
normal (equal) alignment when both eyes are uncovered.
If an emergent cause of strabismus is suspected, obtain imaging. A noncontrast head CT scan may demonstrate findings suggestive of increased
intracranial pressure. An orbital CT scan will demonstrate ocular infiltrative processes, including tumors, abscesses, and cellulitis. If a cause is not
found on CT scan, MRI may be required to evaluate the cranial nerve roots
and brainstem. Obtain urgent subspecialty consultation as indicated.
Congenital strabismus caused by extraocular muscle weakness usually
self-resolves in infancy. Stable strabismus that persists into childhood
should be referred nonemergently to an ophthalmologist. Strabismus
caused by amblyopia often resolves with glasses that equalize the vision,
although a patch over the “good” eye may be required. Surgical correction may be required if glasses and patch fail.
764
SECTION 12: Pediatrics
FIGURE 115-6. Dacryocystitis. (Reproduced with permission from Knoop K, Stack
L, Storrow A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, New
York, Figure 2-10.)
FIGURE 115-5. Pseudostrabismus.
■ LACRIMAL SYSTEM PROBLEMS
Tears are produced in the lacrimal gland and drain at the medial aspect
of the eye through the nasolacrimal duct and lacrimal sac through the
canalicular system to the Hasner valve and finally into the nose. Several
common problems seen in pediatrics can arise in the gland and canalicular system (Table 115-2).
Dacryostenosis Dacryostenosis is a narrowing or obstruction of the nasolacrimal duct, most commonly diagnosed in neonates. It can be unilateral or bilateral. A watery discharge may build up as tears accumulate
and appear thicker and yellowish in color. An important clinical feature
is that there are no other accompanying signs or symptoms, such as fever
and irritability. A child with dacryostenosis does not have erythema of
the conjunctiva or irritation of the surrounding soft tissues.
Treatment of dacryostenosis is supportive. Parents are taught gentle
massage with a downward motion to the nasolacrimal duct three to four
times a day. Dacryostenosis will often resolve as infants grow larger.
However, if still present at 6 to 12 months of age, ophthalmology referral
is indicated for possible dilation of the duct.
Dacryocystitis Inflammation and bacterial superinfection of the lacrimal
sac will cause dacryocystitis. Common pathogens are Streptococcus pneu-
TABLE 115-2 Lacrimal Duct Problems
Problem
Definition
Location
Dacryostenosis
Dacryocele
Narrowing or obstruction of
nasolacrimal duct
Infection of nasolacrimal
duct
Nasolacrimal duct cyst
Dacryoadenitis
Infection of lacrimal gland
Between medial canthus and
nasal bridge
Between medial canthus and
nasal bridge
Between medial canthus and
nasal bridge
Superior-temporal to globe
Dacryocystitis
moniae, Staphylococcus aureus, S. epidermidis, Haemophilus influenzae,
and S. agalactiae. Infants develop a chronic mucopurulent discharge followed by erythema and swelling inframedially to the eye (Figure 115-6).
In children of all ages, dacryocystitis is usually a sequal of bacterial superinfection after a viral upper respiratory infection. The diagnosis is
made when gentle pressure with a finger tip or cotton swab applied to the
nasolacrimal sac causes a reflux of mucopurulent material. Culture the
discharge to identify the causative agent.
Improperly treated dacryocystitis may lead to periorbital and orbital cellulitis. Parenteral antibiotic therapy is required for ill- or toxicappearing children. A cephalosporin, such as cefuroxime (50 milligrams/kg IV every 8 hours) or cefazolin (33 milligrams/kg IV every 8
hours), may be used, or clindamycin (10 milligrams/kg IV every 6 hours)
for penicillin-allergic patients.2 If methicillin-resistant S. aureus (MRSA)
is suspected, vancomycin (10 to 13 milligrams/kg IV every 6 to 8 hours)
may be required.2
Dacryocele Dacryocystitis and dacryostenosis should be differentiated
from a dacryocele. A dacryocele is a small, bluish-hued, palpable mass in
the location of the nasolacrimal duct (inferior and medial to the eye)
without conjunctival erythema, discharge, or other pathologic findings.
Patients should be referred to a pediatric otolaryngologist or ophthalmologist because of the association with obstructive intranasal cysts.
Dacryoadenitis Dacryoadenitis, inflammation or infection of the lacrimal gland, can be acute or chronic. Chronic dacryoadenitis is caused by
noninfectious inflammatory disorders such as Sjögren syndrome, sarcoidosis, or thyroid disease. Acute dacryoadenitis is usually infectious.
In both acute and chronic disease, children will have soft tissue swelling,
especially in the region of the lateral upper lid. With infectious causes,
systemic symptoms such as malaise and fever may also be present.
Viral dacryoadenitis causes less intense discomfort and erythema than
bacterial dacryoadenitis. Causative viral pathogens include Epstein-Barr
virus, mumps virus, coxsackievirus, cytomegalovirus, and varicella-zoster
virus. Bacterial dacryoadenitis is associated with intense eye discomfort,
tenderness, and erythema. The most common causative bacterial pathogen
causing dacryoadenitis is S. aureus, but streptococci, Neisseria gonorrhea,
Chlamydia trachomatis, and Brucella melitensis have also been implicated.
The first-line treatment of bacterial dacryoadenitis is PO or IV antibiotics against S. aureus. For mild infections, a PO first-generation cephalosporin, such as cephalexin (25 milligrams/kg PO every 6 hours) until the
infection has resolved, is appropriate.2 If MRSA is suspected, sulfamethoxazole-trimethoprim [20 milligrams/kg PO (or IV) every 12 hours] or line-
CHAPTER 115: Eye Problems in Infants and Children
765
zolid [10 milligrams/kg PO (or IV) every 12 hours] may be used.2 For
more severe infections, parenteral antibiotic therapy is indicated. Nafcillin
(37.5 milligrams/kg IV every 6 hours) is appropriate when MRSA is not
suspected.2 Vancomycin (10 to 13 milligrams/kg IV every 6 to 8 hours) is
indicated for severe dacryoadenitis caused by MRSA.2
■ PERIORBITAL AND ORBITAL CELLULITIS
Periorbital, or preseptal, cellulitis must be distinguished from orbital, or
postseptal, cellulitis. The orbital septum is a connective tissue extension of
the orbital periosteum that extends into the upper and lower eyelids and
acts as a barrier to the spread of infection. Spread of infection may be facilitated by the valveless drainage system of the midface region. Because there
are no valves, bacteria can travel hematogenously in an anterograde fashion (i.e., away from the heart) despite a retrograde venous system.3
Periorbital Cellulitis The average age of presentation with periorbital
cellulitis is 2 years old. Periorbital cellulitis can be caused by local infection, hematogenous spread, and extension of sinusitis.
Local infection, such as conjunctivitis, dacryoadenitis, dacryocystitis,
hordeolum, or even a minor traumatic cellulitis after an insect bite or small
scratch, can spread to the periorbital area (Figure 115-7). The most common bacterial pathogens are S. aureus and group A Streptococcus.
Hematogenous spread of nasopharyngeal pathogens can also lead to
significant periorbital cellulitis. Affected children tend to be younger (often <18 months old) and have a history of a viral upper respiratory infection followed by abrupt onset of fever and eyelid swelling. Before the use
of the H. influenzae type b vaccine, the most common bacterial pathogens were H. influenzae (80%) and S. pneumoniae (20%). Now the most
common pathogens are S. pneumoniae and S. pyogenes.4
Finally, periorbital cellulitis may be the result of acute sinusitis. Sinusitis can be associated with reactive edema and mild inflammation of
the eyelids noted upon awakening that regresses during the day as dependent edema resolves. Unilateral periorbital edema that does not regress may indicate cellulitis. Bacteria that cause sinusitis, such as S.
pneumoniae, nontypeable H. influenzae, and Moraxella catarrhalis, are
the most common pathogens for this type of periorbital cellulitis.
Clinical Features and Diagnosis Periorbital cellulitis is characterized by an
erythematous, tender, indurated, swollen eyelid and periorbital area
(Figure 115-8). There is no associated decrease in VA, conjunctival injection, impairment of extraocular movements, proptosis, pain with
eye movement, or other intraorbital pathology. It may not be possible
to always differentiate clinically between an insect bite near the eye, a mild
FIGURE 115-7. Hordeolum with preseptal cellulitis. (Reproduced with permission
from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006,
McGraw-Hill, New York, Figure 8-10.)
FIGURE 115-8. Periorbital (preseptal) cellulitis. (Reproduced with permission
from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006,
McGraw-Hill, New York, Figure 8-9.)
allergic reaction, and early periorbital cellulitis. Children with moderate to
severe periorbital cellulitis may have difficulty opening their eyelids for the
examiner, so eyelid retractors maybe required to fully examine the eye and
its movements. A CT scan of the orbits may be required to differentiate between periorbital cellulitis and the often more severe but less common orbital cellulitis.
Treatment, Disposition, and Follow-Up Children who are well appearing
and are afebrile are candidates for outpatient oral antibiotic therapy.
Amoxicillin clavulanate (20 milligrams/kg PO twice a day) is an appropriate oral therapy. Those with more severe periorbital cellulitis or in whom
hematogenous spread is suspected require parenteral therapy and hospitalization. Cefuroxime (50 milligrams/kg IV every 8 hours), ceftriaxone
(50 milligrams/kg IV every 12 hours), or ampicillin-sulbactam (50 milligrams/kg IV every 6 hours) are appropriate choices. Add vancomycin if
MRSA is suspected.
Orbital Cellulitis Orbital cellulitis is usually an extension of a sinus infection into the orbit behind the septum. The average age of presentation is
12 years old. Complications include sub-periosteal abscess, orbital abscess, cavernous sinus thrombosis, panophthalmitis, or endophthalmitis.
The most common bacterial pathogens are S. pneumoniae, H. influenzae,
M. catarrhalis, S. aureus, S. pyogenes, and anaerobic upper respiratory flora such as Bacteroides and Fusobacterium species.
Clinical Features and Diagnosis Orbital cellulitis is characterized by erythema and swelling around the eye. Suspect orbital cellulitis if the eyelid or periorbital inflammation is accompanied by any of the
following: proptosis, impaired extraocular movements, pain with eye
movement, decreased VA, chemosis, or an afferent pupillary defect
(Figure 115-9). Fever may or may not be present.5
Diagnosis of orbital cellulitis is primarily clinical, but an orbital and sinus CT can differentiate between periorbital and orbital cellulitis as well
as delineate any concomitant abscess or other pathology. Culture of the
blood, nares, and conjunctiva may be obtained to help identify the bacterial source. After neuroimaging, consider lumbar puncture for symptoms
such as headache, lethargy, neurologic symptoms, or toxic appearance to
exclude associated meningitis.
Treatment, Disposition, and Follow-Up Orbital cellulitis requires inpatient
management. Consult otolaryngology and ophthalmology for evaluation and possible surgical drainage of abscesses. Parenteral therapy is indicated until significant clinical improvement is noted, followed by oral
766
SECTION 12: Pediatrics
FIGURE 115-9. A and B. Orbital cellulitis. (Reproduced with permission from
Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006, McGrawHill, New York, Figure 8-14.)
antibiotics to complete a 3-week course. Cefuroxime (50 milligrams/kg
IV every 8 hours) or ampicillin-sulbactam (50 milligrams/kg IV every 6
hours) are appropriate first-line antibiotics. If cefuroxime is used or if an
anaerobic infection is strongly suspected, add clindamycin (10 milligrams/kg IV every 6 hours). Also add vancomycin for life-threatening
infections or suspected MRSA.
■ THE RED EYE
There are many problems that may produce a red eye in a child. In this
section, we focus on the most common pediatric complaints, corneal
abrasion and conjunctivitis, then discuss other pertinent pediatric problems, including Kawasaki disease and pediculosis.
Corneal Abrasion Corneal abrasion is discussed in greater detail in
Chapter 236, Eye Emergencies. However, because corneal abrasions frequently occur in children, a brief description is included here.
Clinical Features Corneal abrasions in older children are characterized by
a foreign body sensation, pain, photophobia, injection, and a history of
direct trauma, ultraviolet light exposure, or pain from windblown particulate matter in the eye. Smaller children and infants often lack a history
of trauma to the eye and may present with a chief complaint of inconsolable crying and an otherwise normal physical examination. If instillation
of a numbing drop such as proparacaine 0.5% or tetracaine 0.5% onto
the surface of the eye calms the child, it strongly suggests that injury to
the surface of the eye may be the source of the child’s distress.
Diagnosis and Treatment A thorough eye examination with fluorescein
will confirm the diagnosis of corneal abrasion. An abrasion will fluoresce
to a yellow-green color under a black light or cobalt blue filter in the slit
lamp or Wood lamp. The presence of a vertical linear abrasion suggests
the presence of a retained foreign body, and the upper eyelid should be
everted and the superior conjunctiva examined.
Treatment of a corneal abrasion is erythromycin ophthalmic ointment to help avoid superinfection and provide lubrication. Ciprofloxacin or ofloxacin ophthalmic solutions are safe in children >1 year old.
Avoid topical steroids and ointments containing neomycin. Cyclopentolate 1% drops may alleviate pain by reducing ciliary spasm. Occasionally nonsteroidal anti-inflammatory drops are also used [such as
ketorolac (Acular®)], but this has only been studied in adults. Eye patching is not routinely recommended but may be useful for any child who
frequently attempts to scratch or rub the injured eye. Although the need
for tetanus prophylaxis of a corneal abrasion is debatable, the ED visit
should be used to remind the caregiver to check the child’s tetanus status with the primary care provider. In a tetanus-prone injury, such as a
scratch by a pet that plays outside, updating an unknown tetanus status
is recommended.
For uncomplicated and simple corneal abrasions, follow-up in 48
hours with the pediatrician or other primary care provider is suggested.
Children with large abrasions or involvement of the visual axis should
follow up the next day with ophthalmology. Children or adolescents who
use contact lenses or have a history of herpes should also be evaluated by
an ophthalmologist. Contact lenses should not be worn until all symptoms have resolved. Corneal abrasions heal quickly, and patients who
continue to have a foreign body sensation 2 to 3 days after initial presentation require urgent ophthalmologic reevaluation.
Ophthalmia Neonatorum Ophthalmia neonatorum is conjunctivitis in
neonates up to 30 days old. The five primary categories of neonatal
conjunctivitis are chemical, gonococcal, chlamydial, other bacterial
(nongonococcal/nonchlamydial), and viral. Gonococcal, chlamydial,
and viral neonatal conjunctivitis can all lead to severe morbidity. Although specific diagnoses and treatments are discussed in the following
sections, in the ED it may not be possible to determine the specific etiology. In the case of gonococcal conjunctivitis, infants born in a hospital
receive antibiotic prophylaxis, and so gonococcal infection would be excluded, or Gram stain of the exudate should identify the gram-negative
diplococci. Herpes conjunctivitis is suggested by a maternal history of
herpes virus infection, a positive fluorescein examination, and mucocutaneous herpetic lesions. Chlamydial conjunctivitis cannot be excluded
in the ED, so 14 days of systemic erythromycin (see Chlamydial Ophthalmia Neonatorum) should be administered until a conclusive diagnosis is made.
Chemical Ophthalmia Neonatorum Historically, newborns were given silver nitrate ointment prophylaxis to protect against chlamydial and gonococcal conjunctivitis. This ointment in particular often caused a bilateral
chemical conjunctivitis, bilateral inflamed eyelids, and a watery discharge.
Gram stain of the discharge would reveal the absence of pathologic bacteria and only a few white blood cells. Now that erythromycin ophthalmic
ointment is almost exclusively used, chemical conjunctivitis is much less
common. Treatment of neonatal chemical conjunctivitis is watchful
waiting. Symptoms should resolve within 48 hours.
CHAPTER 115: Eye Problems in Infants and Children
767
FIGURE 115-10. Gonococcal ophthalmia. (Reproduced with permission from
Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006, McGrawHill, New York, Figure 8-1.)
Gonococcal Ophthalmia Neonatorum Erythromycin ophthalmic ointment
prophylaxis is used at birth to diminish the risk of conjunctivitis caused by
N. gonorrhoeae in infants born to infected mothers. Gonococcal conjunctivitis usually presents at 2 to 7 days of life with intense bilateral bulbar
conjunctival erythema, chemosis, and a copious purulent discharge
(Figure 115-10). The diagnosis is made by Gram stain, revealing gramnegative diplococci, and culture using chocolate agar. All infants with
gonococcal conjunctivitis require admission and evaluation for disseminated disease.6 Testing of blood, urine, cerebrospinal fluid, and any other
sites with suspected infection is recommended. Therapy for isolated conjunctivitis in a neonate without hyperbilirubinemia is a single dose of
parenteral ceftriaxone (50 milligrams/kg IV; maximum, 125 milligrams). To avoid exacerbation of hyperbilirubinemia or if disseminated
infection is suspected and longer-term antibiotics are required, use cefotaxime (50 milligrams/kg IV every 8 hours). Irrigate the infant’s eyes with
normal saline frequently to eliminate the purulent discharge. Gonococcal
ophthalmia neonatorum may progress to ulceration and perforation of the
cornea if improperly treated.
Chlamydial Ophthalmia Neonatorum Symptoms of chlamydial conjunctivitis present slightly later than those caused by gonorrhea, typically around 5
to 14 days of age. Signs are unilateral or bilateral purulent discharge with intense erythema of the palpebral conjunctiva (Figure 115-11). Chlamydial
ophthalmia is associated with chlamydial pneumonia. Diagnosis is confirmed with Giemsa stain, culture, or nucleic acid amplification of conjunctival scrapings. Treatment of chlamydial conjunctivitis with or without
pneumonia is a 14-day course of erythromycin base or ethylsuccinate (12.5
milligrams/kg PO every 6 hours). A second course of erythromycin may be
required if full resolution is not achieved. Topical antibiotics are unnecessary and insufficient. Patients with isolated chlamydial ophthalmia who do
not have respiratory symptoms or evidence of pneumonia may be safely
discharged to home with follow-up in 24 hours.
Other Bacterial Neonatal Ophthalmia Neonatorum Bacterial conjunctivitis
due to bacteria other than chlamydia and gonorrhea is also less common
when erythromycin topical prophylaxis has been given.6 The most common bacterial pathogens are S. aureus, nontypeable H. influenzae, and
FIGURE 115-11. A and B. Chlamydial ophthalmia. (Reproduced with permission
from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006,
McGraw-Hill, New York, Figure 8-2.)
S. pneumoniae. Symptoms are variable and usually begin within 2 weeks
of birth with hyperemia, purulent discharge, and edema. Gram stain and
culture will identify the cause. Parenteral or oral therapy is not necessary
in almost all cases, and topical therapy with bacitracin, polymyxin, or
neomycin ointment is sufficient.
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SECTION 12: Pediatrics
Viral Ophthalmia Neonatorum Viral neonatal ophthalmia, caused by herpes simplex types I and II, represents 1% of all neonatal conjunctivitis.
Because there is a significant risk of keratitis and devastating disseminated infection, early identification and treatment is critical. Symptoms develop at 6 to 14 days of life with bilateral lid edema and conjunctival
erythema. Suspect herpes infection in a neonate with conjunctivitis, especially with associated mucocutaneous lesions and a maternal history
of herpes. However, not all neonates with perinatal herpes infections
have a history of maternal infections, so a high index of suspicion is required to make the diagnosis. Herpes conjunctivitis is confirmed with
the presence of keratitis or corneal dendrites on fluorescein examination
and viral culture or nucleic amplification tests. Neonates with suspected
herpetic ophthalmia require hospital admission, full sepsis evaluation
(including lumbar puncture with herpes polymerase chain reaction testing of cerebrospinal fluid), IV acyclovir (20 milligrams/kg IV every 8
hours for 14 to 21 days), and topical antivirals (1% trifluridine, 0.1% iododeoxyuridine, or 3% vidarabine).6
Childhood Conjunctivitis Conjunctivitis is very common in children and
may be caused by viruses, bacteria, allergy, or, less commonly, as a symptom of a systemic disease. Each type of conjunctivitis is discussed in the
following sections.
Viral Conjunctivitis Viral conjunctivitis in childhood is most frequently
caused by adenovirus. Less frequent pathogens are rhinovirus, enteroviruses, influenza, and Epstein-Barr virus. Conjunctivitis caused by the
herpes viruses requires immediate proper therapy because of the danger
of permanent vision loss. Measles virus can also cause conjunctivitis, but
is seen less commonly in the U.S. because of the childhood immunization schedule.
Viral conjunctivitis has several distinct presentations. Pharyngoconjunctival fever presents with fever, acute onset of conjunctivitis, pharyngitis, and preauricular adenopathy. Epidemic keratoconjunctivitis can
present with pain, photophobia, subepithelial defects, and pseudomembranes over the conjunctiva. Follicular conjunctivitis often presents with a
foreign body sensation and erythema of the conjunctiva. On examination,
an aggregation of lymphocytes around networks of blood vessels in the
conjunctiva will give the appearance of follicles. Finally, acute hemorrhagic conjunctivitis presents with hyperemic conjunctiva, subconjunctival
hemorrhages, chemosis, swelling, photophobia, and pain (Figure 115-12).
The treatment of these categories of viral conjunctivitis is supportive
only. Cool compresses may offer patients symptomatic relief. Artificial
tears and topical vasoconstrictors may improve redness and the sensation of dryness. Topical antibiotics are sometimes prescribed to prevent
secondary infection. Symptoms may last >1 week, and viral conjunctivitis is very contagious. Families should not share face cloths, towels, or
pillows.
Conjunctivitis Caused by Herpes Viruses Conjunctivitis caused by varicella
most often occurs during primary infections. However, it can also occur
with herpes zoster ophthalmicus, which is when the varicella virus lies
dormant in the trigeminal nerve and causes recurrent vesicles in the V1
distribution. Herpes virus 1 may also present similarly with unilateral
vesicles in the same distribution. A typical dendritic pattern will be seen
on the cornea with fluorescein examination.
Both primary and secondary varicella conjunctivitis presenting in the
first 72 hours of symptoms should be treated with oral acyclovir (for age
>2 years old: 20 milligrams/kg PO every 6 hours for 5 days; maximum
dose, 3200 milligrams/d) and require an ophthalmology consultation.
Similarly, herpes simplex virus type 1 infections of the eye in children
also require ophthalmology consultation but may be treated with topical
antivirals such as trifluridine, iododeoxyuridine, or vidarabine.6 Topical
steroids may be prescribed by an ophthalmologist for both varicella and
herpes simplex virus type 1 infections of the eye but should not be prescribed by the emergency physician.
Childhood Bacterial Conjunctivitis Bacterial conjunctivitis in childhood is
most frequently caused by Hemophiles species, S. pneumoniae, M. catarrhalis, and S. aureus. Less common pathogens include Pseudomonas
aeruginosa, group B Streptococcus, Escherichia coli, and N. meningitidis.
FIGURE 115-12. A and B. Adenoviral conjunctivitis. (Reproduced with permission from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006,
McGraw-Hill, New York, Figure 8-3.)
Oculoglandular syndrome is a rare infection often caused by Bartonella
henselae (cat scratch disease) or tularemia, which causes ipsilateral conjunctivitis and lymphadenopathy, often axillary. Bacterial conjunctivitis
usually begins in one eye and becomes bilateral in 2 to 5 days. Signs are
normal vision, mucopurulent matting of the lashes (especially after sleep),
and eyelid edema. Photophobia and eye pain are not present, although pa-
CHAPTER 115: Eye Problems in Infants and Children
tients will have some discomfort. Consider chlamydial and gonococcal
conjunctivitis in the differential diagnosis, especially in adolescents.
The diagnosis of bacterial conjunctivitis is primarily clinical. If patients
have concomitant otitis media, the diagnosis is most likely conjunctivitisotitis syndrome caused by nontypeable H. influenzae, and oral antibiotics
should be given. Otherwise, patients should receive a broad-spectrum topical antibiotic such as a fluoroquinolone (ciprofloxacin or ofloxacin ophthalmic), bacitracin-polymyxin, or trimethoprim-polymyxin. An ointment
is preferable to eye drops. Although erythromycin ointment is inexpensive, it does not provide adequate coverage for H. influenzae or M. catarrhalis. Therefore, if erythromycin ointment has been prescribed and the
patient is not clinically improving, a change of topical antibiotic ointment
is likely indicated.
Patients with isolated bacterial conjunctivitis or conjunctivitis-otitis
syndrome may be safely discharged to home. Symptoms that do not improve after 7 days of therapy merit ophthalmology referral.
Allergic Conjunctivitis Children with a history of atopy are most likely to
suffer allergic conjunctivitis, but almost any child can be affected. Children with allergic conjunctivitis may have bilateral itchy eyes, tearing,
thin mucoid discharge, mild redness and eyelid edema, as well as chemosis. In severe cases, patients may have mild photophobia. Treatment of
allergic conjunctivitis centers on allergen avoidance, topical antihistamines, and mast cell stabilizers. Ketotifen (1 drop to each eye every 8 to
12 hours) and olopatadine (1 to 2 drops to each eye daily), which are
both antihistamines and mast cell stabilizers, are very effective.7,8 Topical NSAIDs, such as ketorolac, and topical vasoconstrictors may provide
symptomatic relief. Oral antihistamines are discouraged because they
can cause eye dryness, exacerbating symptoms.
Other Causes of Childhood Conjunctivitis Although almost all pediatric conjunctivitis is due to bacterial, viral, or allergic causes, a differential diagnosis of the red eye should include iritis, keratitis, uveitis, glaucoma,
corneal abrasion, Kawasaki disease, and pediculosis. Kawasaki disease,
glaucoma, and pediculosis of the eyelashes are discussed in the following
sections.
Kawasaki Disease Kawasaki disease, a severe vasculitis that can cause coronary artery aneurysms, predominantly presents in children 1 to 8 years
of age (see Chapter 134, Rashes in Infants and Children). Nonpurulent
bilateral conjunctivitis is a key diagnostic feature of Kawasaki disease. In
typical cases, patients will have a fever (>5 days), dry and erythematous
lips and oropharynx, enlarged cervical lymph node (>1.5 cm), nonvesicular rash, edema, or peeling of the hands and feet. A diagnosis of Kawasaki disease requires inpatient admission, IV gamma globulin, aspirin
therapy, cardiology consult, and, in most institutions, either infectious
disease or rheumatology consultations.
Pediatric Glaucoma Pediatric glaucoma is an important worldwide cause
of visual impairment. It is the result of an abnormality of the trabecular
network and can be classified as either primary or secondary. Primary
pediatric glaucoma is the result of a developmental abnormality. Dysgenesis of the chamber angle leads to decreased outflow of aqueous humor and increased intraocular pressure. With secondary glaucoma,
aqueous outflow is diminished by systemic disease, scarring, inflammation, or infection. Pediatric syndromes associated with glaucoma include
Sturge-Weber syndrome, Lowe syndrome, Down syndrome, neurofibromatosis, and maternal rubella syndrome.
Primary pediatric glaucoma is more common than secondary in children. Estimates of incidence range from 1 in 10,000 to 1 in 18,500
births.9,10 Approximately 75% of cases are bilateral, and 80% of cases will
present before 1 year of age. Primary pediatric glaucoma is often familial,
passed through generations by autosomal recessive inheritance.
Ideally, pediatric glaucoma is diagnosed with a good slit lamp examination and tonometry. Performing an eye examination on a young child
in the ED, however, can be very difficult. Therefore, it is important to be
familiar with the presenting signs of pediatric glaucoma so that proper
ophthalmologic consultation and referral may be made.
Children with pediatric glaucoma will have a number of pathologic
ocular signs and symptoms. Intraocular pressure will be elevated (≥20
769
FIGURE 115-13. Infantile glaucoma. (Reproduced with permission from Shah BR,
Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006, McGraw-Hill, New
York, Figure 8-32.)
mm Hg). The cornea will appear enlarged, and, often, cloudy and edematous (Figure 115-13). Children <1 year of age should not have a corneal diameter >12 mm, and no child should have a corneal diameter
>13 mm.11 There is associated blepharospasm, red infected eye, and myopia. The globe may appear enlarged. If the optic disc can be visualized,
abnormal cupping or asymmetry may be found.
Medical therapy of pediatric glaucoma is temporizing to decrease intraocular pressure while awaiting definitive surgical repair. Acetazolamide
(3 milligrams/kg, PO every 6 hours) can be used for short periods of time
but may cause metabolic acidosis. Pediatric doses are prepared by crushing the tablets used for adults. Topical carbonic anhydrase inhibitors such
as dorzolamide or brinzolamide may also be used with less systemic side
effects. In children without a history of asthma, topical β-blockers such as
timolol may be used. Because children will have more systemic absorption
than adults, the recommended starting dose is as low as one drop daily of
the 0.25% solution. Surgery is required for definitive treatment.
Pediculosis Pediculosis infestations are caused by three types of louse:
Pediculosis humani capitis (head louse), pediculosis humani corporis
(body louse), and Pthirius pubis (pubic louse). All three types feed on
human blood and have specialized claws that allow them to cling onto
hair and clothing. Lice can infest the eyelashes of a child of any age, leading to itching and scratching, with a mild conjunctivitis caused by the
louse’s saliva. Occasionally, by scratching, children can cause a secondary bacterial infection or corneal abrasion.
Do not use pediculicide shampoos to treat pediculosis of the eyelashes, as the shampoo is toxic to the eyes. Rather, after attempts at removal of nits (eggs), smother the lice with petroleum jelly or other
ophthalmic ointment three times a day. Although the head and body
louse may frequently involve the eyelashes of children, if Pthirus pubis is
identified, consider sexual abuse.
■ LEUKOCORIA
Leukocoria is a white-appearing pupil (Figure 115-14). Normally when
a bright light is directed at the pupil, a “red reflex” appears as the light is
reflected off the retina. With leukocoria, the red reflection is blocked.
Despite routine screening during pediatric well-child examinations, leu-
770
SECTION 12: Pediatrics
FIGURE 115-14. Leukocoria (arrow). (Reproduced with permission from Shah
BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006, McGraw-Hill, New
York, Figure 8-19.)
kocoria is frequently discovered by parents after a photograph with flash
photography is noticed to have unequal “red-eye.”12 This is possibly because during the well-child examination, leukocoria can be hidden by
rapid pupillary constriction.
Leukocoria has many causes, as listed in Table 115-3. Of patients age 0
to 10 years old presenting with leukocoria, up to 60% will have congenital
cataracts and 18% will have retinoblastoma.13 Leukocoria requires prompt
emergency evaluation by an ophthalmologist to avoid loss of vision or life.
■ CATARACTS
Cataracts are one of the most common causes of childhood visual impairment.14 They are also the most frequent nonmalignant cause of leuTABLE 115-3 Causes of Leukocoria in Children
Congenital cataracts
Retinoblastoma
Retinal detachment
Retinoschisis
Retinopathy of prematurity
Falciform retinal fold
Persistent hyperplastic primary vitreous
Coats disease
Retinal coloboma
Endophthalmitis or panophthalmitis
Posterior uveitis
Vitreous hemorrhage
High myopia
Sarcoma
Corneal opacity
Phakomatosis
Other tumors
Ocular larva migrans
kocoria. Although cataracts are not treated in the ED, it is important for
the ED physician to recognize and refer children with cataracts promptly
to avoid permanent visual loss.
Pediatric cataracts can be unilateral or bilateral. Unilateral cataracts
have a higher association with other ocular abnormalities and may be idiopathic (92%), hereditary (6%), or secondary to infection or other perinatal injury (2%). Bilateral cataracts on the other hand are more frequently
hereditary (56%), and less commonly idiopathic (38%) or from infection
or other perinatal injury (6%).15 Hereditary cataracts are usually an autosomal dominant trait but can also be autosomal recessive. Cataracts recognized in the neonatal period are most frequently from TORCH infections
(toxoplasmosis, syphilis, rubella, cytomegalovirus, and herpes simplex).
Cataracts may also be associated with a variety of systemic illnesses and
syndromes such as trisomy 21, galactosemia, Lowe syndrome, Alport syndrome, homocystinuria, Wilson disease, and many more.
Children with cataracts present with any one of the following: leukocoria (Figure 115-15), strabismus, and/or nystagmus. On direct examination or examination using a slit lamp, lens opacities will be seen that
can have partial or complete lens involvement, obscuring the retina.
Cataracts require referral to an ophthalmologist. The ophthalmologist
will consider testing TORCH titers, blood sugar, red blood cell galactose1-phosphate (for galactosemia), Venereal Disease Research Laboratory
(for syphilis), urine proteins (for Alport syndrome), homocystine, urine
amino acids (Lowe syndrome), copper levels (Wilson disease), and karyotype (for trisomy 21 and other trisomies). Although there are a wide variety of surgical techniques used, generally, central cataracts that are >3
mm warrant surgical removal. Children >2 years old may benefit from
intraocular lens placement. Patients may also require surgery to repair
strabismus.
■ RETINOBLASTOMA
The most common presenting signs of retinoblastoma are leukocoria
and strabismus. Leukocoria in children with retinoblastoma was detected by family and friends in 80% and only 8% by pediatricians and 10%
by ophthalmologists.16 Children may also present with proptosis, retinal
detachment, glaucoma, hyphema, hypopyon, vitreous hemorrhage, and
symptoms that may be confused with orbital cellulitis.
Retinoblastoma is further discussed in Chapter 136, Oncology and
Hematology Emergencies in Children.
■ RETINAL HEMORRHAGES
The incidence of shaken baby syndrome is approximately 29.7 per
100,000 infants.17 Shaken baby victims have subdural hemorrhages and
encephalopathy, as well as extensive retinal hemorrhages in 83% of cas-
FIGURE 115-15. Cataract. (Reproduced with permission from Shah BR, Lucchesi
M: Atlas of Pediatric Emergency Medicine. © 2006, McGraw-Hill, New York, Figure
8-21.)
CHAPTER 116: The Nose and Sinuses
es.18 Because child abuse is discussed elsewhere (see Chapter 290, Child
Abuse and Neglect), this section focuses on ocular pathology.
Retinal hemorrhages appear to be caused by shaking of the infant’s
head and are not sequelae of intracranial injury. One theory suggests that
retinal hemorrhages are the result of an abrupt increase in venous pressure from elevated intracranial pressure or elevated thoracic pressure. A
second theory postulates that the injury is the result of vitreoretinal traction and shearing sustained during the violent acceleration and deceleration of the child’s head. Although the presence of retinal hemorrhages
clinches the diagnosis of child abuse and shaken baby syndrome in most
settings, rarely, victims of a severe head crush injury have had similar
findings.19 Although children may suffer vision loss from ocular pathology, the most common cause of blindness in shaken baby syndrome is
cortical blindness rather than retinal hemorrhage.
Obtain ophthalmologic consultation to detect retinal hemorrhage if
child abuse is suspected.
Acknowledgments: The authors gratefully acknowledge the contributions of Dr. Richard Malley, the author of this chapter in the previous
edition, and Dr. Forrest James Ellis, MD, for his thoughtful review of this
chapter.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
116
The Nose and Sinuses
Joanna Cohen
Dewesh Agrawal
ACUTE BACTERIAL SINUSITIS
Rhinosinusitis is the term used for infections that involve both the nose
and sinuses. Bacterial sinusitis may be an acute, subacute, or chronic infection. Acute bacterial sinusitis is a bacterial infection of the paranasal
sinuses with complete resolution in <30 days. Subacute bacterial sinusitis
is defined by resolution between 30 and 90 days, and chronic sinusitis
lasts >90 days.1 The most common predisposing factor for bacterial sinusitis is a viral upper respiratory infection (URI). The incidence of viral
URIs in children ages 6 months to 35 months is approximately six episodes per patient-year, with approximately 8% of those becoming complicated by acute bacterial sinusitis. Bacterial sinusitis in children is most
common in the 12 to 23 months age group, probably because these children are most likely to be in day care, predisposing them to URIs.2 In
1996, health care costs in the U.S. incurred from treating sinusitis in children <12 years of age had been estimated at $1.8 billion a year.3
771
The most common pathogen of acute bacterial sinusitis is Streptococcus pneumoniae, recovered in 30% of children with acute sinusitis. Nontypeable Haemophilus influenzae and Moraxella catarrhalis are recovered
in 20% of children each.5,6 In addition to the more common pathogens,
chronic sinusitis may also be caused by Staphylococcus aureus, anaerobes,
and, rarely in children, fungus, including Aspergillus, Fusarium, Bipolaris,
Curvularia lunata, and Pseudallescheria boydii.7
■ CLINICAL FEATURES
Children with acute bacterial sinusitis typically present with high fever
and purulent nasal discharge. Headache, particularly behind the eye, is a
variable presenting symptom, whereas complaints of facial pain in children are rare.1 The physical examination findings of acute bacterial sinusitis are often similar to those of uncomplicated viral sinusitis, with
swollen and erythematous turbinates and mucopurulent discharge. However, reproducible unilateral tenderness to percussion or direct pressure
of the frontal or maxillary sinus may indicate acute bacterial infection,
and periorbital edema might indicate ethmoid sinusitis.1 Transillumination of the maxillary sinuses is unreliable in children <10 years of age.8
■ DIAGNOSIS
Although the gold standard for diagnosis of acute bacterial sinusitis is the
recovery of ≥104 colony-forming units/mL of bacteria from the paranasal
sinus,5 sinus aspiration is painful and impractical in the ED. Therefore,
diagnosis is often based on clinical criteria that help to distinguish acute
bacterial sinusitis from an uncomplicated viral URI in an ill-appearing
child (Table 116-1).1
Imaging studies are not needed to confirm a diagnosis of acute bacterial sinusitis in children <6 years of age with persistent symptoms1
because of the high incidence of sinus mucosal abnormalities in patients
with simple upper respiratory symptoms or no clinical symptoms at all.
In one study, mucosal sinus changes were evident in 97% of infants who
had a URI in the 2 weeks preceding a cranial CT done for unrelated reasons.9 Plain films have limited utility because they require correct positioning that is technically difficult in young children, and there is only a
70% to 75% correlation of culture confirmation with abnormal-appearing sinus radiographs.10 A cranial CT with contrast is recommended
for suspected complications of bacterial sinusitis, including preseptal
or postseptal cellulitis, subperiosteal abscess, cavernous sinus thrombosis, osteomyelitis of the frontal bone (Pott puffy tumor), subdural
empyema, epidural or brain abscess, and meningitis.11 The American
Academy of Pediatrics also recommends paranasal sinus CT scans for
patients in whom surgery is being considered.1
■ TREATMENT
Patients with mild symptoms suggestive of a viral infection can be observed for 7 to 10 days, with no antibiotics prescribed. However, if symptoms persist or are severe (Table 116-1), suspect acute bacterial sinusitis
and prescribe antibiotics to speed recovery, prevent suppurative complications, and minimize asthma exacerbations in susceptible children
(Figure 116-1).1
■ PATHOPHYSIOLOGY
The sinuses are air cavities lined with ciliated columnar epithelium that
helps mucus clearance by pushing mucus and debris out of the sinus ostia into the nasal cavity. Blockage of the ostia by mucus and inflammation predisposes to bacterial sinusitis. The ethmoid and maxillary
sinuses are present at birth and are most commonly involved in sinusitis
in children. The sphenoid sinuses form at 3 to 5 years of age. The frontal
sinuses do not appear until 7 to 8 years of age and remain incompletely
pneumatized until late adolescence. The most common predisposing
factors for acute bacterial sinusitis are diffuse mucositis secondary to viral rhinosinusitis in 80% of cases and allergic inflammation in 20% of
cases.4 Less common predisposing factors include nonallergic rhinitis,
cystic fibrosis, dysfunctional or insufficient immunoglobulins (Igs), ciliary dyskinesia, and anatomic abnormalities.5
TABLE 116-1 Clinical Features of Acute Bacterial Sinusitis
Persistent symptoms
Nasal or postnasal discharge
and/or
Daytime cough
Lasting 10–30 d
Severe symptoms
Fever ≥39°C (102.2°F)
Purulent nasal discharge for ≥3 d
Ill-appearing child
Data from American Academy of Pediatrics. Subcommittee on Management of Sinusitis and
Committee on Quality Improvement. Clinical Practice Guideline: Management of sinusitis.
Pediatrics 108: 798, 2001.
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SECTION 12: Pediatrics
Child presenting with
suspected acute bacterial sinusitis
Severe symptoms
Treat with amox/clav,
cefuroxime,
cefpodoxime, or
cefdinir
Mild/moderate symptoms
Child in day care
or recently treated
with antibiotics?
Yes
No
Improved?
No: returns
for further
management
Yes:
follow up
with PCP
Treat with high-dose amoxicillin
If allergic to PCN,
tx with cefuroxime,
cefpodoxime,
cefdinir,
azithromycin, or
clarithromycin
Improved?
Treat with IV
cefotaxime
or ceftriaxone
Sinus CT
and sinus
aspiration
Improved?
Yes:
follow-up
with PCP
No: returns
for further
management
No: returns
for further
management
Subsequent antibiotic
treatment based on
Gram stain and culture
Yes:
follow-up
with PCP
FIGURE 116-1. Management of uncomplicated acute bacterial sinusitis in children. amox
= amoxicillin; clav = clavulanate; PCN = penicillin; PCP = primary care provider; tx = treat.
(Adapted with permission from American Academy of Pediatrics. Subcommittee on Management of Sinusitis and Committee on Quality
Improvement. Clinical Practice Guideline: Management of sinusitis. Pediatrics 108: 798, 2001.)
High-dose amoxicillin (80 milligrams/kg/d PO) for 10 to 14 days is
the antibiotic of choice for uncomplicated mild to moderate sinusitis
in children.1,4 Oral second- and third-generation cephalosporins,
such as cefprozil (7.5–15 milligrams/kg PO twice a day for uncomplicated mild to moderate sinusitis), cefuroxime (15 milligrams/kg PO
twice a day), and cefpodoxime (5 milligrams/kg PO twice a day), are
adequate alternatives for patients with mild penicillin allergy. Because of the cross-reactivity of IgE-mediated allergic reactions between
cephalosporins and penicillins, clarithromycin (7.5 milligrams/kg PO
twice a day) or azithromycin (10 milligrams/kg PO on day 1, then 5 milligrams/kg PO daily for 5 to 7 days) are recommended for patients with
severe penicillin allergies.1 Antibiotics for acute bacterial sinusitis should
be given for 10 to 14 days. For patients who do not improve in 48 to 72
hours on amoxicillin, a β-lactamase inhibitor (clavulanate) should be
added (amoxicillin-clavulanate, 22.5 milligrams/kg PO twice a day). Sulfamethoxazole-trimethoprim, erythromycin, and first-generation cephalosporins are not considered effective choices for sinusitis.12 Adjunctive
therapy with intranasal steroids (e.g., fluticasone propionate, one to two
sprays per nostril daily, or beclomethasone, one to two sprays per nostril
twice a day) has shown modest benefits13,14 and is recommended if antibiotics do not result in improvement in the first 3 to 4 days of treatment.1
Complications of acute bacterial sinusitis are rare but usually involve
the orbit or central nervous system. They should be treated aggressively.
Obtain a CT scan in the ED for proptosis or impairment of extraocular muscle movement, as these findings suggest orbital inflammation
usually from extension of an ethmoidal infection (Figure 116-2). Frontal
and sphenoidal inflammation can lead to intracranial extension, causing
frontal lobe and subdural abscesses as well as meningitis and empyema.
IV antibiotics and possible surgical management may be necessary. An
ophthalmologist and/or neurosurgeon should be consulted promptly for
complications.1
CHRONIC BACTERIAL SINUSITIS
Factors associated with chronic sinusitis include older age, allergic rhinitis,
recurrent viral URIs, immunodeficiency, ciliary dyskinesia, anatomic ab-
FIGURE 116-2. Sinusitis. Adolescent with pansinusitis complicated by periorbital
cellulitis. The patient was also found to have osteomyelitis of the frontal bone (Pott
puffy tumor). (Reproduced with permission from Knoop K, Stack L, Storrow A: Atlas
of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, New York.)
CHAPTER 116: The Nose and Sinuses
normalities, and fungal colonization of the sinuses.7 Nasal saline washes to
aid nasal drainage and bacterial clearance have been recommended by some
experts.15 Antibiotics for chronic sinusitis should cover the usual pathogens
of acute sinusitis as well as aerobic and anaerobic β-lactamase–producing
bacteria. Commonly used oral antimicrobial agents include amoxicillinclavulanate (22.5 milligrams/kg PO twice a day), clindamycin (8 milligrams/kg PO three times a day), and the “newer” quinolones (moxifloxacin,
400 milligrams PO daily) for adolescents, though this drug has not been approved by the U.S. Food and Drug Administration for use in children.16 Antibiotics for chronic sinusitis should be given for at least 4 weeks.17
For patients with chronic sinusitis who have failed antibiotic trials and
nasal saline irrigation, otolaryngology referral is needed, because definitive therapy may involve endoscopic sinus surgery, in which the ostiomeatal area is opened, antrostomies are created, and ethmoid partitions
are removed. This has an estimated success rate of 83% in a combined
pediatric and adult study.18 In the pediatric population, however, a less
invasive approach known as a functional endoscopic sinus surgery, which
is essentially a drainage procedure, has been shown to have good efficacy, with 90% of patients showing marked reduction in symptoms.19
773
pruritus. On physical examination, there may be hypertrophy of the nasal
turbinates and clear secretion from the nares. Concomitant wheezing suggests an association with asthma. A patient with severe symptoms who
does not respond to treatment may warrant a referral to an allergist for
skin testing to detect immediate hypersensitivity reactions to allergens.
Due to the lack of technique standardization, the utility of nasal cytology
is limited to research. In addition, total IgE levels are neither sensitive nor
specific for atopic disease.23
■ TREATMENT
Children with recurrent or refractory sinusitis should be evaluated for immune deficiencies with quantitative Ig levels, IgG subclasses, IgA, and T and
B cell counts. The most commonly diagnosed immune deficiencies in patients presenting with recurrent or refractory sinusitis are selective IgA deficiency, common variable immunodeficiency, and IgG subclass deficiency.20
Children with cystic fibrosis have thick mucus that predisposes them to
sinusitis and is diagnosed through chloride sweat testing. Suspect cystic fibrosis in a child who presents with nasal polyps or chronic sinusitis, particularly in conjunction with failure to thrive and chronic cough.4
Treatment involves recommending environmental controls such as
avoidance of allergens and irritants, including pollutants and cigarette
smoke. Nasal saline irrigation with a syringe or spray reduces the use of
antibiotics and other medications.24 In one small study, children treated
with hypertonic (3%) nasal saline solution had lower symptom scores.25
Antihistamines and intranasal steroids are the mainstays of drug therapy for allergic rhinitis. Second-generation antihistamines, such as loratadine (5 milligrams daily age 2 to 6 years old; 10 milligrams daily >6
years of age) and cetirizine (2.5 to 5.0 milligrams daily age 2 to 6 years
old; 5 to 10 milligrams daily >6 years of age), are preferable because they
are less likely to cross the blood–brain barrier and therefore cause less sedation than first-generation antihistamines such as diphenhydramine
and hydroxyzine. Intranasal corticosteroids reduce inflammation of the
nasal mucosa. Daily morning dosing minimizes the impact on the hypothalamic–pituitary–adrenal axis.26
Other newer therapies target the immune system directly. Montelukast,
a leukotriene receptor antagonist, and disodium cromoglycate, a mast cell
stabilizer, have been used with success for symptom reduction.27,28 Although treatment with monoclonal anti-IgE antibodies has been shown to
be effective for allergic rhinitis, it is not commonly used at this time.29
ALLERGIC RHINITIS
NASAL FOREIGN BODIES
Allergic rhinitis is an IgE-mediated chronic or recurrent inflammatory
response of the nasal mucosa that is induced by an allergen, and typically
affects children over the age of 2 years old. The International Study on
Asthma and Allergies in Childhood determined that the worldwide
prevalence of symptoms of allergic rhinoconjunctivitis is 2.2% to 14.6%
in children ages 6 to 7 years old and 4.5% to 45.5% in adolescents ages 13
to 14 years old. The International Study on Asthma and Allergies in
Childhood also showed that approximately 80% of pediatric patients
with asthma have allergic rhinitis and that allergic rhinitis makes it more
difficult to control asthma, making it an important topic for the emergency medicine physician caring for children.21
■ EPIDEMIOLOGY
■ PATHOPHYSIOLOGY
The child with a nasal FB may present with local pain (23% to 55%), nasal discharge (7% to 36%), epistaxis, or admission by the child. Alternatively, the parent may witness the child placing something in the nose or
the object may be found during routine child care. Most children with
nasal FBs are asymptomatic (Figure 116-3).30,31
■ SPECIAL POPULATIONS
Seasonal allergic rhinitis (hay fever) is usually caused by airborne allergens
such as pollen, whereas perennial allergic rhinitis is usually caused by dust
mites, animal dander, and mold. Allergic rhinitis is an IgE-mediated inflammatory response in the nasal mucosa that occurs after sensitization
with a specific allergen. IgE binding triggers mast cell degranulation and
subsequent histamine release. The binding of histamine to the histamine1 receptor on nasal neurons and nasal vasculature is the ultimate mechanism responsible for the nasal itch, sneeze, rhinorrhea, and nasal obstruction of allergic rhinitis.
■ CLINICAL FEATURES
Allergic rhinitis presents with clear rhinorrhea, nasal pruritus, and sneezing. Ocular symptoms, such as conjunctival hyperemia and pruritus, may
coexist. Symptoms can lead to sleep disturbance, limitations in activity,
and poor school performance.22
■ DIAGNOSIS
Patients with allergic rhinitis report symptoms of paroxysmal sneezing,
nasal pruritus, rhinorrhea, oropharyngeal pruritus, hyperemia, and ocular
Foreign body (FB) insertion is a common pediatric complaint in the ED.
FBs in the external ear canal predominate, followed by nasal FBs. Children who insert objects into their nose are, in general, younger than patients with auditory FB insertion.30 Although pharyngeal FBs can present
in adults, nasal FBs are almost exclusively a pediatric problem. Common
objects include beads, paper, rocks, toy parts, and organic material such
as peas, corn, seeds, nuts, and legumes (see Chapter 143A, Pediatric Procedures: Nasal and Otic Foreign Bodies).
■ CLINICAL FEATURES
■ DIAGNOSIS
Most nasal FBs can be directly visualized. Have a high index of suspicion for a nasal FB in an appropriately aged child who presents with
persistent, unilateral, purulent, foul-smelling nasal discharge.
■ TREATMENT
Although occasionally a nasal FB is removed by physiologic mechanisms
such as sneezing, many are removed by the ED physician. The key to successful removal is immobilization. Approximately 20% of patients undergoing nasal FB removal in the ED are given procedural sedation, most
commonly with ketamine.32 The patient should be placed in a supine position, and the nasal mucosa should be pretreated topically with 1% lidocaine
and 0.5% phenylephrine.30 Phenylephrine shrinks inflamed nasal mucosa,
allowing for easier removal of entrapped FBs and also reduces the likelihood of procedural epistaxis. The most common methods for removal in-
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SECTION 12: Pediatrics
Other causes of epistaxis include facial trauma, FBs, sinusitis, or increased vascular pressure secondary to excessive coughing. Less commonly, epistaxis can be the presenting complaint of a coagulopathy,
leukemia, or nasal tumor. Most pediatric epistaxis originates from Kiesselbach plexus, a venous vascular plexus on the anterior nasal septum.
Although anterior bleeds usually ooze, posterior bleeds tend to be more
profuse because they originate from the sphenopalatine artery. This type
of bleeding, while rare, carries a higher risk of airway compromise, aspiration of blood, and life-threatening hemorrhage.
■ TREATMENT
FIGURE 116-3. A 6-year-old child was brought to the ED with a complaint of a
foul-smelling serosanguineous discharge from the right nostril. He confessed to
putting a button in his nostril about 1 week before this visit. (Reproduced with permission from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. ©
2006, McGraw-Hill, New York. Figure 9-9.)
Most epistaxis can be controlled with conservative methods, such as
pinching the nostrils together for 5 to 10 minutes with the neck slightly
extended. Ice or phenylephrine can also be applied to the nose to promote vasoconstriction. Application of cotton gauze under the upper lip
can be used to compress the labial artery. Cautery with heat or silver
nitrate can be used if the bleeding site can be identified. If all else fails,
the nares can be packed with absorbable gelatin foam, oxidized cellulose, or preformed devices [Rhino Rocket Child®, (Shippert Medical
Technologies Corporation, Centennial, CO)]. See Chapter 239, Epistaxis, Nasal Fractures, and Rhinosinusitis for the procedure and related
information.
■ DISPOSITION AND FOLLOW-UP
clude forceps, the Foley catheter technique, applied positive pressure via an
anesthesia bag, and the use of a suction catheter. All have varying degrees
of success and failure (see Chapter 143A, Pediatric Procedures: Nasal and
Otic Foreign Bodies). Alligator forceps work best if the object is close to the
anterior nares that can be easily grasped. If the object is friable, there is a
risk of pulling it apart and leaving pieces in the nose. Other techniques include the advancement of a lubricated 5F or 6F Foley balloon catheter past
the object, inflating the balloon with air and withdrawing the catheter to
gently remove the FB; positive pressure applied to the mouth while occluding the contralateral naris; or use of a suction catheter to remove the object.30 A particularly deep nasal FB may need to be removed by an
otolaryngologist. Although it is possible to aspirate a nasal FB, this is
rare,30,31 and most complications of nasal FBs occur during attempted
removal. Complications of nasal FB removal include failure to remove the
object, epistaxis, laceration, and, rarely, septal perforation. If irrigation is
attempted and the object is expandable, such as rice, vegetable matter, or
sponge, the FB can swell, impeding its extraction.
■ SPECIAL CONSIDERATIONS
A button battery in the nasal cavity can cause liquefactive necrosis
and septal perforation in as little as 7 hours.33 For this reason, remove
button batteries as quickly as possible. Do not instill any type of nasal
drops before removal because the electrical charge of the battery will
produce electrolysis of any electrolyte-rich fluid. This results in a severe alkaline burn. Button batteries are often not directly visualized in
the ED because of extensive mucopurulent discharge and mucosal edema. For this reason, consider a plain radiograph to characterize a nonvisible FB or unilateral foul-smelling nasal discharge.34
Although more common in the external ear, live FBs in the nose also
require special mention. Cockroaches, mosquitoes, and beetles are all
uncommon FBs. They are often related to sleeping on the floor or poor
hygiene. The recommended approach is to first kill the insect with 2%
lidocaine or mineral oil and then attempt removal.
EPISTAXIS
Epistaxis usually occurs in children ages 2 to 10 years old. It is rare in infants and older children.34
Nose bleeds in children are most often secondary to nose picking or
rhinitis sicca. Rhinitis sicca is more common in northern latitudes during the winter when the humidity is low and dry air heating systems can
cause nasal mucosa desiccation and frequent bleeding.
Most children with simple epistaxis can be sent home with instructions to
avoid digital trauma and apply petroleum jelly to the nares at night to help
lubricate the mucosa. Children with recurrent or severe epistaxis and a
family history of a bleeding disorder, or abnormal screening prothrombin
time or activated partial thromboplastin time should be referred to a hematologist for a complete coagulopathy evaluation. Approximately one
third will have a diagnosable coagulopathy, most often von Willebrand
disease type 1.35 If bleeding is recurrent, unilateral, and associated with nasal obstruction, a neoplasm may be suspected, and an otolaryngology consult is warranted. In an adolescent male with profuse unilateral epistaxis
requiring packing, juvenile nasal angiofibroma should be suspected, and
the patient should be evaluated with a CT scan.
Acknowledgments: The authors gratefully acknowledge the contributions of Kimberly S. Quayle, Susan Fuchs, and David M. Jaffe, the authors of this chapter in the previous edition.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
117
The Mouth and Throat
Derya Caglar
Richard Kwun
Lesions of the mouth and throat are common in children and can range
from benign conditions that require no intervention to significant systemic illness requiring extensive treatment and support (Table 117-1).
Making the distinction between these conditions can be difficult. Mouth
pain secondary to viral infections of the oropharynx are among the most
common presenting complaints of pediatric patients; however, most require no treatment beyond supportive care and pain control. Bacterial
infections of the mouth and throat, such as pharyngitis and uvulitis,
cause local and systemic illness and rarely can lead to life-threatening
complications. The management of dental injuries, whether from neglect or trauma, differs for primary and permanent teeth.
CHAPTER 117: The Mouth and Throat
775
TABLE 117-1 Common Causes of Oral Lesions in Children
Anterior
Posterior
Diffuse
Aphthous ulcers
Contact stomatitis
Herpes simplex
gingivostomatitis
Adenovirus pharyngitis
Coxsackie virus
Cytomegalovirus
Epstein-Barr virus
Streptococcal pharyngitis
Autoimmune disease
Candidiasis (thrush)
Chemotherapy-related
mucositis
Trauma
Vincent angina
Medication-related
[phenytoin (Dilantin)]
Stevens-Johnson
syndrome
Varicella zoster
NORMAL VARIANTS
Epstein pearls are remnants of embryonic development that present as
white, slightly raised nodules seen most commonly midline at the junction of the soft and hard palates of neonates. They are often seen incidentally during feeding and do not cause the child any pain or discomfort.
Most resolve spontaneously.
Geographic tongue (Figure 117-1) can be a source of great parental
concern. It is a benign, asymptomatic condition and is often incidentally
noticed by parents during another illness. Patients will present with an
area of erythema and atrophy of the papillae of the tongue surrounded by
a serpiginous, elevated white or yellow border usually located in the anterior two thirds. The lesions will improve and disappear gradually over time
but tend to recur in other areas of the tongue. There is no known cause, although it has been associated with childhood allergies and atopy. No treatment other than reassurance is necessary.
Mucoceles (Figure 117-2) and ranulas are lesions of the oral mucosa
that present as small, bluish, discrete, mucosal swellings on the lower lip
or sublingual areas.1 Intervention is needed only with disruption of feeding or development of speech. Adjacent salivary glands are usually removed in addition to the lesion to prevent recurrence.
Eruption cysts are smooth, painless bluish-black areas of swelling found
over an erupting tooth that usually resolve with the eruption of the underlying tooth. Although these findings are frightening to the parent, they are
benign in nature, often asymptomatic, and require no intervention.
FIGURE 117-1. Geographic tongue.
FIGURE 117-2. Mucocele. (Reproduced with permission from Wolff KL, Johnson
R, Suurmond R: Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology, 6th
ed. © 2009, McGraw-Hill, New York, Figure 34-14.)
NONINFECTIOUS LESIONS
Aphthous ulcers, also known as canker sores, are the most common form
of recurrent oral ulcers, occurring in 5% to 25% of the general population.2 They present in children and adolescents as painful, shallow ulcers
on the oral mucosa (Figures 117-3 and 117-4). The etiology of canker
sores is unknown; they often recur and are found most frequently on the
buccal and lingual mucosa. Spontaneous resolution after 7 to 10 days is
the norm. Topical medications, such as antimicrobial mouthwashes and
topical analgesics, can achieve the primary goal of reducing pain but do
not hasten healing or improve recurrence or remission rates.
Less commonly, periodic fever, aphthous stomatitis, pharyngitis, and
cervical adenitis syndrome can cause aphthous ulcers in children 2 to 6
years of age. These patients present with fever, malaise, exudative pharyngitis, cervical lymphadenopathy, and multiple oral ulcers lasting 4 to 6
days, often recurring multiple times a year. Recurrence is the key to diagnosis, as this constellation of symptoms is difficult to distinguish from
viral infection, particularly in the ED where patient care is cross-sectional rather than longitudinal. The cause of fever, aphthous stomatitis,
pharyngitis, and cervical adenitis remains unknown, but most patients
respond well to oral steroids, with resolution of symptoms within 24
FIGURE 117-3. Aphthous ulcers: Note the multiple ulcers of various sizes located
on the lip and gingival mucosa. These lesions rarely occur on the immobile oral
mucosa of the gingiva or hard palate. (Reproduced with permission from Knoop K,
Stack L, Storrow A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill,
New York. Figure 6-33.)
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SECTION 12: Pediatrics
FIGURE 117-5. Herpangina: Typical elliptical or oval-shaped papulovesicular
lesions with erythematous rims are seen on the posterior soft palate. (Reproduced
with permission from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine.
© 2006, McGraw-Hill, New York. Figure 3-67.)
FIGURE 117-4. Aphthous ulcers: minor, multiple, very painful, gray-based ulcers
with erythematous halos on the labial mucosa. (Reproduced with permission from
Wolff KL, Johnson R, Suurmond R: Fitzpatrick’s Color Atlas & Synopsis of Clinical
Dermatology, 6th ed. © 2009, McGraw-Hill, New York. Figure 31-1.)
hours. Some studies have also shown that tonsillectomy leads to complete resolution of symptoms.3
STOMATITIS
■ VIRAL INFECTIONS
The majority of infectious mouth and throat lesions are of viral etiology.
Enteroviral infections commonly cause oral lesions with or without other physical findings such as fever, rash, abdominal pain, or diarrhea.
Herpes viruses can cause oropharyngeal lesions that are extremely painful, may be recurrent, and are usually associated with high fever during
primary infection. Adenovirus can cause acute pharyngitis in association
with fever and tonsillar erythema. Exudative pharyngitis in association
with fevers, fatigue, and malaise is frequent in infections with EpsteinBarr virus (EBV) and cytomegalovirus (CMV).
Herpangina Herpangina is an enteroviral infection that causes a vesicular enanthem (Figure 117-5) of the tonsils and soft palate, affecting children 6 months to 10 years of age during late summer and early fall. It is
primarily caused by Coxsackie viruses A1–A6, A8, A10, A22, and B3.4
These vesicles are often very painful and are accompanied by fever, difficulty swallowing, and dysphagia. Patients may complain of headache,
vomiting, and abdominal pain. Diagnosis is primarily clinical. Viral culture is the gold standard for confirmation of infection; however, enteroviral polymerase chain reaction can detect enteroviral RNA from
nasopharyngeal secretions, blood, urine, or feces much sooner and with
higher sensitivity (77% to 100%, 95% from throat culture).5
Treatment is palliative because the symptoms are usually mild and lesions heal spontaneously after 3 to 5 days. Antipyretics and systemic analgesics aid with supportive care. Viscous lidocaine is generally not
recommended for pain relief given the risk of ingestion and associated
seizures.6 A mixture of diphenhydramine and Maalox applied orally in a
swish and swallow fashion can provide local pain relief. When topical
treatment does not suffice, systemic analgesics, including narcotics, may
be necessary. Close attention must be paid to hydration status, as children can quickly become dehydrated, requiring admission for IV fluid
replacement if the patient cannot tolerate oral intake.
Hand, Foot, and Mouth Disease Hand, foot, and mouth (HFM) disease is
also seen with enteroviral infections.7 Coxsackie virus A16 is the most
common cause of HFM disease, but A5, A9, A10, B2, B5, and enterovirus
71 subtypes have also been implicated.8,9 The disease is typically seen in
children <5 years old but is most common in infants and toddlers. HFM
has a seasonal distribution and primarily occurs in the spring and summer months.
The illness generally follows a mild course, starting with a low-grade
temperature lasting 2 to 3 days and associated with decreased appetite,
malaise, vague abdominal pain, and mild upper respiratory symptoms.
Children will then develop an enanthem of vesicles, followed by the exanthema, though both can occur simultaneously.
Oral lesions usually begin as erythematous macules and evolve into
vesicles and ulcers over the course of 1 to 3 days, with new lesions appearing throughout the duration of illness. Lesions typically involve the
palate, buccal mucosa, gingiva, and tongue. Pain from these ulcerations
often leads to decreased oral intake and mild dehydration.
The associated rash appears on the palms of the hands, soles of the
feet, and on the buttocks (Figure 117-6). Lesions begin as erythematous
macules that later may develop into small nontender vesicles. The oral
and skin lesions tend to resolve over 4 to 7 days.
Diagnosis is primarily clinical, although the virus can be isolated from
swabs of the vesicles and from stool specimens. Treatment involves supportive and symptomatic care with antipyretics, topical and oral analgesics, and oral rehydration. In rare cases, pain may lead to inadequate oral
intake. Analgesics and IV fluids may be needed to treat dehydration.
Rare complications of these infections include viral meningitis, meningoencephalitis, myocarditis, and sepsis.10,11
Herpes Simplex Gingivostomatitis Herpes simplex virus (HSV) can cause
a variety of symptoms in the pediatric population. Infants and toddlers
will often present with high fever, pharyngitis, and gingivostomatitis
during their primary infection.12 Initial HSV infection is often very difficult to distinguish from other viral etiologies. Most children go undiagnosed with HSV until they later present with a more classic painful labial
reactivation lesion.
Acute herpetic gingivostomatitis is the most common presentation of
primary HSV infection in children13,14 (Figure 117-7). It usually presents
at 6 months to 5 years of age, although primary HSV infection may occur
in older children and adults. Ninety percent of cases are due to HSV 1,
however HSV 2 has also been found to cause disease.15 HSV transmission
occurs via contact with infectious saliva, typically from caregivers who
CHAPTER 117: The Mouth and Throat
777
FIGURE 117-7. Herpes simplex virus (HSV): Extensive vesicular lesions along the
vermilion border and surrounding tissues are consistent with HSV infection. (Reproduced with permission from Knoop K, Stack L, Storrow A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, New York. Figure 6-31.)
may be unaware of their infectious risk. The incubation period is 2 to 12
days, with a mean of 4 days.
Clinically, the disease presents with abrupt onset of high fever, irritability, decreased oral intake and drooling, and swollen, erythematous,
friable gingiva. Physical findings include vesicular lesions in the oral cavity, ulcerations, and tender cervical lymphadenopathy. Symptoms may
persist for up to 3 weeks but more commonly last <1 week.16
Diagnosis is primarily clinical. Viral culture has been the gold standard
of laboratory testing for years. However, the recovery of virus from lesions
is low (7% to 25%). HSV polymerase chain reaction is a newer, more accurate assay with improved sensitivity in detecting infection17 (Table 117-2).
Tzanck smear of fluid from unroofed lesions 24 to 48 hours old showing
multinucleated cells can also confirm the diagnosis but cannot differentiate infection between viruses within the herpesvirus family.
FIGURE 117-6. Hand, foot, and mouth disease: typical erythematous macules on
the palms or soles. (Reproduced with permission from Shah BR, Lucchesi M: Atlas
of Pediatric Emergency Medicine. © 2006, McGraw-Hill, New York. Figure 3-65.)
TABLE 117-2 Herpes Simplex Virus Testing
Viral culture
Polymerase chain reaction
Time of Use
Sensitivity (%)
Vesicular lesions 24–48 h
Any lesions present
27–90
59–89
Reproduced with permission from Mell HK: Management of oral and genital herpes in the
emergency department. Emerg Med Clin North Am 26: 457, 2008.
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SECTION 12: Pediatrics
Treatment consists of supportive care with oral analgesics/antipyretics (acetaminophen or ibuprofen), topical analgesics, and systemic antiviral therapy for severe disease (acyclovir, 15 milligrams/kg PO divided
five times a day for 7 days). Immunocompromised patients are at significantly higher risk for systemic dissemination, and hospitalization and
treatment with IV acyclovir should strongly be considered.
Prognosis of a primary HSV infection beyond the fetal/neonatal period is usually very good. As the primary infection resolves, a lifelong latent residency of the virus within the trigeminal ganglion occurs, which
may lead to less severe recurrences at the same site in the future.
PHARYNGITIS
It is important to distinguish between superficial and deep space infections of the mouth and throat. Deep space infections are discussed in
Chapter 119, Stridor and Drooling, and are typically associated with toxic appearance, high fever, drooling, stridor, or changes in phonation,
trismus, or torticollis. Simple pharyngitis, on the other hand, accounts
for 1% to 2% of all visits to outpatient clinics and EDs, resulting in 7.3
million annual visits for children.18 Viral etiologies predominate in children with acute pharyngitis19 (Table 117-3).
Symptoms associated with acute pharyngitis include sore throat, odynophagia, fever, headache, abdominal pain, nausea and vomiting, cough,
hoarseness, coryza, diarrhea, arthralgias, myalgias, and lethargy. Physical examination may reveal tonsillopharyngeal erythema and/or exudates, soft palate petechiae, uvulitis, anterior cervical lymphadenitis,
rash, conjunctivitis, anterior stomatitis, and discrete ulcerative lesions. It
is often difficult to distinguish between viral and bacterial causes of pharyngitis based on physical examination alone,20 and tonsillar exudate
does not imply bacterial etiology. This often results in overdiagnosis of
bacterial etiology and unnecessary antibiotic treatment. Most viral infections are self limited and require only symptomatic treatment. Interestingly, patient satisfaction appears to be greatest when a physician shows
concern and provides reassurance, and is not related to whether or not
antibiotics are prescribed.21
Pharyngitis is the best known acute clinical manifestation of EBV
(Figure 117-8). It often begins with malaise, headache, and fevers before
development of the more specific signs of exudative pharyngitis and posterior cervical lymph node enlargement. Splenomegaly and hepatomegaly can also occur. Patients mistakenly treated for a bacterial pharyngitis
with amoxicillin or ampicillin often develop a characteristic pruritic
maculopapular rash that aids in diagnosis.
Diagnosis is often clinical, although a heterophile test (monospot) can
aid in diagnosis. It is important to remember that this test relies on crossreactivity of patient antibodies and is relatively insensitive in pediatric
patients (25% positive in 10 to 24 months vs. 75% in 24 to 28 months).
Furthermore, the monospot test typically does not turn positive in cases
of EBV until symptoms have been present for 1 week or more. A negative
TABLE 117-3 Common Causes of Viral Pharyngitis in Children
Rhinovirus
Coronavirus
Adenovirus
Herpes simplex virus 1 and 2
Parainfluenza virus
Echovirus
Coxsackie virus A
Epstein-Barr virus
Cytomegalovirus virus
Human immunodeficiency virus
Influenza virus A & B
Reproduced with permission from Gerber MA: Diagnosis and treatment of pharyngitis in
children. Pediatr Clin North Am 52: 729, 2005.
FIGURE 117-8. Marked white exudates on the tonsils of a child with Epstein-Barr
virus infection. (Reproduced with permission from Mei Kane KS, Ryder JB, Jonson
RA, et al: Color Atlas & Synopsis of Pediatric Dermatology. New York, McGraw-Hill,
2002, p. 576.)
test, therefore, does not exclude the diagnosis of EBV; when necessary,
testing for EBV immunoglobulin M (IgM) and IgG is both sensitive and
specific, although results are not immediately available. Atypical lymphocytes may be present on the complete blood count, if obtained. EBV
infection may be associated with other organ involvement, including
hepatitis. Patients with right upper quadrant tenderness should have liver enzymes evaluated.
Treatment is largely supportive; however, some patients may require
IV fluids and pain medication. Although there is no evidence of efficacy,
a dose of oral or parenteral steroid can be considered to reduce tonsillar
enlargement when swallowing or respiratory symptoms are attributed to
enlarged tonsils. When splenomegaly is noted, proper counseling regarding risk factors and symptoms of splenic rupture should be given.
CMV infection can very closely mimic EBV mononucleosis. Symptoms
and signs of the two infections are almost identical. Indeed, patients presenting with classic infectious mononucleosis who are heterophile-negative
are often infected with CMV. Fever, malaise, and systemic complaints predominate in the clinical picture of CMV, with less prominent cervical lymphadenopathy or splenic enlargement than EBV. Distinguishing between
the two etiologies is difficult, and, often, the diagnosis is confirmed with
laboratory testing for CMV IgM and IgG. Treatment is again supportive.
Acute retroviral syndrome, or acute infection with human immunodeficiency virus (HIV), may present similarly to EBV pharyngitis in 50%
to 70% of patients. Differences implicating HIV from other viral illnesses
may include presence of high-risk behaviors in the social history, the
acuity of onset, the absence of exudate and prominent tonsillar hypertrophy, presence of a rash, and mucocutaneous ulceration.
Acute infection with HIV is uncommon in the pediatric patient, although it must be considered in adolescents in whom high-risk behaviors
are identified. In addition to the usual causes of pharyngitis, opportunistic
infections, such as Candida albicans and Mycobacterium avium, should be
considered in the immunocompromised patient.22
■ BACTERIAL INFECTIONS
Group A β-hemolytic Streptococcus (GABHS) pharyngitis is the most
commonly occurring form of acute bacterial pharyngitis for which antibiotic therapy is indicated.23 It typically occurs in the winter and early
spring, is rare <2 years of age,24 and primarily affects children ages 5 to
15 years old.25 Although GABHS accounts for only 15% to 30% of pharyngitis in children, approximately 53% of children with pharyngitis receive antibiotics.26 Additionally, a substantial proportion of patients
treated for GABHS pharyngitis receive an inappropriate antibiotic. Clinical trials are under way to evaluate the safety and efficacy of a multivalent group A Streptococcus vaccine.27
CHAPTER 117: The Mouth and Throat
TABLE 117-4 Centor Criteria for Group A β-Hemolytic
Streptococcus Pharyngitis
Tonsillar exudates
Tender anterior cervical lymphadenopathy
Absence of cough
History of fever
Reproduced with permission from Centor RM, Witherspoon JM, Dalton HP, et al: The diagnosis of strep throat in adults in the emergency room. Med Decis Making 1: 239, 1981.
Several clinical prediction rules have been created to identify cases of
GABHS pharyngitis, and most are modifications of the original Centor
criteria28 (Table 117-4). Current guidelines recommend the use of Centor
criteria to determine which patients require testing for GABHS infection,
as these criteria have proven reliable and easy to use. With zero or one criteria, GABHS is unlikely, and testing and treatment for GABHS are not indicated. With two or more criteria, testing should be performed using a
rapid antigen detection test (RADT) and/or culture.29
Although bacterial culture remains the gold standard, with a sensitivity of 90% to 95%, the 18- to 48-hour wait time for definitive diagnosis is
often impractical, and the use of RADT has become popular. The sensitivity of RADTs varies from 80% to 90%. It is recommended that practitioners who use RADTs to rule out GABHS do so only after confirming
in their own practice that their RADT has a sensitivity similar to that of
a throat culture. When two or more Centor criteria are present, but the
RADT is negative, a confirmatory throat culture is recommended.
The antibiotic treatment of GABHS pharyngitis has been shown to:
(1) shorten the duration of illness, (2) prevent transmission, (3) prevent
suppurative complications (e.g., acute otitis media, acute sinusitis, and
peritonsillar abscess), and (4) prevent systemic illness such as rheumatic
fever, rheumatic heart disease, and poststreptococcal glomerulonephritis.
Antibiotics for the treatment of GABHS pharyngitis should be reserved,
however, for patients with a positive RADT or culture, or those meeting
clinical criteria for diagnosis. It is important to keep in mind that GABHS
pharyngitis is typically a self-limited disease, with fever and constitutional
symptoms diminishing markedly at days 3 and 4 after symptom onset, and
that antibiotics only decrease the duration of symptoms by approximately
16 hours.30 Treatment can be delayed safely for up to 9 days after symptom
onset and still prevent major nonsuppurative sequela. There is no definitive evidence that antibiotic use can prevent acute glomerulonephritis.
Further confounding the decision to treat is the possibility that a percentage of patients may be GABHS carriers, and that acute infection with another organism may be causing disease rather than GABHS pharyngitis.
Penicillin remains the treatment of choice, based on its efficacy, safety,
narrow spectrum, ease of dosing, compliance, and cost (Table 117-5).
No clinical isolate of GABHS has been documented to be penicillin resistant. Treatment failures may be attributable to viral pharyngitis with
GABHS carriage, medication noncompliance, or to reinfection of patients successfully treated for GABHS. Ten days of oral therapy with
twice-a-day dosing is recommended for complete pharyngeal eradication; similar efficacy is achieved with a single IM dose of penicillin,
which is based on the patient’s age.
Several alternative therapy options exist for those unable to take penicillin. The efficacy of amoxicillin appears to be comparable to that of
penicillin and is acceptable in children who more easily tolerate the taste
of the suspension. Erythromycin and first-generation cephalosporins are
suitable alternatives in penicillin-allergic patients. Clindamycin may be
required for erythromycin-resistant GABHS in the penicillin-allergic
patient. Practitioners should be aware of increasing macrolide resistance
worldwide. Although <5% of GABHS isolates in the U.S. appear to be
erythromycin resistant, this may increase given higher resistance patterns in other parts of the world and the widespread use of macrolides for
the treatment of upper and lower respiratory tract infections.
Routine antibiotic prophylaxis is not recommended for household
members exposed to GABHS, as the risk of developing subsequent phar-
779
TABLE 117-5 Antibiotics for the Treatment of Streptococcal Pharyngitis
Medication
Dosage
Penicillin V or amoxi- Child: 250 milligrams two times daily
cillin (first line)
Adolescent/adult: 500 milligrams
twice a day
Benzathine penicil- 25,000–50,000 milligrams/kg daily;
lin G
maximum dose, 1.2 million units
Azithromycin (alter- Child: 12 milligrams/kg once daily
native for penicillin- Adolescent/adult: 500 milligrams on
allergic patients)
day 1 then 250 milligrams on days
2–5
Erythromycin esto- Child: 20–40 milligrams/kg daily,
late or ethylsuccidivided in two doses
nate (alternative for Adolescent/adult: 400–800 millipenicillin-allergic
grams two to four times a day
patients)
Cephalexin
25–50 milligrams/kg daily, divided in
two or four doses
Route
Duration
PO
10 d
IM
One dose
PO
5d
PO
10 d
PO
10 d
yngitis is approximately 10%.31 Although tonsillectomy is indicated for
recurrent tonsillitis in children, there is no clear evidence to support tonsillectomy in cases of recurrent pharyngitis or pharyngotonsillitis.32
Benefits of antibiotic treatment for other bacterial pharyngitides are
unclear at this time. There have been no cases of acute rheumatic fever
due to non-GABHS, such as groups C and G Streptococcus. If treated, antibiotics used in the treatment of GABHS are appropriate for pharyngitis
due to groups C and G Streptococcus.
Several other bacterial etiologies must be considered in patients with
pharyngitis. These include Neisseria gonorrhoeae, Corynebacterium diphtheriae, Arcanobacterium haemolyticum, Yersinia enterocolitica, Yersinia pestis,
Francisella tularensis, Mycoplasma pneumoniae, and Chlamydia species.
Gonococcal pharyngitis is difficult to distinguish from other bacterial
causes of pharyngitis. A careful sexual history, including exposure to partners with known sexually transmitted diseases and oral sex practices,
should be elicited in all adolescent patients presenting with pharyngitis.
Gonococcal infection of the throat may be associated with infection elsewhere, including proctitis, vaginitis, and/or urethritis. The diagnosis requires special culture on Thayer-Martin medium, although nucleic acid
amplification testing is also available. A positive culture in a prepubertal
child is highly suspicious for sexual abuse, and further investigation is warranted with involvement of the appropriate child protection agencies. IM
ceftriaxone (250 milligrams) is the only therapy recommended by the Centers for Disease Control and Prevention for the treatment of uncomplicated
pharyngeal gonorrhea. Empiric treatment for concomitant chlamydia with
the addition of 1 gram of azithromycin should be given unless it has specifically been ruled out.
Although occurrence is rare in developed countries due to vaccination,
C. diphtheriae should be considered in those patients who are under- or
unimmunized. Toxigenic strains of this bacterium produce an exotoxin
that causes localized necrosis of the respiratory mucosa and can lead to
both cardiac and neurologic complications. Pseudomembrane formation
in the respiratory tract can result in airway obstruction. Identification of
the causative organism is made using Loeffler or tellurite selective medium. Treatment of pharyngeal diphtheria is aimed at bacterial eradication
as well as exotoxin neutralization. Penicillin and erythromycin are the antibiotics of choice, along with equine diphtheria antitoxin. Serious sequelae can be prevented with prompt antibiotic administration, and treatment
should be started when diphtheria is clinically suspected.
A. haemolyticum closely mimics GABHS pharyngitis and may also produce a scarlatiniform rash in teenagers; rarely, it produces a membranous
pharyngitis similar to that of diphtheria. It may be missed on routine cultures and may be more readily detected on human-blood agar plates. Both
macrolide and β-lactam antimicrobial agents are effective treatment.
780
SECTION 12: Pediatrics
Permanent Teeth
6
7
8 9
10
11
12
Permanent maxillary
13 left second premolar
14
5
4
Permanent
3
maxillary
right first molar 2
15
16
1
Permanent
32
mandibular
right third molar 31
30
17
18
19
20
29
21
28
22
27
26 25 24 23
FIGURE 117-9. Uvulitis: edematous, erythematous uvula. (Reproduced with permission from Knoop K, Stack L, Storrow A: Atlas of Emergency Medicine, 2nd ed.
© 2002, McGraw-Hill, New York. Figure 5-28.)
Primary Teeth
C
UVULITIS
Isolated inflammation of the uvula is unusual and has infectious and noninfectious causes. When associated with pharyngitis, the most common
bacterial etiology is GABHS.33 In the unimmunized patient, Haemophilus
influenzae type b is the next most common cause, and may occur with epiglottitis. Other bacterial causes are Fusobacterium nucleatum, Prevotella
intermedia, S. pneumoniae, and C. albicans. Noninfectious causes include
trauma from instrumentation, irritant inhalation, vasculitis, allergic reaction, and angioedema.34
The inflamed uvula appears erythematous, enlarged, and edematous
(Figure 117-9). Patients may present with fever, sore throat, difficulty
swallowing, odynophagia, drooling, and/or respiratory distress.
Uvulitis is a clinical diagnosis. When there is associated pharyngitis,
an RADT and/or throat culture should be performed. H. influenzae diagnosis requires culture on Loeffler or tellurite selective medium.
Antibiotics to cover GABHS should be based on RADT or throat culture results. Acute airway obstruction is unusual with isolated uvulitis;
however, when epiglottitis is also present, emergent airway management
and intubation may be required. When allergic reaction or angioedema
are suspected, treatment may include epinephrine, antihistamines, and
steroids. Precipitants, such as inhaled irritants, allergens, and responsible medications, such as an angiotensin-converting enzyme inhibitor,
should be removed from the environment.
D
E
F G
H
I
B
Primary
A
maxillary
right first molar
T
S
J
The evaluation and management of pediatric dental trauma, including
subluxation, luxation, intrusion, extrusion, avulsion, and fracture, differs between primary and secondary (permanent) teeth (Figure 117-10).
Most dental injuries occur in the toddler years, when children are
learning to walk, although in all children, nonaccidental trauma must be
considered.35 Up to 75% of abused children may have orofacial injuries,
and a high index of suspicion must be maintained. Two other significant
periods of trauma include school-aged children, from play injuries, and
adolescents, mainly due to sports. The most commonly injured teeth are
the maxillary central incisors.
Primary tooth eruption begins at approximately 6 months of age and
is usually complete by 3 years of age. Secondary teeth may begin to erupt
at 6 years of age and can continue past adolescence if the wisdom teeth
are not extracted.
Subluxation refers to loosening of the tooth without displacement and
is due to periodontal ligament damage (Figure 117-11). On examination,
Primary maxillary
left second molar
K
L
R
Primary
Q PO N M
mandibular
right lateral incisor
Primary mandibular
left canine
FIGURE 117-10. Primary and secondary dentition.
the tooth is mobile, and sulcal bleeding may be present. When subluxation
is noted in a primary tooth, no intervention is required; however, permanent teeth may require splinting. Dental follow-up is recommended in either case, as pulpal necrosis may occur.36
Luxation is a loosening and displacement of a tooth from its normal
anatomic position and occurs when the periodontal ligament is torn.
The tooth is often nontender and immobile, and may be fixed in its new
position. Primary teeth may be allowed to passively reposition, although
Enamel
ORAL PROBLEMS
■ DENTAL TRAUMA
Permanent
mandibular
left canine
Dentin
Crown
Pulp
Cementum
Root
Periodontal
ligament
Alveolar
bone
Apex
FIGURE 117-11. Normal anatomy of the tooth.
CHAPTER 117: The Mouth and Throat
dental consultation is recommended. Permanent teeth require active repositioning and splinting as soon as possible.
Intrusion occurs when a tooth is driven apically into the socket, displacing into the alveolar bone. The tooth appears shortened, or even absent, and, when visible, is not mobile or tender. Ninety percent of
intruded teeth will re-erupt spontaneously in 2 to 6 months. Extraction
of primary teeth is indicated when the apex is displaced toward the permanent tooth germ, as determined by radiography. Permanent teeth
with immature root formation may be allowed to re-erupt; mature teeth
require orthodontic or surgical extrusion.
Extrusion occurs when a tooth is displaced from its socket. The tooth
appears elongated and is mobile, due to tearing of the periodontal ligament. Both primary and permanent teeth should be repositioned and
splinted as soon as possible. If the injury is severe enough, primary teeth
may require extraction in the ED.
Avulsed primary teeth should not be replanted, as this may damage
the permanent tooth germ. Permanent teeth, by contrast, require urgent
reimplantation, as success is time dependent. There is 85% to 97% survival of permanent teeth when replaced within 5 minutes, and near-zero
survival at 1 hour. Avulsed teeth should be handled by the crown to
avoid damaging the periodontal ligament. Debris should be removed
with gentle rinsing in saline or water; scrubbing should be avoided as it
may cause further damage. If the tooth cannot be replanted within 5
minutes, it should be stored, in order of preference, in ViaSpan, Hanks’
Balanced Salt Solution, cold milk, saliva, physiologic saline, or water.
Fracture of the tooth enamel alone (a type 1 fracture using the Ellis
classification system), or of the enamel and dentin (Ellis type 2 fracture),
may be managed conservatively (Figures 117-12 and 117-13). Treatment of an Ellis type 3 fracture, involving the pulp (Figure 117-14), includes pulp capping, and partial and complete pulpectomy. A root
fracture, or Ellis type 4 fracture, involves the dentin, pulp, and cementum. In both primary and permanent teeth, the coronal fragment should
be repositioned and stabilized in its anatomically correct position. In primary teeth, alternatively, the coronal fragment may be extracted, allowing the periodontal ligament and neurovascular supply to heal.
Radiographs may be obtained to confirm any of the diagnoses above
or to confirm a tooth avulsion when there is question of an intrusion into
the alveolar bone.
781
FIGURE 117-13. Ellis type II fracture: Bilateral maxillary central incisor injuries
with exposed enamel and dentin consistent with an Ellis class II fracture. (Reproduced with permission from Knoop K, Stack L, Storrow A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, New York. Figure 6-6.)
lacerations of the maxilla usually heal well without intervention. By contrast, the vascularity around the mandibular frenulum often requires primary closure. Tongue lacerations can usually be treated conservatively,
especially if the wound is <1 cm in length, is in the central portion of the
tongue, does not gape, and bleeding is controlled.37 Tongue lacerations
greater than one third of the total diameter and those at the tip causing
forking that may affect speech require suturing.
In general, lacerations limited to the inner mucosal surface heal well on
their own and do not require primary repair. By contrast, full thickness
lacerations and those that disrupt the vermillion border require suturing.
■ CARIES, GINGIVITIS, AND NEGLECT
Large lacerations of the gingiva should be repaired using absorbable sutures. These wounds can allow entry of foreign bodies, such as food particles, dental fillings, and tooth fragments, leading to infection. Frenulum
Defined as the “willful failure of parent or guardian to seek and follow
through with treatment necessary to ensure a level of oral health essential for adequate function and freedom from pain and infection,” dental
neglect is a form of child abuse probably underreported by medical providers. Poor oral hygiene may be secondary to family isolation, lack of finances, parental ignorance, unfluoridated water, bottle-propping, or
lack of perceived value of oral health, and clinicians should determine
whether one or more of these situations are contributing factors.38
FIGURE 117-12. Ellis type I fracture: Note the fracture of the left upper central
incisor. The sole involvement of the enamel is consistent with an Ellis type I injury.
(Reproduced with permission from Knoop K, Stack L, Storrow A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, New York. Figure 6-5.)
FIGURE 117-14. Ellis type III fracture: A fracture demonstrating blood at the
exposed dental pulp. This sign is pathognomonic for an Ellis class III fracture.
(Reproduced with permission from Knoop K, Stack L, Storrow A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, New York. Figure 6-7.)
■ SOFT TISSUE INJURIES OF THE MOUTH
782
SECTION 12: Pediatrics
Children are particularly susceptible to developing caries soon after
the initial eruption of teeth if care is not taken to properly examine and
clean the new teeth. “Baby bottle” caries occur in 24% to 28% of all children aged 2 to 5 years old.39 Risk factors include prolonged breast or bottle feeding (beyond 12 months), prolonged pacifier use, frequent
consumption of beverages high in sugar, and use of a bottle at bed time.
Parents should be encouraged to minimize beverage choices high in
sugar content. Teeth should be cleaned daily from 6 months to 24
months of age with a soft toothbrush and increased to twice a day thereafter. Transition to a training cup by 1 year of age and removal of the bottle can also significantly reduce the occurrence of caries. Additionally, an
initial screening dental examination between 12 and 18 months of age is
recommended to look for signs of decay or a need for fluoride supplementation. In communities without fluoridated water, supplemental fluoride should be prescribed by the primary care provider.
Gingivitis is inflammation of the gums that presents as tender, erythematous, often ulcerated or vesiculated areas of tissue. It is seen mainly in the
setting of poor dental hygiene but can occur with viral and bacterial infections, certain medications [e.g., phenytoin (Dilantin)], or even as a presentation of leukemia. Although there are many causes of gingivostomatitis,
viral infections are particularly common in the pediatric population.
Acute necrotizing ulcerative gingivitis (ANUG) is a progressive infection of the gingiva, leading to pain, significant edema, and ulceration. The
incidence typically peaks in the teens to early 20s but may be seen in younger children in developing countries due to poor access to adequate dental
care or malnutrition. Other factors that may predispose patients to ANUG
include smoking, immunosuppression, viral infections, stress, and sleep
deprivation. Patients present with fever, halitosis, decreased appetite, and
generalized malaise. ANUG is a mixed infection that includes spirochetes,
specifically, Prevotella intermedia. Untreated, ANUG can spread beyond
the gingiva to involve deeper tissues or the tissues of the mouth floor (Ludwig angina) or face. Treatment consists of analgesia to facilitate better oral
hygiene and antimicrobial oral rinses. Patients with more extensive disease
or systemic symptoms may require admission for local debridement and
parenteral antibiotic therapy with penicillin or metronidazole. The patient
should be referred to a dentist for close follow-up care.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
118
Neck Masses in Children
Osama Y. Kentab
Nadeemuddin Qureshi
Neck masses are a common complaint seen in children presenting to the
ED. Ninety percent are benign in nature.1 Congenital, inflammatory,
and malignant lesions make up most of the masses in the pediatric head
and neck region.2,3 This chapter provides an overview of pediatric neck
masses and outlines a simplified approach to their clinical diagnoses and
management.
Neck masses can be classified by anatomic site of presentation (Figure
118-1) or by pathology. Table 118-1 presents a combined approach that
is practical for the ED.
INFLAMMATORY NECK MASSES
■ PRESENTATION AND GENERAL APPROACH
Neck swelling, even when asymptomatic and incidentally discovered, is
often of great concern to the caretaker. Some inflammatory masses are insidious, and others may progress rapidly. Infectious causes must be distin-
Digastric
Hyoid bone
Omohyoid
Carotid triangle
Digastric
triangle
Occipital
triangle
Sternocleidomastoid
Muscular
triangle
Trapezius
Omohyoid
Subclavian
triangle
FIGURE 118-1. Neck anatomy and cervical triangles.
guished from noninfectious causes. Associated symptoms of fever, chills,
pain, recent upper respiratory or GI infections help to identify infection. A
family history of illnesses and sick contacts, exposure to domestic animals
(particularly cats), and diet (exposure to undercooked meat) also suggest
infection. Determine immunization status. Note the size, location, and feel
of the mass, the color of overlying skin, its relationship to underlying
structures, its mobility, and whether the mass is tender or warm, firm, rubbery, or fluctuant. Benign reactive lymph nodes are usually mobile, firm,
TABLE 118-1 Neck Masses in Children
Inflammatory
Neck Masses
Cystic Neck Masses
Cervical
lymphadenopathy
Congenital
malformations
Suppurative lymph
nodes
Granulomatous disease
Mycobacterium
tuberculosis
Atypical
mycobacterium
Cat scratch disease
Toxoplasmosis
Sarcoidosis
Histoplasmosis
Actinomycosis
Fungal infection
Sialoadenitis
Solid Neck Masses
Benign lesions
Fibromatosis colli
Thyroglossal duct
Inflammatory adenopathy
cysts
Hemangioma
Branchial apparatus
Lipoma/lipoblastoma
cyst
Neurofibroma
Cysts
Paraganglioma
Sinuses
Goiter
Fistulas
Salivary gland masses
Dermoid cysts
Epidermal inclusion cysts
Teratomas
Teratoma
Lymphatic
Hematomas
malformations
Subcutaneous emphysema
Lymphangioma
Drugs
(cystic hygroma)
Phenytoin
Thymic cysts
Allopurinol
Bronchogenic cysts
Hydralazine
Laryngoceles
Malignant lesions
Lymphoma
Rhabdomyosarcoma
Neuroblastoma
Metastatic adenopathy
Thyroid cancer
CHAPTER 118: Neck Masses in Children
not attached to undersurfaces, and mildly tender; cystic masses are usually
soft, ballotable and mobile masses; malignant lesions are more frequently
hard, nontender, and may be fixed to underlying structures and therefore
immobile.
Further diagnostic studies are directed by the clinical presentation.
Localized, uncomplicated cervical lymphadenitis, for instance, usually
does not require further investigation and may be empirically treated
with antibiotics. If the mass does not regress with antibiotics, blood testing (complete blood count, erythrocyte sedimentation rate, cultures, serologies) or imaging with CT, MRI, or US may be needed. Some masses
may require tissue diagnosis with fine needle aspiration or biopsy, and
others may require incision and drainage with staining and culture of
purulent material. Subacute and chronic cases may be evaluated with
special testing for Bartonella henselae, mycobacteria, or fungi. Skin testing for tuberculosis may be indicated.
■ CERVICAL LYMPHADENOPATHY
Cervical lymphadenopathy is a common, frequently normal finding in
children. Approximately 55% of healthy children in all age groups have
palpable cervical nodes that are not associated with acute infection or
systemic illness.4 In children, most enlarged lymph nodes are related to
an inflammatory process, not malignancy. Lymph nodes <3 mm in diameter are normal.5 Cervical nodes ≤1 cm in diameter are normal in
783
children >12 years of age, and small nodes in the anterior cervical triangle are usually benign.6 Lymphadenitis, on the other hand, is an infectious process and is a leading cause of inflamed lymph nodes (Figure
118-2).2 Viral infections, particularly of the upper respiratory tract, are
the most common cause of lymphadenitis in children. These include Epstein-Barr virus, cytomegalovirus, varicella zoster, adenovirus, rhinovirus, enteroviruses, herpes simplex virus, and human immunodeficiency
virus/acquired immunodeficiency syndrome. Some bacteria can cause
cervical lymphadenitis as well, most commonly group A Streptococcus
and Staphylococcus aureus. Table 118-2 provides a description of common presentations of cervical adenitis, their causes, and clinical features.
■ CERVICAL LYMPHADENITIS
Most cases of cervical lymphadenopathy or lymphadenitis do not require specific therapy, as they often represent reactive enlargement or viral infection. Suppurative cases may be treated with empiric therapy for
suspected bacterial infections and should cover S. aureus and Streptococcus pyogenes. Amoxicillin plus clavulanic acid (Augmentin), 30 to 40
milligrams/kg/d divided twice a day, or clindamycin (30 to 40 milligrams/kg/d divided three to four times a day) for 10 to 14 days are firstline therapy. Failure to improve in 36 to 48 hours should prompt reassessment of diagnosis and therapy. Culture of the potential infectious
source may guide effective antimicrobial treatment. The presence of a
TABLE 118-2 Clinical Features of Cervical Lymphadenitis
Clinical
Presentation
Common
Organism
Acute unilateral Staphylococcus
cervical
aureus
lymphadenitis Streptococcus
pyogenes
Group B
Streptococcus
Acute bilateral Epstein-Barr virus
cervical
Herpes simplex
lymphadenitis virus 1 and 2
Cytomegalovirus
Adenovirus
Enterovirus
Rubella virus
Roseola virus
Varicella-zoster
virus
Influenza virus
Parainfluenza
virus
Respiratory
syncytial virus
Chronic
Bartonella
unilateral
henselae
cervical
Mycobacterium
lymphadenitis tuberculosis
M. aviumintracellulare
M. scrofulaceum
Toxoplasma gondii
FIGURE 118-2. Cervical lymphadenopathy in a 6-year-old girl with infectious
mononucleosis. (Reproduced with permission from Shah BR, Lucchesi M: Atlas of
Pediatric Emergency Medicine. © 2006, McGraw-Hill, New York. Figure 11-14.)
Features
2–6 cm, tender with overlying skin
erythema.
“Cellulitis-adenitis syndrome” in neonates
usually caused by group B Streptococcus.
Small rubbery mildly tender lymph nodes.
Posterior acute bilateral cervical lymphadenitis mostly seen with rubella and infectious mononucleosis.
Mycoplasma pneumonia and S. pyogenes
can present as bilateral lymphadenitis.
Bartonella (“occuloglandular fever”): Classically results from a kitten scratch on an
extremity followed by ipsilateral cervical
and axillary lymphadenitis and ipsilateral
conjunctivitis. May also present as a
chronic solitary cervical lymphadenopathy.
Mycobacteria: Initially a papule or pustule
at the inoculation site followed by development of lymphadenopathy, thinning of
the overlying skin with color changes
from red to lilac; spontaneous drainage
or sinus formation is common.
784
SECTION 12: Pediatrics
fluctuant infectious mass (abscess) requires incision and drainage, as antibiotic therapy alone is insufficient. Chronic cases of lymphadenitis are
often treated surgically, although antimicrobial treatment is indicated in
some cases. Specific inflammatory conditions, their presentation, evaluation, and individual management, are described in greater detail below.
■ SUPPURATIVE LYMPHADENITIS
Infected lymph nodes may undergo liquefactive necrosis, known as suppurative lymphadenitis. Affected nodes are typically tender and fluctuant. S.
aureus and group A Streptococcus are the most common organisms associated with suppurative lymphadenitis (Figure 118-3). Unilateral node involvement is often secondary to primary infection of the tonsils or the
pharynx, whereas bilateral involvement is more common with viral infections.6 When associated with symptoms such as torticollis or trismus, suspect retropharyngeal abscess, and obtain a contrast CT scan to define the
extent of infection (see Chapter 119, Stridor and Drooling). Complications
of retropharyngeal abscess include spontaneous rupture and mediastinitis.
Treatment typically involves systemic antibiotics (e.g., clindamycin) and
may require surgical consultation for drainage. Resolution of cervical adenopathy may take weeks to months or may persist indefinitely, but should
not exhibit signs of acute inflammation or infection after treatment.
■ GRANULOMATOUS DISEASE
Mycobacterium tuberculosis and atypical mycobacteria cause insidious
and chronic infections of the cervical lymph nodes. These infections are
most common in children 1 to 5 years old and are rare after the age of 12
years old. Affected nodes are usually submandibular or preauricular and
may spontaneously drain. Treatment is surgical excision. Other causes of
granulomatous lymphadenitis include Bartonella henselae (cat scratch
disease), toxoplasmosis, sarcoidosis, histoplasmosis, actinomycosis, and
other fungal infections. Treatment should be begun only after definitive
diagnosis is made. Empirical treatment is not advised.
■ CAT SCRATCH DISEASE
Cat scratch disease is caused by infection with B. henselae (formerly Rochalimaea henselae) a gram-negative bacterium. Although B. henselae may
cause a subacute or chronic unilateral lymphadenitis, it is often associated
with fever, headache, malaise, and anorexia. The term Parinaud oculoglandular fever consists of tender regional adenopathy (epitrochlear, axillary,
preauricular), fever, and ipsilateral conjunctivitis. Other systemic complications are rare but may include encephalitis, seizures, liver or spleen infection, and even bone infections and endocarditis. Diagnosis depends on
a careful history, including exposure to cats (kittens are more likely to car-
ry the bacterium), and is confirmed with antibody titers, polymerase chain
reaction, or culture from lymph node aspirates. Testing is positive in 88%
of subjects with cat scratch disease.7 Empiric treatment is not recommended. Most infections resolve on their own in 2 to 5 months, with local treatment (heat) and analgesics or surgical drainage. Antibiotics active against
the organism include rifampin (preferred in children),8 erythromycin, trimethoprim-sulfamethoxazole, and doxycycline, but clear benefit to treatment has not been shown. Doxycycline is contraindicated in growing
children because of its effects on teeth and bones.
■ TOXOPLASMOSIS
Toxoplasma gondii is a protozoan parasite with a complex life cycle that
can infect humans through ingestion of undercooked meat, exposure to
oocysts in cat feces, or through maternal-fetal transmission (congenital
toxoplasmosis). Infection with the parasite is widespread: an estimated
22.5% of children in the U.S. are infected by the age of 12 years old; in some
countries, up to 95% of the population has been infected. Most infections
are asymptomatic in healthy people, although acute infection may cause
fever, myalgias, malaise, sore throat, and cervical or other lymphadenopathy. The lymphatic system is the most common organ system involved.
Immunocompromised patients may develop reactivation and are prone to
central nervous system and ocular disease. Diagnosis is usually made serologically with antibody titers. Direct staining of aspirated or biopsied
lymph nodes may also demonstrate the parasites. Treatment is not typically needed in healthy children, although symptomatic infection responds to
pyrimethamine plus either sulfadiazine or trisulfapyrimidines.
■ SIALOADENITIS
Most sialoadenitis (infection of the salivary glands) is bacterial, although
viruses (such as the mumps virus) may also cause infection of the glands
and local nodes. Usual organisms include S. aureus and Streptococcus, as
well as gram-negative and anaerobic bacteria. Careful examination reveals swelling over the angle of the mandible and the face, which distinguishes parotitis from cervical adenitis. Submandibular glands may also
become infected, often in association with calculi that cause obstruction
of the duct and enlargement of surrounding submandibular lymph nodes
(Figure 118-4A). Fever, pain, and chills are often associated with acute
suppurative sialoadenitis. The diagnosis is usually clinical, with tenderness
over the affected salivary gland and pus at the duct orifice: Stensen duct
drains the parotid gland and is located adjacent to the upper second molar
(Figure 118-4B); the submandibular gland is drained through the Wharton duct, which is adjacent to the lingual frenulum (Figure 118-4C). Imaging with CT or US is usually reserved for patients in whom the
diagnosis is unclear. Gram stain may guide therapy, which usually includes antibiotics, analgesics, and increasing saliva production with oral
fluids and lemon drops.
CYSTIC NECK MASSES
Table 118-3 lists common congenital neck cysts by location, clinical presentation, and age of onset. The most common (thyroglossal duct cysts,
branchial apparatus cysts, dermoid cysts, and cystic hygromas) are discussed in the following sections.
■ CONGENITAL MALFORMATIONS
FIGURE 118-3. Acute suppurative cervical lymphadenitis in a 5-year-old girl
whose lab tests were positive for group A β-hemolytic streptococci (GABHS).
(Reproduced with permission from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006, McGraw-Hill, New York. Figure 3-2.)
Thyroglossal Duct Cysts Thyroglossal duct cysts (Figure 118-5) are the
most common midline neck masses in children. They account for approximately 70% of the congenital neck masses and are the second most
common benign neck mass after lymphadenopathy.9
Thyroglossal duct cysts are epithelium-lined cysts that result from the
persistence of any segment of the thyroglossal duct along its course from
the foramen cecum of the tongue to the pyramidal lobe of the thyroid.
They are located in the midline of the neck within the anterior triangle.10
Most of the lesions are infrahyoid (65%), whereas suprahyoid cysts account for 20%, and another 15% of lesions are at the level of the hyoid
bone.11,12 The pathognomonic clinical feature is a painless fluctuant mass
CHAPTER 118: Neck Masses in Children
785
FIGURE 118-4. A. Sialoadenitis presenting with painful swelling over the right
parotid gland was confirmed by (B) purulent discharge from Stenson duct after
application of firm pressure on the cheek. C. Sialolithiasis at Wharton duct. (Reproduced with permission from Knoop K, Stack L, Storrow A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, New York. Figures 5-32 and 5-33.)
that moves with swallowing or protrusion of the tongue. As with other
congenital cystic malformations, there may be a history of recurrent
swelling or past infections in the area.
Although CT scan and MRI are both useful, CT provides better visualization of the hyoid bone and pre-epiglottic fat compared with MRI
TABLE 118-3 Common Congenital Neck Cysts by
Location, Signs, and Age of Onset
Type
Location
Signs
Age
Thyroglossal
duct
Dermoid/
epidermoid
Branchial
Midline (infrahyoid
mostly)
Midline
(suprahyoid)
Anterior triangle
near angle of the
mandible
Lateral neck
Cyst moves with swallowing or tongue protrusion.
Cyst may move with swallowing.
Associated with draining
sinus.
Birth—elderly
Size fluctuates.
Transilluminates.
Soft, enlarges in first few
weeks of life.
Infancy—adult
Laryngocele
Cystic
hygroma
Posterior triangle
Birth—adult
Childhood—
adult
Birth—infancy
and may be useful in planning surgical excision. On US, the cysts are well
defined, thin walled, anechoic, and homogenous. Mixed echogenicity on
US suggests infection of the cyst.11 Evaluation of thyroid function and
thyroid gland position are important because cysts may contain ectopic
thyroid tissue.13,14 Acutely infected cysts require antibiotics. Surgical excision is done after infection subsides.
Branchial Apparatus Cysts The branchial apparatus consists of six branchial arches separated by five branchial clefts and appears by the 15th day
of intrauterine life. Incomplete obliteration of the branchial apparatus,
predominately the cleft, is postulated to lead to branchial cleft anomalies
such as cysts, sinuses, or fistulae.10 Almost 75% of these cysts arise from
remnants of the second branchial cleft.12 Cysts are masses situated anterior to the sternocleidomastoid (SCM) muscle near the angle of the mandible. The cysts are round, smooth, and mobile, but are not tender unless
they become infected (Figure 118-6). There may be a history of recurrent swelling or infection in the same area. The cyst may spontaneously
rupture, forming an external sinus or fistula along the anterior border of
the SCM muscle. The diagnosis is clinical but can be confirmed with US,
which shows a thin-walled, anechoic fluid-filled cyst.11 CT or MRI may
be useful in planning the surgical excision. Oral antibiotics are indicated
for infected cysts before surgery.
Dermoid Cysts/Teratomas Dermoid cysts or teratomas are developmental
anomalies involving pluripotent embryonal stem cells and occur most of-
786
SECTION 12: Pediatrics
FIGURE 118-6. Axial CT scan of a branchial cleft cyst. (Reproduced with permission from Lalwani A: Current Diagnosis and Treatment in Otolaryngology, 2nd ed.
© 2008, McGraw-Hill, New York. Figure 26-3.)
SOLID NECK MASSES
■ BENIGN LESIONS
FIGURE 118-5. Thyroglossal duct cyst in an adolescent girl.
ten in children <3 years old. Dermoid cysts are usually suprahyoid15 and
midline, and are often misdiagnosed as thyroglossal duct cysts.14,16 Dermoid cysts are mobile, but they do not move with tongue protrusion,
which helps to differentiate them from thyroglossal duct cysts. CT and
MRI are used for diagnosis.10,15 Management is surgical excision.
Fibromatosis Colli Fibromatosis colli presents in the neonatal period as
a mass in the SCM muscle. Histologically, part of the involved muscle is
replaced by dense fibrous tissue.19 Parents will often notice the mass in
the first weeks of life and may notice limited range of motion of the neonate’s neck (torticollis). There may be a history of birth trauma. Physical
examination reveals a firm, solid, immobile mass located within the
SCM muscle itself, often associated with some degree of torticollis. The
mass moves with the muscle when the head is turned on examination.
The diagnosis can often be made clinically or confirmed by US. The natural history is spontaneous resolution over a period of 4 to 8 months, and
gentle stretching or physical therapy is the only treatment needed.12,19
Hemangioma Hemangiomas are congenital vascular malformations. Infantile hemangiomas usually grow rapidly until 9 to 10 months of age, af-
■ LYMPHATIC MALFORMATIONS
Lymphangioma (Cystic Hygroma) Cystic hygromas result from sequestration of lymphatic channels that fail to communicate with the internal
jugular system, leading to blockage of the lymphatic channels.17 They
can occur anywhere in the body, but nearly 75% arise within the neck,
most commonly along the jugular chain of lymphatics. The term cystic
hygroma is applied to all lymphangiomas that may have capillary, cavernous, and cystic components coexisting within the same lesion.18 Cystic hygromas are soft, painless, and compressible, and can be very large
(Figure 118-7). Morbidity associated is usually secondary to compression of surrounding structures, and large neck lesions may cause serious
airway and feeding problems. Sixty-five percent present at birth, and
90% are clinically detected by the end of the second year.11,17 Most of
these lesions are diagnosed prenatally by the use of US and are slow
growing. Sudden enlargement can occur, however, from infection or
hemorrhage into the lesion. Careful inspection of the oral cavity and palpation of the trachea for deviation is important in assessing the airway of
patients with large cystic hygromas. The margins of the mass and the
presence of mediastinal extension is best visualized with CT or MRI.
Treatment consists of watchful waiting in asymptomatic patients, although tracheostomy may be required to protect the airway in large, inoperable lesions. Injection of sclerosing agents can be used in some
lesions, although surgical excision is definitive treatment.17
FIGURE 118-7. Cystic hygroma presents as a bright, supraclavicular, soft, and
compressible mass. (Reproduced with permission from Knoop K, Stack L, Storrow
A: Atlas of Emergency Medicine, 2nd ed. © 2002, McGraw-Hill, New York. Figure
14-35.)
CHAPTER 118: Neck Masses in Children
787
ter which spontaneous regression is the norm, although involution may
take up to 10 years. Hemangiomas of the head and neck may compromise the airway as they enlarge, presenting as respiratory distress often
associated with biphasic stridor unrelated to upper respiratory infection
and unresponsive to racemic epinephrine. When palpable, they are soft,
mobile, and frequently have a bluish hue.20 Suspected airway lesions may
require endoscopy by a pediatric otolaryngologist. Visible lesions are
best characterized with CT or MRI. Almost 90% of hemangiomas resolve
spontaneously without the need for therapy. Lesions showing unusually
rapid growth, hemorrhage, recurrent infection, or compression of adjacent structures may require treatment by a specialist, including steroids
and laser or surgical excision.
Neurofibroma/Schwannomas Neurofibromas and schwannomas are rare
usually large tumors often involving the orbits, the skull base, or the parotid region, and are almost always associated with neurofibromatosis
type I, which includes multiple café au lait spots (Figure 118-8), plexiform neuromas, and schwannomas. The best modality for diagnosis and
characterization of the lesion is MRI.
Goiter Children with diffuse enlargement of the thyroid gland usually
have symptomatic thyrotoxicosis or Hashimoto thyroditis,21 although
goiter can be seen in some parts of the world (Figure 118-9). Most cystic
masses in the region of the thyroid gland are thyroglossal cysts. Thyroid
masses are evaluated with thyroid function studies, sonography, nuclear
scintigraphy, and biopsy.22
■ MALIGNANT LESIONS
A few rare malignancies in children may present to the ED because of a
neck mass. The first step in evaluation is a careful history and physical
examination to identify the location of the mass (midline, anterior, or
posterior triangle).24,25 The anterior triangle extends from the undersurface of the mandible to the junction of the SCM muscle and the sternoclavicular joint. The posterior triangle extends posterior to the SCM
muscle from the clavicle to the base of the mastoid, with the trapezius
muscle defining the posterior border.
A solitary posterior cervical mass suggests malignancy.26 In a study of
4768 patients with nasopharyngeal carcinoma, asymptomatic posterior
triangle neck mass was the most common presenting symptom, occurring
in 76% of patients.27 Supraclavicular masses suggest lymphomas4 or malignant metastasis from areas below the clavicle (lungs, GI, or gynecologic).28 By contrast, central midline swelling suggests the thyroid with
ducts, cysts, or dermoids as likely diagnoses.
The most common childhood malignancies presenting as neck masses
are briefly reviewed below.
Lymphoma Lymphoma is the most common malignancy of the head and
neck in children (see Chapter 136, Oncology and Hematology Emergen-
FIGURE 118-8. Café au lait spots in neurofibromatosis. (Reproduced with permission from Shah BR, Lucchesi M: Atlas of Pediatric Emergency Medicine. © 2006,
McGraw-Hill, New York. Figure 7-61.)
FIGURE 118-9. Goiter resulting from Graves disease in a 16-year-old girl. (Reproduced with permission from Shah BR, Lucchesi M: Atlas of Pediatric Emergency
Medicine. © 2006, McGraw-Hill, New York. Figure 14-3.)
cies in Children).10 Almost 50% of head and neck lymphomas are Hodgkin
disease, which usually presents in teenagers. Neck mass is the primary presenting sign in 80% of children with Hodgkin disease and 40% of children
with non-Hodgkin lymphoma. Non-Hodgkin lymphoma primarily affects
children between 2 and 12 years of age, and extranodal involvement is common, occurring more commonly in patients with acquired or congenital
immune disorders. Burkitt lymphoma is a non-Hodgkin lymphoma and is
considered the fastest growing human tumor. Most lymphomas present as
large, firm, mobile masses, commonly located in the anterior triangle or the
supraclavicular area. CT and MRI are used for diagnosis.29
Rhabdomyosarcoma Rhabdomyosarcoma is the second most frequent
malignancy of the head and neck in children.10,29 This disease has a peak
presentation at 2 to 5 years old and again at 15 to 19 years old, with a slight
male predominance. Tumors can involve the orbit, nasopharynx, middle
ear, nasal cavity, and paranasal sinuses.30 Neck tumors may present with
brachial plexus palsy. Contrast-enhanced CT and MRI are the best imaging modalities for diagnosis and staging.24 For further discussion, see
Chapter 136, Oncology and Hematology Emergencies in Children.
Neuroblastoma Neuroblastoma is a malignancy of the sympathetic chain
and can originate anywhere along its path, although the adrenal glands are
the most common primary site.12,31 Most present before the age of 5 years
old, and those arising in the neck (5%) have a better prognosis than those
tumors of adrenal origin.10 Neuroblastoma can rarely present as an
asymptomatic mass, alone or with local compressive signs such as hoarseness, dysphagia, airway obstruction, Horner syndrome, or cranial nerve
palsies. Diagnostic imaging includes US, which typically shows an echogenic mass; CT and MRI provide better detail.32 For further discussion, see
Chapter 136, Oncology and Hematology Emergencies in Children.
788
SECTION 12: Pediatrics
Metastatic Adenopathy Malignant cervical lymphadenopathy may also
represent metastatic disease. Common tumors that metastasize to the
neck include nasopharyngeal carcinoma and some GI tumors. CT and
MRI are best to diagnose and define extension of the tumor.29 The location of the metastasis may give a hint to the origin of the primary tumor:
posterior triangle nodes are often seen in nasopharyngeal carcinoma,
whereas isolated supraclavicular nodes suggest mediastinal mass.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
119
Stridor and Drooling
Joseph D. Gunn III
Stridor is a high-pitched, harsh sound produced by turbulent airflow
through a partially obstructed airway. Both inspiratory and expiratory
stridor are associated with obstruction of the airway. Two important
physical principles influence the clinical presentation of patients with
stridor. As air is forced through a narrow tube, it undergoes a decrease
in pressure (the Venturi effect). This decrease in lateral pressure causes
the airway walls to collapse and vibrate, generating stridor. The second
physical principle is airway resistance. Resistance is inversely proportional to the fourth power of the airway radius. This translates into a 16fold increase in resistance when the radius is reduced by half. Even 1 mm
of edema in the normal pediatric subglottis reduces its cross-sectional
area by >50%. Thus, a small amount of inflammation can result in significant airway obstruction in children.
Immediately assess the child with stridor, as respiratory compromise
may require maneuvers to secure the airway. The presence of stridor
constitutes a difficult airway, and advanced airway management may be
necessary (see Chapter 29, Pediatric Airway Management). A thorough
history and examination will often lead to a “working diagnosis.” If time
permits, ask about the time and events surrounding the onset of stridor,
the presence of fever, known congenital anomalies, perinatal problems,
prematurity, and previous endotracheal intubation.
The level of obstruction can often be identified on examination. Partial
obstruction of the upper airway at the nasopharynx and oropharyngeal
levels produces sonorous snoring sounds known as stertor. Obstruction
of the supraglottic region may cause inspiratory stridor or stertor. Obstruction of the glottis and subglottic and tracheal areas often cause both
inspiratory and expiratory stridor. Consider airway foreign body until
proven otherwise if there is marked variation in the pattern of stridor.
The noise made by a child with stridor is often interpreted as wheezing by
parents unfamiliar with stridor. Clarify what the parent means when the
word “wheezing” is used—whether the sound occurs when the child
breathes in or breathes out. The provider can imitate a stridor sound to
help ED diagnosis. The differential diagnosis of stridor depends upon the
child’s age (Table 119-1).
STRIDOR IN CHILDREN <6 MONTHS OLD
An infant <6 months with a long duration of symptoms typically has a
congenital cause of stridor. The major causes are laryngomalacia, tracheomalacia, vocal cord paralysis, and subglottic stenosis. Less common but
important considerations include airway hemangiomas and vascular
rings and slings. Stridor presenting in the first 6 months of life will often
require direct visualization of the airway through endoscopy or advanced imaging. The timing of this evaluation (emergent or outpatient)
is dictated by the severity of symptoms and clinical suspicion.
TABLE 119-1 Causes of Stridor
Children <6 mo of age
Laryngotracheomalacia
Vocal cord paralysis
Subglottic stenosis
Airway hemangioma
Vascular ring/sling
Children >6 mo of age
Croup
Epiglottitis
Bacterial tracheitis
Foreign body aspiration
Retropharyngeal abscess
Laryngomalacia, the most common cause of congenital stridor, accounts for 60% of all neonatal laryngeal problems and results from a developmentally weak larynx. Collapse occurs with each inspiration at the
epiglottis, aryepiglottic folds, and arytenoids. Generally, stridor worsens
with crying and agitation but often improves with neck extension and
when the child is prone. Laryngomalacia usually manifests shortly after
birth and generally resolves by age 18 months old. Symptom exacerbations may occur with upper respiratory infections or increased work of
breathing from any cause. Diagnosis can often be made with flexible fiberoptic laryngoscopy. Surgical intervention may be required if a child
suffers from failure to thrive, apnea, or pulmonary hypertension. Surgical management, when required, is based on the pattern of supraglottic
collapse. In many cases, the tracheal support structures are similarly affected, leading to a diagnosis of laryngotracheomalacia.
The next most common cause of neonatal stridor is vocal cord paralysis. This can be congenital or acquired. Unilateral vocal cord paralysis
is more common and presents with feeding problems, stridor, hoarse
voice, and cry changes. Children with bilateral cord paralysis often have
a normal voice associated with stridor and dyspnea. These children are
more likely to present with cyanosis and apneic episodes. Flexible nasolaryngoscopy is the referenced standard for making the diagnosis of vocal cord paralysis. Endotracheal intubation can be difficult in patients
with bilateral cord paralysis. Needle cricothyroidotomy and subsequent
tracheotomy may be required to secure the airway.
Subglottic stenosis may be acquired or congenital and is diagnosed
when there is a narrowing of the laryngeal lumen. Congenital stenosis is
usually diagnosed in the first few months of life when the patient is noted
to have persistent inspiratory stridor. Mild cases may present later in childhood with recurrent or persistent croup. Prolonged endotracheal intubation in premature babies is the most common cause of acquired subglottic
stenosis. Treatment is based on the severity of the stenosis. In most cases,
symptoms from subglottic stenosis resolve by a few years of age.
Hemangiomas are benign congenital tumors of endothelial cells or vascular malformations that can occur anywhere on the body (80% are located above the clavicles), including the airway where they can cause
obstruction and stridor. Hemangiomas typically enlarge throughout the
first year of life, may not be noticed at birth, and tend to spontaneously
regress by age 5 years old. A thorough examination of the skin of an undressed infant is an important aspect of the evaluation of stridor in the
first 6 months of life, as hemangiomas may be multiple, and findings
externally may be a clue to the presence of an airway hemangioma. Definitive diagnosis requires airway visualization through endoscopy. Although most hemangiomas spontaneously regress, large malformations
and those causing significant respiratory symptoms may require treatment with steroids, laser, or surgery.
Vascular rings and slings are rare congenital anomalies of the aortic
arch and pulmonary artery in which abnormal embryonic development
leads to anomalous vessels that can compress the trachea or esophagus.
Examples include a double- or right-sided aortic arch. As with other
congenital causes of stridor, symptoms are often present from birth or
CHAPTER 119: Stridor and Drooling
early in the first month of life and may be progressive and exaggerated
during intercurrent upper respiratory infections; difficulty with feeding
may also occur if the esophagus is compressed. As these anomalies are
rare, a high index of suspicion is required for diagnosis. Chest x-ray may
reveal subtle narrowing or anterior compression of the trachea on the
lateral view, or an abnormal aortic arch. Further evaluation includes
bronchoscopy, CT angiography, and echocardiography to evaluate for
associated congenital heart anomalies. Definitive treatment is surgical.
STRIDOR IN CHILDREN >6 MONTHS OF AGE
The child >6 months old with a relatively short duration of symptoms
(hours to days) characteristically has an acquired cause of stridor.
Causes are either inflammatory/infectious, such as croup or epiglottitis, or noninflammatory, such as a foreign body aspiration. The remainder of this chapter discusses the most common causes of acquired
stridor (Table 119-2).
■ CROUP
Croup (laryngotracheobronchitis) is the most common cause of stridor outside the neonatal period. Children 6 months to 3 years old are
most commonly affected, with a peak in the second year of life. The incidence of croup is highest in the fall and the early winter months, but it
may occur throughout the year. The most common viruses detected in
789
croup include parainfluenza, respiratory syncytial virus, human bocavirus, and rhinovirus.1
Clinical Features Croup is acquired through inhalation of the virus. The
clinical course varies according to the underlying viral etiology, but typically begins with 1 to 2 days of nasal congestion, rhinorrhea, cough, and
low-grade fever before the onset of classic croup symptoms. These classic
symptoms include a harsh cough often described as barking or “like a
seal,” hoarse voice, and stridor. Parents may also report inadequate oral intake. Symptoms are often perceived to be worse at night. The severity of
symptoms is related to the amount of edema and inflammation of the airway. Assess for tachypnea, stridor at rest, nasal flaring, retractions, mental
status (lethargy or agitation), and oxygen desaturation. The “typical” duration of symptoms ranges from 3 to 7 days. Children generally have the
most severe symptoms on the third and fourth days of illness and subsequently begin to improve.
Diagnosis The diagnosis of croup is clinical. Laboratory tests are generally unnecessary. Plain radiographs are not required in uncomplicated
croup and should be reserved for evaluating children with findings suggestive of another diagnosis, such as epiglottitis, retropharyngeal abscess, and aspirated foreign body. If obtained, radiographic findings on
posteroanterior chest radiograph in patients with croup may demonstrate subglottic narrowing (“steeple sign”) (Figure 119-1). This finding
is unreliable, however, and may be present in normal children, and absent in up to 50% of children with croup.
TABLE 119-2 Common Acquired Causes of Stridor
Viral Croup
Etiology
Age
Bacterial Tracheitis
Epiglottitis
Peritonsillar
Abscess
Retropharyngeal
Abscess
Foreign Body
Aspiration
Polymicrobial
S. pyogenes
S. aureus
Gram-negative rods
Oral anaerobes
6 mo–4 y old
Rare >4 years
Variable
Foods
Peanuts
Sunflower seed
Balloons/other toys
Any
6 mo–5 y old most
common
Parainfluenza viruses Staphylococcus aureus
(occasionally respira- (most)
tory syncytial virus and S. pneumoniae
rhinovirus)
Haemophilus influenzae
Streptococcus
pneumoniae
S. aureus
H. influenzae
Polymicrobial
S. pyogenes
S. aureus
Oral anaerobes
6 mo–3 y old
Peak 1–2 y old
All ages
Classically 1–7 y old
10–18 y old (most)
6 mo–5 y old (rare)
Onset
1–3 d
Effect of
positioning
on symptoms
None
Stridor
Inspiratory and
expiratory
Seal-like bark
3 mo–13 y old
Mean, 5–8 y old
2–7 d viral upper
respiratory infection
Suddenly worse over
8–12 h
None
80% <3 years
Insidious over 2–3 d after Immediate or delayed
an upper respiratory
possible
infection or local trauma
Rapid, hours
Antecedent
pharyngitis
Worse supine
Prefer erect, chin
forward
Inspiratory
Worse supine
Neck stiffness and
hyperextension
Usually none
Location-dependent
Uncommon
Inspiratory when severe
Location-dependent
No
No
No
Often transient or
positional
Muffled
“Hot potato”
Muffled
“Hot potato”
Often muffled
“Hot potato”
Location-dependent
Primarily if at or above
glottis
Rare—often if esophageal
Rare—typically if
esophageal
Often normal
Possible radiopaque
density
Ball-valve effect
Segmented atelectasis
Voice
Hoarse
Not muffled
Inspiratory and
expiratory
Usually
Possible thick sputum
Usually normal
Possibly raspy
Drooling
Dysphagia
No
Occasional
Rare
No
Yes
Yes
Often
Yes
Yes
Yes
Radiologic
appearance
Subglottic narrowing
“steeple”
Subglottic narrowing
Irregular tracheal
margins
Enlarged epiglottis
Thickened aryepiglottic folds
May see enlarged
tonsillar soft tissue
Thickened bulging retropharyngeal soft tissue
Cough
790
SECTION 12: Pediatrics
FIGURE 119-1. Anteroposterior neck radiograph in patient with croup; note presence of the “steeple sign” (arrow). (Courtesy of W. McAlister, MD, Washington University School of Medicine, St. Louis, MO.)
Treatment Croup is often classified as mild, moderate, or severe (Table
119-3), and treatment is directed primarily at mitigating airway obstruction. Although numerous scoring systems for croup have been published, in general, these are more useful as research tools than for clinical
practice. Their primary role is to provide a semiobjective scale by which
to classify patients for comparative studies, and their usefulness as a tool
for clinical decision making with individual patients is much less clear.
TABLE 119-3 Assessment of Croup Severity
Mild
Moderate
Severe
Occasional barking
cough
No audible stridor
at rest
Mild or no chest wall/
subcostal retractions
No agitation and
distress
Frequent barking
cough
Easily audible stridor
at rest
Chest wall/subcostal
retractions at rest
Little or no agitation
and distress
Frequent barking cough
The score, if calculated, should only be used as one piece of data in the
decision-making process.
Most children with croup are not brought for medical attention. Children with croup presenting to the ED should be placed in a position of
comfort, often in the lap of the caretaker. Assess respiratory distress through
observation, without disturbing the child. Agitation and crying increase oxygen demand and may worsen airway compromise. Humidified air or cool
mist do not improve clinical symptoms.2,3 Current standard treatment is the
use of nebulized epinephrine and corticosteroids (Table 119-4). Nebulized
epinephrine is the mainstay of treatment for moderate to severe croup
patients with marked retractions and stridor at rest. Mild croup generally does not require epinephrine. All patients with croup benefit from
the administration of oral steroids as a one-time dose. Those with
moderate or severe croup who require nebulized epinephrine should
be observed in the ED for 3 hours before considering discharge.4
Epinephrine Studies comparing L-epinephrine with racemic epinephrine
show no significant difference in response.5,6 Epinephrine works by rapidly decreasing airway edema through vasoconstrictive alpha effects.
Clinical effects of epinephrine are seen in as few as 10 minutes and last
for more than 1 hour.7,8 Use of epinephrine decreases the number of
children with croup requiring intubation, intensive care unit admission,
and admission to the hospital in general.
ED observation for about 3 hours is recommended. Ledwith’s group
monitored patients for 3 hours after epinephrine nebulization and found
that 38% of the patients who had a recurrence requiring admission did so
between the second and third hour.9 Prendergast et al. also demonstrated
that there was an upward trend in the croup score between the second and
third hours in those patients ultimately requiring admission.10
β Agonists such as albuterol should not be used for croup or when
signs of upper airway obstruction from edema are present (i.e., stridor). Vascular β-receptors cause vasodilation that may worsen edema
and exacerbate upper airway obstruction.
Corticosteroids Children with mild, moderate, and severe croup all benefit
from corticosteroids, which reduce both the severity and duration of croup
episodes.11,12 Corticosteroids improve symptoms by anti-inflammatory effects in the upper airway. Studies demonstrating the efficacy of steroids
against placebo have used dexamethasone and nebulized budesonide. Dexamethasone is equally effective if given parenterally or orally. Most clinicians initially prescribe oral corticosteroids because of ease of administration.
Oral administration of the IV formulation of dexamethasone has the advantage of lower volume due to increased concentration when compared with
the oral preparation, and may be associated with less vomiting. Nebulized
budesonide and IM dexamethasone are alternatives to PO dexamethasone in
children who are vomiting. Because even patients with very mild croup
symptoms can benefit from steroid administration, most ED patients
diagnosed with croup should be treated with corticosteroids.
Heliox Heliox may be a treatment option in severe, refractory croup. Heliox is a gaseous mixture of helium with oxygen. Replacing nitrogen with
TABLE 119-4 Croup Pharmacotherapy
Medication
Dose
Notes
Dexamethasone
0.15–0.6 milligrams/kg
PO/IM (10 milligrams
maximum)
Budesonide
L-epinephrine
(1:1000)
Racemic
epinephrine
(2.25%)
2 milligrams nebulized
0.5 mL/kg nebulized
(5 mL maximum)
0.05 mL/kg/dose
nebulized (maximum
0.5 mL)
Give for mild, moderate, or severe
croup. May crush pills and mix in
juice or applesauce.
May give IV solution PO without
dilution.
Consider if PO steroids vomited.
Use for moderate or severe croup;
may need repeat dose if severe.
Use for moderate or severe croup;
may need repeat dose if severe.
Prominent inspiratory and
occasionally expiratory stridor
Marked sternal retractions
Agitation and distress
Reproduced with permission from Guideline for the diagnosis and management of croup. Alberta,
ON, Canada: Alberta Medical Association, 2008. Available at: http://www.topalbertadoctors.org/
informed_practice/clinical_practice_guidelines/complete%20set/Croup/croup_guideline.pdf.
Accessed May 14, 2010.
CHAPTER 119: Stridor and Drooling
TABLE 119-5 Criteria for Discharge from ED in Patients with Croup
3 h since last epinephrine
Nontoxic appearance
Able to take fluids well
Caretaker able to recognize change in child’s condition and has adequate transportation to return if necessary
Parents have a phone and no social issues for concern
the less dense helium decreases airway resistance and improves gas flow
through a compromised airway. Heliox is typically given in a 70% helium/30% oxygen ratio. Studies of the use of heliox in croup show no definitive advantage over conventional treatment.13–15
Disposition and Follow-Up Most children with croup can be safely discharged to home (Table 119-5). Observe in the ED for 3 hours after epinephrine administration. Children with persistent stridor at rest,
tachypnea, retractions, and hypoxia or those who require more than
two treatments of epinephrine should be admitted to the hospital. Intubation is reserved for cases of severe croup not responding to medical
treatment. When intubation is necessary, use endotracheal tubes smaller
than recommended for patient size and age to avoid traumatizing the inflamed mucosa.
■ EPIGLOTTITIS
791
that such maneuvers could trigger worsening distress, no documented
reports show this to be unsafe. Patients with suspected epiglottitis who
are initially seen in an office or clinic without pediatric or otolaryngologic subspecialty support should be transported to a referral center accompanied by personnel who can manage the airway.
Lateral neck radiographs are usually unnecessary in patients with the
classic presentation of epiglottitis. When the diagnosis is uncertain, obtain
soft tissue neck radiographs with the neck extended during inspiration. Affected children typically hold their heads in a sniffing position and have
prolonged inspiration already, making it quite simple to obtain radiographs. Lateral neck radiographs may show an enlarged epiglottis protruding from the anterior wall of the hypopharynx (often called the “thumb
sign”) and thickened aryepiglottic folds (Figure 119-2). If suspicion for the
diagnosis still exists despite normal-appearing radiographs, direct visualization of the epiglottis is necessary to exclude the diagnosis (Figure 119-3).
Treatment Keep the child seated and upright in a position of comfort. Provide oxygen. Administer nebulized racemic or L-epinephrine to decrease
airway edema. Alert the referral center or pediatric otolaryngologist as
soon as possible so decisions concerning intubation or tracheotomy can be
made in concert with consultants and support personnel can be mobilized.
Intubation should be done by the most skilled individual available as soon
as the diagnosis is made. Sedation, paralytics, and vagolytics are used as indicated. For a child who is able to maintain an airway, the decision to administer paralytics must be accompanied by absolute certainty that
intubation will be successful. Have multiple endotracheal tube sizes imme-
Epiglottitis, or supraglottitis, is an acute inflammatory condition of the
epiglottis that may progress rapidly to life-threatening airway obstruction.
The epiglottis is a leaf-shaped cartilaginous structure with a thin epithelial
layer. The epiglottis arises from the base of the posterior tongue and covers
the larynx during swallowing. Widespread implementation of an effective
Haemophilus influenzae type B vaccine has significantly reduced the number of cases of childhood epiglottitis. In the postvaccine era, most cases of
infectious epiglottitis are caused by streptococcal and staphylococcal species. Candida species can cause epiglottitis in the immunocompromised
patient. Noninfectious causes, such as thermal injury, caustic burns, and
direct trauma, may cause swelling and inflammation of the epiglottis with
a clinical picture identical to that of infectious epiglottitis in the absence of
fever.
Clinical Features Infection typically presents with the abrupt onset of fever, drooling, and sore throat. Symptoms may progress rapidly, such that
the child may be unable to handle oral secretions and develops stridor
and respiratory distress. Cough is often absent, but the voice may be
muffled. Most children appear toxic and anxious and may assume a tripod or sniffing position with the neck hyper-extended and the chin forward to maintain the airway.
Diagnosis The ideal approach to the diagnosis of epiglottitis varies, depending on the practice and the environment. Each institution should
have a written “suspected epiglottitis management protocol.” Important
components of all protocols are listed in Table 119-6.
In older children and those with mild respiratory distress, gentle direct visualization of the epiglottis may be attempted. Despite concerns
TABLE 119-6 Suspected Epiglottitis Management Protocol
1. Immediate recognition and triage to a resuscitation area
2. Continuous monitoring by someone trained in the management of a difficult
airway
3. Rapid consultation with appropriate colleagues from otolaryngology and
anesthesiology
4. Consideration and risk-benefit analysis of patient transfer with appropriate
personnel present during the transfer
5. Bedside radiology without disturbing the patient or, if moved to the x-ray suite,
constant monitoring by a physician with appropriate airway equipment and
skills
FIGURE 119-2. Lateral neck view of a child with epiglottitis. (Courtesy of W. McAlister, MD, Washington University School of Medicine, St. Louis, MO.)
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SECTION 12: Pediatrics
FIGURE 119-3. Epiglottitis at laryngoscopy. (Reproduced with permission from
Knoop K, Stack L, Storrow A: Atlas of Emergency Medicine, 3rd ed. McGraw-Hill,
New York. Part 2 Specialty Areas, Chapter 14, Pediatric Conditions, Figure 14-38.)
diately available. If endotracheal intubation is unsuccessful, an emergent
surgical airway is required (see Chapter 29, Pediatric Airway Management). Administer a second- or third-generation cephalosporin, such as
cefuroxime or ceftriaxone, to ensure adequate coverage of the most common infectious pathogens. With the increasing incidence of Staphylococcus aureus and highly resistant Streptococcus pneumoniae as a cause of
epiglottitis, one may also empirically add vancomycin to the antibiotic regimen. Antibiotics are typically continued for 7 to 10 days. Steroids are often employed to decrease mucosal edema of the epiglottis.
■ BACTERIAL TRACHEITIS
Bacterial tracheitis, also known as membranous laryngotracheobronchitis
or bacterial croup, is an uncommon infection that can cause life-threatening upper airway obstruction. It can be a primary or secondary infection. The mean age of presentation is now 5 to 8 years of age compared
with the 4 years of age that has been classically described.16,17
Clinical Features Bacterial tracheitis is often a secondary infection after
a viral upper respiratory tract infection. A history of URI symptoms followed by sudden worsening with high fever, stridor, and cough (which
may be productive of thick sputum), and a toxic appearance suggest the
diagnosis. Increasing upper airway obstruction occurs from thick mucopurulent secretions of the trachea. Management is similar to that of
epiglottitis, with patients ideally going to the operating room for sedation, intubation, and bronchoscopy. Cultures and Gram stain of the mucopurulent secretions should be obtained at this time, as Gram stain
findings may help guide the antibiotic therapy. Bronchoscopy may be
therapeutic, as the removal of purulent pseudomembranes improves tracheal toilet and may lessen upper airway obstruction. For continued
management, most patients with bacterial tracheitis require intubation
and ventilatory support.
The most commonly isolated pathogen obtained from culture at bronchoscopy is S. aureus. Other organisms implicated in bacterial tracheitis include S. pneumoniae, S. pyogenes, Moraxella catarrhalis, H. influenzae, and
anaerobes.18,19 Initial antibiotic choices include ampicillin/sulbactam or the
combination of a third-generation cephalosporin and clindamycin. Consider the addition of vancomycin in areas of increasing methicillin-resistant
S. aureus. Laboratory studies other than tracheal cultures are of limited use
in the diagnosis. Neck radiographs are not needed to make the diagnosis.
When obtained to evaluate for other potential diagnostic entities, neck
films may show subglottic narrowing of the trachea and irregular tracheal
margins in patients with tracheitis (Figure 119-4). Because no single clinical or radiographic feature can definitively make a diagnosis, bronchoscopy
is the diagnostic method of choice in bacterial tracheitis.
FIGURE 119-4. Lateral neck view of patient with bacterial tracheitis. Note presence of irregular tracheal margins (arrows). (Courtesy of W. McAlister, MD, Washington University School of Medicine, St. Louis, MO.)
■ AIRWAY FOREIGN BODY
Foreign body aspiration can be a life-threatening emergency that requires immediate intervention. Airway foreign body aspiration occurs
most commonly in children between 1 and 3 years old as a result of increasing mobility and oral exploration. Foreign body aspiration in
children <6 months old often involves a well-meaning sibling who places
an object in the infant’s mouth. The most common objects aspirated fall
into two groups: food and toys. Commonly aspirated foods include peanuts, sunflower seeds, carrots, raisins, grapes, and hot dogs.
A high index of suspicion is needed to diagnose foreign body aspiration. Consider foreign body aspiration in a young child with respiratory symptoms, regardless of the duration of symptoms, because
many children may present >24 hours after foreign body aspiration.
If the clinical scenario clearly indicates the presence of a foreign body or
airway obstruction, immediately implement a protocol for obstructed
airway management. A foreign body aspiration should be highly suspected when there is a history of sudden coughing and choking in the
CHAPTER 119: Stridor and Drooling
793
child. This is the most predictive of all signs and symptoms in foreign
body aspiration.20,21 In many cases, the choking episode is not witnessed by a caregiver.
Clinical Features Although the location of the aspirated foreign body
plays a role in determining the symptoms and signs on presentation,
there is great overlap between groups, and some children may be asymptomatic on presentation. “Classic dogma” is that laryngotracheal foreign
bodies cause stridor and hoarseness, whereas bronchial foreign bodies
cause unilateral wheezing and decreased breath sounds. Eighty percent
to 90% of airway foreign bodies are found in the bronchi. Patients may
present with severe immediate onset stridor or even cardiopulmonary
arrest, but a significant proportion will have no cough, wheeze, or stridor. The most important factor in reducing mortality from an airway
foreign body is the recognition of the child in acute airway distress.
Diagnosis Radiographs are helpful to confirm the diagnosis of airway
foreign body but should not be used to exclude the diagnosis, as plain
chest radiographs are normal in >50% of tracheal foreign bodies and
one fourth of bronchial foreign bodies.22 Anteroposterior and lateral
neck radiographs are the radiographic examinations of choice to identify
laryngeal and tracheal foreign bodies. Suspected bronchial foreign bodies
can be evaluated with the use of posteroanterior and lateral chest films
(Figure 119-5). More than 75% of airway foreign bodies in children <3
years of age are radiolucent.20,23–25 Indirect radiologic signs of a radiolucent airway foreign body include unilateral obstructive emphysema,
atelectasis, and consolidation. Unilateral obstructive emphysema is seen
when a foreign body obstructs air flow, mainly on expiration. This generates
a check-valve obstruction that results in hyperinflation of the affected side
and mediastinal shift to the opposite side (Figure 119-6). A foreign body
that obstructs a bronchus may produce focal atelectasis and consolidation
visible on chest films. Inspiratory and expiratory chest radiographs can aid
in the diagnosis by showing hyperinflation (air trapping) on expiratory
films. Bilateral decubitus chest films may also be used to demonstrate air
trapping: on lateral decubitus films, the dependent lung should collapse
normally, but it remains inflated in bronchial obstruction (Figures 119-7
and 119-8). It is important to note again that a clinically suspected foreign
body aspiration should ultimately be ruled out by bronchoscopy.
Treatment Children with complete airway obstruction require immediate
medical attention. They are typically unable to breathe or speak and require
emergency implementation of BLS measures to relieve airway obstruction.
For detailed discussion of management of airway obstruction in children,
see Chapter 14, Resuscitation of Neonates and Chapter 15, Resuscitation of
Children. If BLS maneuvers fail, direct laryngoscopy and foreign body extraction with Magill forceps should be attempted. When the foreign body is
not visible or able to be removed, orotracheal intubation with dislodgment
of the foreign body more distally (often into the right mainstem bronchus)
can relieve the complete obstruction and may be life saving. If the foreign
body cannot be removed and ventilation cannot be provided through an
endotracheal tube, needle cricothyroidotomy or emergency tracheostomy
should be performed (see Chapter 29, Pediatric Airway Management).
Those patients who do not have complete airway obstruction should have
their respiratory status closely monitored while preparations are made for
bronchoscopic removal under general anesthesia.
■ RETROPHARYNGEAL ABSCESS
The retropharyngeal space occupies the space between the posterior
pharyngeal wall and the prevertebral fascia and extends from the base of
the skull to approximately the level of the second thoracic vertebrae. This
space is fused down the midline and contains two chains of lymph nodes
extending down each side. These lymph nodes tend to regress by age 4
years old, thereby explaining the greater frequency of retropharyngeal
abscess in young children. The formation of a retropharyngeal abscess is
believed to be secondary to suppuration of these lymph nodes that have
been seeded from a distant infection. Localized penetrating trauma with
subsequent invasion of this space by bacteria is another cause of retropharyngeal infection. This most commonly occurs in children who fall
with a stick or other similar object in their mouth. Infection can also
FIGURE 119-5. A. Posteroanterior and (B) lateral chest radiographs showing
radiopaque bronchial foreign body. (Courtesy of W. McAlister, MD, Washington
University School of Medicine, St. Louis, MO.)
occur from traumatic esophageal instrumentation or ventral extension
of vertebral osteomyelitis. Retropharyngeal infection typically progresses from an organized phlegmon to a mature abscess.
Clinical Features Most cases of retropharyngeal abscess evolve insidiously over a few days after a relatively minor upper respiratory infection.
Fever is typically present but may be absent in >10% of patients.26–29
Signs and symptoms include neck pain, fever, dysphagia, excessive
drooling, and neck swelling. The child may maintain the neck in an un-
794
SECTION 12: Pediatrics
FIGURE 119-7. Normal decubitus film with left side down.
Treatment Carefully monitor and stabilize the airway. Obtain IV access
to administer fluids, antibiotics, and CT contrast. Retropharyngeal cellulitis and small localized abscesses may be treated successfully with antibiotic therapy alone. All other cases should undergo operative incision and
drainage, usually by an otolaryngologist. Steroids can reduce airway edema, inflammation, and the progression of cellulitis into an abscess. Most
retropharyngeal abscesses are found to contain mixed flora when cultured.30 Common organisms include S. aureus, S. pyogenes, S. viridans,
and β-lactamase producing gram-negative rods. Oral anaerobes are also
FIGURE 119-6. A. Inspiratory and (B) expiratory chest radiographs showing air
trapping on the left with shift of the mediastinum to the right caused by a peanut in
the left mainstem bronchus. (Courtesy of W. McAlister, MD, Washington University
School of Medicine, St. Louis, MO.)
usual position, with stiffness, torticollis, and hyperextension. A unique
finding is bulging of the posterior oropharynx. Abscess progression can
lead to stridor and respiratory distress. Pleuritic chest pain is an ominous
sign, indicating extension of the infection into the mediastinum.
Diagnosis Classically, with suspected retropharyngeal abscess, initial imaging includes a soft tissue lateral neck radiograph. The radiograph should
be taken during inspiration with the neck extended to limit false positive
results. The diagnosis of retropharyngeal abscess/cellulitis is suggested
when the retropharyngeal space at C2 is twice the diameter of the vertebral body or greater than one half the width of the C4 vertebral body
(Figure 119-9). Rarely, gas may be seen within the mass. Definitive diagnosis is based on CT scan with IV contrast, which can differentiate cellulitis from abscess, identify the anatomic spaces involved, and help with
planning a surgical approach to treatment. CT sensitivity for retropharyngeal abscess is thought to be near 100%. A CT scan may demonstrate necrotic nodes, inflammatory phlegmon, or fluid collection within a ringenhancing abscess (Figure 119-10). Unstable patients should be intubated
before going to the radiology suite for CT scan. Patients requiring sedation
to obtain a scan may require presedation intubation if airway obstruction
is present. Patients without airway compromise should be escorted to radiology by a physician accustomed to managing the difficult pediatric airway, and appropriate equipment should accompany the patient.
FIGURE 119-8. Decubitus film, right side down, with foreign-body aspiration on
the right side.
CHAPTER 119: Stridor and Drooling
795
FIGURE 119-10. Contrast-enhanced neck CT showing a retropharyngeal fluid
collection (arrow).
FIGURE 119-9. Lateral soft tissue neck radiograph demonstrating retropharyngeal swelling (arrow).
frequently seen. Single-agent antimicrobial therapy includes ampicillin/
sulbactam or clindamycin. Unusual complications of retropharyngeal abscess include airway obstruction, spontaneous abscess perforation, mediastinitis, sepsis, aspiration, and jugular venous thrombosis.
Treatment In nontoxic-appearing adolescents with good follow-up and
with findings most consistent with peritonsillar cellulitis, a trial of oral
antibiotics may be the best choice for treatment. Most cases of peritonsillar abscess are managed as outpatients with prompt aspiration or incision and drainage using local anesthetics in the ED. Young and
uncooperative children may require procedural sedation to facilitate adequate evaluation and drainage. Complications of needle aspiration and
incision and drainage include hemorrhage, puncture of the carotid artery, and airway aspiration of purulent material. CT with IV contrast is
the imaging modality of choice for assessment of suspected infection in
patients who have failed incision and drainage and whom trismus or lack
of cooperation prevents a thorough intraoral examination.
Most peritonsillar abscesses are polymicrobial infections. Predominant organisms include: anaerobes, group A β-hemolytic streptococci, S.
■ PERITONSILLAR ABSCESS
Peritonsillar abscess is a deep oropharyngeal infection. It can occur in patients of any age, but most commonly occurs in adolescents and young
adults. The disease typically begins as a superficial infection that progresses to an accumulation of pus in a space between the tonsillar capsule and
the superior constrictor muscle. Most are unilateral, and <10% are bilateral at the time of diagnosis.
Clinical Features and Diagnosis Patients with peritonsillar abscess typically present with sore throat, fever, chills, trismus, and voice change (“hot
potato voice”). Patients will often complain of “the worst sore throat” of
their life and may drool due to difficulty swallowing their saliva. Ipsilateral
ear pain and torticollis may be present. Visualization of the oral cavity on
physical examination in patients with peritonsillar abscess may show bulging of the affected tonsil and deviation of the uvula away from the involved
tonsil (Figure 119-11).
Differentiating peritonsillar cellulitis from peritonsillar abscess can be
difficult. If the child is toxic, consider a peritonsillar abscess until proven
otherwise. Imaging with US or CT scan may be required to differentiate
tonsillitis from abscess.
FIGURE 119-11. Peritonsillar abscess. (Reproduced with permission from Knoop
K, Stack L, Storrow A: Atlas of Emergency Medicine, 3rd ed. McGraw-Hill, New York.
Part 1 Regional Anatomy, Chapter 5, Ear, Nose & Throat Conditions, Figure 5-27.)
796
SECTION 12: Pediatrics
aureus, and H. influenzae.30 The fluid obtained from needle aspiration
should be sent for Gram stain and culture. IV antimicrobial therapy may
include ampicillin-sulbactam or clindamycin. Outpatient management
antibiotic choices include clindamycin and amoxicillin/clavulanate. Single high-dose steroid administration may improve symptoms in patients
with peritonsillar abscess.31
■ LUDWIG ANGINA
Ludwig angina is a potentially life-threatening, rapidly expanding infection of the submandibular space. The submandibular space is composed
of two spaces subdivided by the mylohyoid muscle into the sublingual
and submylohyoid space (submaxillary space) and extends from the
floor of the mouth to muscular attachments at the hyoid bone. Infectious
expansion into this space spreads superiorly and posteriorly and often
involves the entire submandibular space (Figure 119-12). Eighty-five
percent of cases arise from an odontogenic source, often from the
spread of periapical abscesses of mandibular molars.
Clinical Features Ludwig angina usually begins with a mild infection
that progresses rapidly to severe mouth pain, drooling, trismus, tongue
protrusion, and brawny neck swelling. The child may lean forward to
maximize airway diameter. Stridor may develop with subsequent progressive airway obstruction. Control the airway early, as intubation can
be extremely difficult late in the clinical course of the disease. One case
series reports that 11 out of 20 patients had an unsuccessful attempt at
intubation resulting in emergent tracheotomy.32 Treatment is antibiotics
and oral surgery to remove the dental abscess that is the source of the infection. IV antibiotics should cover β-lactamase–producing aerobic or
anaerobic gram-positive cocci and gram-negative bacilli. Consideration
must be given to including coverage of community-acquired methicillin-resistant S. aureus as well.
■ OROPHARYNGEAL TRAUMA
Traumatic oropharyngeal injuries in children typically occur during a
fall with an object in the mouth. Such injuries are often referred to as
“pencil injuries” and most commonly occur in patients between 2 and 4
years of age. When evaluating these injuries, ask if a foreign body was removed intact, or if part of the object may have broken off into the soft
tissue. If there is suspicion of retained foreign body, imaging is required.
Clinical Features Children with oropharyngeal trauma may present with
bleeding, drooling, or dysphagia. Most wounds do not require surgical intervention and closure, but large gaping wounds and those with persistent
bleeding may require closure under sedation or anesthesia. Prophylactic
antibiotics play an inconclusive role in the treatment of intraoral wounds.33
There are rare but well-known complications of penetrating pharyngeal
injury. Entrance of free air into the neck or chest can result in stridor and
acute airway obstruction. Subsequent retropharyngeal infection from introduction of bacteria into the penetrating wound can occur. A more severe complication of oropharyngeal trauma is carotid artery injury. The
carotid artery is closely associated with the lateral oropharynx and is at risk
of injury from penetrating and blunt impact forces. Penetrating injury results in massive hemorrhage, whereas blunt injuries can cause compression of the carotid artery between the object and upper cervical vertebrae.
The resultant shearing effect can cause an intimal tear in the vessel with
subsequent thrombosis formation. Symptoms may evolve over hours to
days and can result in significant neurologic sequelae (stroke in the distribution of the common carotid territory).
Diagnosis and Treatment Neither mechanism nor degree of injury is
helpful in determining the possibility of neurovascular compromise. Soft
tissue lateral neck films can assist in the evaluation of air in soft tissues,
radiopaque foreign bodies, and evaluating for abscess. Normal retropharyngeal soft tissue in airway films is no more than one half of the width
of the adjacent vertebral body. An increase in the width and the presence
of air in the retropharyngeal space indicates pharyngeal injury and may
warrant further investigation. CT is superior to plain radiographs for the
detection of free air, inflammation, or abscess. CT angiography is needed
if carotid injury is suspected and should be considered for patients who
are unstable, who cannot be adequately assessed, and for those in whom
lateral pharyngeal trauma raises concern for vascular injury.34 Treatment is specific for the complication and involves consultation with surgery or otolaryngology.
Acknowledgment: The authors gratefully acknowledge the contributions of Randolph Cordle, MD, the author of the related chapter in the
previous edition.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
120
Geniohyoid
muscle
Mylohyoid
muscle
Submandibular
space:
Sublingual
space
Submaxillary
space
Superficial
fascial layer
Wheezing in Infants
and Children
Donald H. Arnold
David M. Spiro
Melissa L. Langhan
WHEEZING
The respiratory system is comprised of lung parenchyma and compliant
airways. Flow limitation and elevated airway resistance induce flutter of
the airway wall that generates the high-pitched sounds known as wheezing. Wheezing implies obstructive airway disease when diffuse, and focal
obstruction when localized. However, severe flow limitation may exist
without wheezing. Because intrathoracic airway lumen size normally increases during inspiration and decreases during expiration, wheezing is
generally more prominent during expiration. Bronchiolitis is the most
frequent cause of wheezing in infants, and asthma is the most frequent
cause in children and adolescents.
■ PATHOPHYSIOLOGY
FIGURE 119-12. Spread of infection within the submandibular space of the neck.
The nasal passages account for 50% of total airway resistance. Nasal resistance may increase substantially in the presence of nasal mucus or
edema, a clinically important event, especially in the infant with bron-
CHAPTER 120: Wheezing in Infants and Children
797
FIGURE 120-1. Normal inspiration and expiration (A,B) and dynamic airway compression (C) with development of auto–positive end-expiratory pressure (Auto-PEEP).
chiolitis. The conducting airways extend from the trachea to the terminal
bronchioles and do not participate in gas exchange. More distally, the
transitional and respiratory zones have increasing numbers of alveoli and
comprise the gas-exchanging units.
Lung tissue has elastic properties. Forces of stretch and recoil are active on each lung as a unit and on the chest wall. The resting state of these
forces is generally considered to be at functional residual capacity, the
lung volume at which the inherent outward elastic force of the chest wall
equals the inherent inward elastic recoil of the stretched lung.2 An important consequence of elastic recoil is the generation of force on the
lung to contract when inflated above functional residual capacity. The
force required to stretch elastic tissue, and the resulting opposite force
generated by the elastic tissue itself, is dependent on how far the tissue is
stretched from this equilibrium state. Normally, at functional residual
capacity, the tissue is relaxed at end expiration, and inspiration begins
with minimal effort at the onset of inspiratory muscle contraction. However, in the presence of hyperinflation above functional residual capacity, this inward elastic force (elastic recoil) must be overcome before a
breath can be initiated, a phenomenon referred to as auto–positive endexpiratory pressure (auto-PEEP). So, in the presence of hyperinflation, a
greater negative inspiratory pressure needs to be generated by the patient
in order to initiate a breath.
Inspiration is an active process aided by the diaphragm and external
intercostal muscles and, during exertion, by the accessory muscles
(scalene and sternocleidomastoid).2 Expiration is normally a passive
process, facilitated by elastic recoil of the stretched lung. Positive pressure generated during relaxed expiration, as a result of elastic recoil,
compresses conducting airways and decreases their lumen size in a homogeneous fashion down to functional residual capacity. In the presence of diffuse (e.g., asthma, bronchiolitis) or focal (e.g., foreign body)
intrathoracic airway obstruction, the normally passive process of expiration becomes active in an attempt to overcome airway resistance. Abdominal and internal intercostal muscles are recruited. Positive
intrapleural pressure is generated, and increasing external pressure is applied to the airways. This leads to progressively increasing airway obstruction as expiration proceeds, a phenomenon referred to as dynamic
airway compression (Figure 120-1).4
Dynamic airway compression results in prolonged expiratory time. The
net result is failure of alveoli and distal airways to empty fully at end expiration, air trapping, increased functional residual capacity, and auto-PEEP.
Auto-PEEP results from the elastic stretch of hyperinflated lung tissue
above functional residual capacity. Before subsequent inspiratory flow can
begin, inspiratory muscles must overcome this load, which substantially increases the work of breathing. Finally, air trapping and subsequent atelectasis result in areas with ventilation–perfusion mismatch and hypoxemia.
Infants have inherently smaller airways, highly compliant bronchial
and bronchiolar cartilage, and more peripheral airway smooth muscle.
These factors result in an even greater tendency toward airway collapse
during expiration, air trapping, and auto-PEEP. Thus, infants not only
are more likely to experience wheezing illnesses but are also more likely
than older children or adults to suffer the physiologic consequences of
airway obstruction.
■ CLINICAL FEATURES
Determine precipitating events (e.g., outside play, tobacco or pet exposure); prior history of asthma or other wheezing illness; prior hospital
admission, particularly to a critical care unit; prior endotracheal intubation; and occurrence of a choking or gagging episode suggesting aspiration of a foreign body or bacteria from the oral cavity. Ask about a family
history of asthma or atropy.
Pertinent physical examination findings include respiratory rate.
Normal respiratory rates for healthy children at rest and at sea level are
provided in Table 120-1.5 Similar data for children <4 years old are not
available. However, age-dependent ranges for respiratory rate based on
the National Asthma Education and Prevention Program (NAEPP)
guidelines may be used as a guide (Table 120-2).
Oxygen saturation should be monitored by pulse oximetry. Most infants
and children with asthma or bronchiolitis have ventilation–perfusion mis-
TABLE 120-1 Age-Related 50th and 97.5th Percentiles
for Respiratory Rate (Breaths/Minute)
Age
50th percentile
97.5th percentile
4
5
6
7
8
9
10
11
12
13
14
15
16
22
21
21
20
19
19
18
17
17
16
15
14
14
26
25
24
24
23
23
22
21
21
20
20
19
18
Reproduced with permission from Wallis LA, Healy M, Undy MB, Maconochie I: Age related
reference ranges for respiration rate and heart rate from 4 to 16 years. Arch Dis Child 90:
1117, 2005.
798
SECTION 12: Pediatrics
TABLE 120-2 Evaluation of Asthma Exacerbation Severity in the ED
Symptoms
Breathlessness
Talks in:
Alertness
Signs
Respiratory rate*
Mild
Moderate
Severe
Imminent Respiratory Arrest
While walking
While at rest (infant—softer,
shorter cry, difficulty feeding)
Prefers sitting
Phrases
Usually agitated
While at rest (infant—stops
feeding)
Sits upright
Words
Usually agitated
Drowsy or confused
Can lie down
Sentences
May be agitated
Increased
Increased
Often >30 breaths/min
Respiratory Rates in Awake Children
Age
Normal Rate
<2 mo
<60 breaths/min
2–12 mo
<50 breaths/min
1–5 y
<40 breaths/min
6–8 y
<30 breaths/min
Commonly
Usually
Use of accessory muscles
Usually not
Wheezing
Moderate, often only end-expi- Loud, throughout exhalation Usually loud, throughout
ratory
inhalation and exhalation
<100 beats/min
100–200 beats/min
>120 beats/min
Normal Heart Rates in Children
2–12 mo
<160 beats/min
1–2 y
<120 beats /min
2–8 y
<110 beats /min
<10 mm Hg
10–25 mm Hg
>25 mm Hg (adult)
20–40 mm Hg (child)
Heart rate
Pulsus paradoxus
Functional Assessment
PEF or forced expiratory volume in
≥70%
1 s (% predicted or % personal best)
Partial pressure of CO2†
<42 mm Hg
Arterial O2 saturation (pulse oximetry) >95%
40%–69% or response lasts
<2 h
<42 mm Hg
90%–95%
Paradoxical thoracoabdominal
movement
Absent
Bradycardia
May be absent due to respiratory muscle fatigue
<40%
<25% (PEF testing may not be
needed in severe attack)
≥42 mm Hg (possible respiratory failure)
<90%
Abbreviation: PEF = peak expiratory flow.
Notes: The presence of several specified features, but not necessarily all, indicates the general classification of an exacerbation.
Many of these parameters have not been systematically studied, especially as they correlate with each other. Thus, they serve only as general guides.
The emotional impact of asthma symptoms on the patient and family is variable but must be recognized and addressed and can affect approaches to treatment and follow-up.
*Respiratory rates for ages 4–16 y are also provided in Table 120-1.
†End-tidal
CO2 levels are generally 3–5 mm Hg lower than partial pressure of CO2 levels.
match leading to mild hypoxemia (>92%) that readily corrects with minimal
oxygen supplementation. More severe hypoxemia suggests alveolar disease (pneumonia), pneumothorax, or true pulmonary shunt. Diaphoresis,
confusion, or drowsiness are ominous signs indicating imminent respiratory
failure. The clinician should check for audibility and symmetry of breath
sounds to assess adequacy of ventilation. The use of accessory muscles indicates an increased work of breathing. Inspiratory to expiratory ratios of less
than the normal 2:1 (such as 1:2 or 1:3) reflect the prolonged expiratory
times seen with obstructive airway disease. Perhaps more important than
the initial physical examination findings are the changes in these findings
in response to bronchodilator administration and other treatments.
■ DIAGNOSIS
The differential diagnosis of wheezing in infants and children is extensive and
is best approached by consideration of presenting signs and symptoms, overall clinical course, and results of ancillary testing, if indicated (Table 120-3).
Although asthma and bronchiolitis account for most wheezing episodes in children, a broader differential diagnosis should always be
kept in mind. This is particularly important when the patient’s history
or physical findings are not entirely consistent with a diagnosis of as-
thma or bronchiolitis, or when the illness does not respond to interventions appropriate to these disease processes. For example, persistent
wheezing that has been present from the newborn period and associated
with failure to thrive may suggest cystic fibrosis (Table 120-3).
Stridor is usually not associated with lower airway obstruction. The presence of stridor should suggest croup, laryngotracheomalacia, foreign
body, airway polyp or hemangioma, or extrinsic tracheal compression
from vascular malformation or soft tissue mass (see Chapter 119, Stridor and Drooling). The association of wheezing with feedings suggests
gastroesophageal reflux or tracheoesophageal fistula with aspiration. Expiratory grunting is a physiologic response to air space (alveolar) disease and
an attempt to maintain alveolar inflation by generating PEEP. The presence
of grunting and fever suggests pneumonia, loss of surfactant, or other alveolar disease. Grunting may also result from extra-pulmonary disease including GI emergencies. Inspiratory crackles (fine rales) may result from
atelectasis associated with asthma.6 If rales persist after bronchodilator
treatments, pneumonia should be considered. Specific diagnostic studies
may assist in elucidating these alternate diagnoses. Foreign-body aspiration and congestive heart failure present with wheezing, and the correct
diagnosis must be quickly established. For further discussion, see Chapter
CHAPTER 120: Wheezing in Infants and Children
799
TABLE 120-3 Differential Diagnosis of Wheezing According to Presenting Signs and Symptoms
Signs, Symptoms, and Context
Possible Diagnoses
Ancillary Testing
Feeding-related cough, gagging, or emesis; present from
birth
Gastroesophageal reflux
Tracheoesophageal fistula
Cystic fibrosis
Ciliary dyskinesia
Immunodeficiency
Congenital heart disease with left-to-right shunt and
congestive heart failure
Myocarditis
Vascular ring or other great vessel malformation; airway
polyp or hemangioma
Epiglottitis
Foreign-body aspiration
Vocal cord dysfunction (paradoxical vocal cord motion)
Esophageal pH probe
Barium swallow
Sweat chloride concentration
Ciliary biopsy
Immunoglobulin assays
CXR; ECG; echocardiography
Multiple lower respiratory tract illnesses or infections; failure to thrive; present from birth
Diffuse rales; tachycardia; hepatomegaly; cardiac murmur
Associated stridor; positional changes with neck flexion,
extension, or rotation
Stridor with high fever; ill appearance; drooling
Abrupt-onset stridor and/or wheezing; history of choking
episode
Stridor and/or wheezing that changes with position, or is
exacerbated during feeding or upper respiratory illness
Tachypnea; fever; rales; grunting
URI symptoms; seasonal outbreaks; nasal flaring
Episodic exacerbations with wheezing and/or cough; seasonal or after exposure to allergens; responds to bronchodilators
Tracheomalacia
Laryngomalacia
Pneumonia
Bronchiolitis
Asthma
CXR; CT angiography; barium swallow; bronchoscopy
Soft tissue radiographs of neck*
Right and left lateral decubitus CXRs*
Bronchoscopy
Flexible fiberoptic laryngoscopy
Observation
Bronchoscopy
CXR
Viral antigen testing
Pulmonary function testing; trial of albuterol;
allergy testing
Abbreviations: CHF = congestive heart failure; CXR = chest x-ray; URI = upper respiratory infection.
*Caution must be exercised in transporting the patient with possible epiglottitis or foreign-body aspiration outside of the ED, because complete airway obstruction can be sudden.
Reproduced with permission from Weiss LN: The diagnosis of wheezing in children. Am Fam Physician 77: 1109, 2008.
122A, Pediatric Heart Disease: Congenital Heart Defects, and Chapter
122B, Pediatric Heart Disease: Acquired Heart Disease.
BRONCHIOLITIS
Bronchiolitis is the most common lower respiratory tract infection in infants and children ≤2 years of age. It remains the leading cause for hospitalization in children <1 year of age at an annual cost of over $500 million.7
Bronchiolitis is most commonly caused by respiratory syncytial virus
(RSV), but may be caused by other viral agents, including human metapneumovirus, adenovirus, influenza, rhinovirus, and parainfluenza viruses.
Within the first 2 years of life, up to 90% of children will have been infected
by RSV.8 Of those, 40% manifest with a lower respiratory tract infection.9
■ PATHOPHYSIOLOGY
Bronchiolitis is inflammation of the lower respiratory tract, with edema,
epithelial cell necrosis, bronchospasm, and increased mucus production
within the bronchioles.10 These features result in variable degrees of
atelectasis or hyperinflation of the lower airways. The increase in airway
resistance and development of lower airway obstruction results in increased work of breathing. Because the nasal passages account for 50% of
total airway resistance, increased mucus production may cause upper airway obstruction due to the small nasal passages of infants. This in itself can
cause modest respiratory distress, particularly in young infants, who are
obligate nasal breathers.
RSV is transmitted by direct contact with contaminated secretions, including large droplets into the mucosa of the eyes and nose. Infected secretions found on fomites remain contagious for several hours. Because RSV
is highly infectious, self-contamination and nosocomial spread are common. Hand washing and contact precautions are important to limit the
spread of disease. The incubation period for RSV ranges from 2 to 8 days.
■ CLINICAL FEATURES
Although bronchiolitis can be seen throughout the year, its peak occurrence in North America is from November to March, coinciding with
the high incidence of RSV infection during this time. Typically, rhinorrhea, tachypnea, wheezing, and coughing are present. Use of accessory
muscles, nasal flaring, and fever may also occur. These symptoms last
on average 7 to 14 days and are often the worst in the initial 3 to 5 days
of the illness. Associated symptoms include irritability, cyanosis, and
poor feeding.
A subset of infants with bronchiolitis develop severe disease and apnea. Apnea episodes may be brief and self-limited or progress to more
frequent and prolonged episodes that lead to hypoxia and the need for
endotracheal intubation. Several factors associated with a greater risk of
severe disease and apnea are listed in Table 120-4.10,11 Infants with bronchiolitis and these risk factors may have prolonged hospital stays, greater
need for mechanical ventilation, and higher mortality rates. Apnea may
develop in infants with mild signs and symptoms of disease and bronchiolitis should be considered in the patient presenting to the ED with apparent life-threatening event (see Chapter 112, Sudden Infant Death
Syndrome and Apparent Life-Threatening Event).
On chest examination, wheezing and crackles are heard diffusely
throughout both lung fields. Respiratory rates may vary from within normal ranges to tachypnea, which can be profound. Although there are no
normative data for respiratory rates in this age group, normal ranges
TABLE 120-4 Risk Factors for Severe Disease and
Apnea in Infants with Bronchiolitis
Prematurity (<37 wk gestational age)
Age <12 wk
Postconception age <48 wk
Witnessed episode of apnea
Underlying cardiopulmonary disease
Immunodeficiency
Chronic lung disease
800
SECTION 12: Pediatrics
are a mean of 50 breaths/min in term newborns, 40 breaths/min at 6
months, and 30 breaths/min at 1 year in otherwise healthy children.10
The respiratory rate should be measured over 1 full minute in these age
groups. Accessory muscle use and intercostal or subcostal retractions develop as respiratory distress worsens. The patient should be assessed for
signs of dehydration such as dry mucous membranes, inadequate urine
output, and a sunken fontanelle, because the increase in respiratory effort and/or upper airway obstruction may inhibit feeding, especially in
younger infants. Increased work of breathing may also result in increased insensible losses and a higher metabolic rate, which also contribute to dehydration with bronchiolitis.
■ DIAGNOSIS
Bronchiolitis is diagnosed based on findings of the history and physical examination, which include typical symptoms of rhinorrhea,
tachypnea, and wheezing in a child <2 years of age. There are several
published clinical scoring systems for assessing the severity of illness and
change over time, but none has been validated. The Respiratory Distress
Assessment Instrument, which is composed of measurements of wheezing, retractions, and respiratory rate, is the most widely used scoring system (Table 120-5).12 There is good interrater reliability, but scores are
not predictive of outcome.
Pulse oximetry readings should be monitored to detect hypoxemia
that may not be readily suspected on physical examination. Serial examinations and continuous pulse oximetry provide a better sense of the severity of illness than does a single assessment.
Rapid viral antigen detection tests may be useful. Sensitivity of the rapid
tests generally ranges from 80% to 90% and specificity from 90% to 99%.
Test results remain positive as long as the virus is being shed. For RSV, this
may be up to 2 weeks after the onset of symptoms. The use of reverse-transcriptase polymerase chain reaction testing to detect nucleic acid offers
greater sensitivity. Results of viral culture are not available for several days
and are not useful for guiding ED treatment.
Ancillary tests, such as blood work and radiographs, are not routinely
needed unless other diagnoses need to be excluded.10 The incidence of serious bacterial infections in infants <28 days of age with bronchiolitis is 3%
to 10%, similar to that in other neonates with fever, so the standard testing
of blood, urine, and cerebrospinal fluid is indicated. In infants >60 days of
age, the incidence of serious bacterial infection in association with bronchiolitis remains 3% to 5%, with the most common infection being a urinary
tract infection.13 Chest radiographs are not routinely indicated, but may be
considered when the illness is severe or the course is atypical to ensure that
pneumonia is not present. Although the chest radiograph in bronchiolitis
may demonstrate atelectasis, bacterial pneumonia is unusual.
TABLE 120-5 Respiratory Distress Assessment Instrument
Points
0
1
2
3
4
Wheezing
Expiration
None
End
One half
Three
fourths
All
Inspiration
Location
None
None
Part
All
Segmental: ≤2 Diffuse: ≥3 of
of 4 lung fields 4 lung fields
Retractions
Supraclavicular
Intercostal
Subcostal
Respiratory rate
None Mild
Moderate
None Mild
Moderate
None Mild
Moderate
Initial respiratory rate = 0
Decrease in respiratory rate of 10% = +1
Increase in respiratory rate of 10% = –1
Marked
Marked
Marked
Reproduced with permission from Lowell DI, Lister G, Von KH, McCarthy P: Wheezing in
infants: the response to epinephrine. Pediatrics 79: 939, 1987.
TABLE 120-6 ED Management of Bronchiolitis
No Respiratory Distress
[PO2 ≥95, no risk factors for
admission (Table 120-4)]
Ill-Appearing, Dehydrated, Apneic
[PO2 <95 or other risk factors for
severe illness (Table 120-4)]
Continuous pulse oximetry
Observation during feeding
if concern about more severe
illness
Nasal saline drops and nasal
suctioning
Antibiotics if evidence of
urinary tract infection or otitis
media
Continuous pulse oximetry
IV hydration if dehydrated
Racemic epinephrine, 0.1% solution (0.5 mL in
3.5 mL NaCl) given as needed. Most infants
who require racemic epinephrine need admission. Otherwise, observation for 4 h before
discharge.
Supplemental oxygen to keep oxygen saturation >93%
Ventilatory support for apnea
■ TREATMENT
Table 120-6 outlines ED management of bronchiolitis.
Infants with bronchiolitis who do not meet criteria for admission can
be managed as outpatients, and few need hospital admission. Treatment
is frequent use of nasal saline drops and nasal suctioning. Caretakers
should employ frequent hand washing to minimize contagion.
The American Academy of Pediatrics recommends maintaining an
oxygen saturation of >90%.10 Although there is concern that intermittent hypoxia (oxygen saturation of <95%) may affect later cognition,14,15
as of this writing, there have been no direct studies that evaluate the effects of mild hypoxia (90% to 94%) on cognition.
There is no consistent evidence that either α- or β-adrenergic bronchodilators are of benefit for the standard treatment of bronchiolitis.
Although some studies have demonstrated a transient improvement in
clinical scores and pulse oximetry after administration of racemic epinephrine, there is no long-term impact on the course of illness.16,17 Administration of racemic epinephrine should be considered in infants with
respiratory distress or hypoxia, although only a small percentage of children will demonstrate a clinical response. Racemic epinephrine is somewhat superior to albuterol,18 probably because the α agonist effects of
epinephrine may decrease mucosal edema. Scores measured using an objective method of evaluation such as the Respiratory Distress Assessment
Instrument (Table 120-5) can be recorded before and after racemic epinephrine administration to document clinical change, and if there is no
improvement with the first dose, the drug should not be repeated. Although racemic epinephrine has a clinical advantage over albuterol, it is
not prescribed for home use. On the other hand, for infants who improve
after receiving albuterol, albuterol can be prescribed at discharge, either as
a metered dose inhaler with a spacer device or as a nebulized solution.
Heliox Heliox administration can be a temporizing measure for moderate to severe bronchiolitis,19–21 and use is the same as for asthma.
Ventilatory Support Ventilatory support must be provided if supplemental oxygen does not correct hypoxia, or if respiratory distress worsens. The use of bi-level positive airway pressure (BiPAP) or continuous
positive airway pressure often allows intubation to be avoided. However,
intubation with assisted ventilation is sometimes necessary and is further detailed in the section Endotracheal Intubation and Assisted Ventilation in the management of ventilatory and respiratory failure.
Corticosteroids The role of corticosteroids in treatment of bronchiolitis
is controversial. No difference was found in hospitalization rates or respiratory status between those infants who received a 1-milligram/kg
dose of dexamethasone and those who received a placebo.22 A number
of meta-analyses and the American Academy of Pediatrics guidelines
have concluded that corticosteroid administration is not indicated in infants with bronchiolitis.10,23 A large Canadian study published in the
New England Journal of Medicine in 2009, however, has reopened debate
on the topic. In this randomized, controlled trial, the authors found an
unexpected synergy when steroids were given together with racemic epi-
CHAPTER 120: Wheezing in Infants and Children
nephrine. Although infants assigned to the steroid-only group did not
show clinical benefit, those who received racemic epinephrine and dexamethasone (1 milligram/kg in the ED followed by 0.6 milligram/kg/d for
an additional 5 days) had lower rates of hospitalization.24 This finding
was unexpected, and the study underpowered, but the finding once
again raises debate about the potential benefit of steroids and α agonists
as a treatment option in children with bronchiolitis.
Nebulized Hypertonic Saline Nebulized 3% hypertonic saline solution is
an alternative treatment for bronchiolitis that has produced improvement
in clinical scores in several recent studies.25–27 Nebulized hypertonic saline
is thought to decrease mucus production and airway inflammation, and its
use has been studied in children with cystic fibrosis. In hospitalized children, the use of 4 mL of 3% hypertonic saline solution both with and without epinephrine led to decreases in length of stay as well as clinical
improvements in infants with bronchiolitis.26
■ DISPOSITION AND FOLLOW-UP
Admission and Discharge Criteria The majority of children with bronchiolitis can be discharged from the ED. Assurance of an adequate home
environment and follow-up care is essential for discharge. Factors for
predicting safe discharge from the ED are listed in Table 120-7. Factors
such as sex, race, duration of symptoms, parental history of asthma, and
prior ED visits were not found to be related to safety for discharge.28
Oxygen saturation of <95% or the inability to adequately feed and maintain hydration are the most common reasons for admission for bronchiolitis.
Infants with witnessed episodes of apnea require admission. Admission is
recommended for those with risk factors for apnea even when they are
clinically well appearing. Most experts recommend admission of all infants
<1 month of age who test positive for RSV, regardless of severity of symptoms, as apnea can develop without respiratory distress in these patients.
Follow-Up Those children with mild bronchiolitis who demonstrate no
significant increase in respiratory effort and are able to maintain adequate
oral intake should follow up with their primary care provider within 24
hours. Caregivers should be educated regarding the signs and symptoms
of increasing respiratory distress, including an increase in respiratory rate,
presence of retractions, and inability to feed. They should be advised to
bring the child for immediate reevaluation if any of these develop. Parents
should be counseled that symptoms may persist for 1 to 2 weeks to help
avoid unnecessary ED returns for persistent mild symptoms.
■ PRACTICE GUIDELINES
More detailed information is provided in a practice guideline issued in
2006 by the American Academy of Pediatrics Subcommittee on Diagnosis and Management of Bronchiolitis that has been endorsed by the
American Academy of Family Physicians, the American College of
Chest Physicians, and the American Thoracic Society entitled “Diagnosis and Management of Bronchiolitis.” It is available at: http://aappolicy.
aappublications.org/cgi/content/full/pediatrics;118/4/1774.
TABLE 120-7 Predictors of Safe Discharge from the
ED for Infants with Bronchiolitis
Age >2 mo
Respiratory rate less than normal for age
<45 breaths/min for 0–2 mo
<43 breaths/min for 2–6 mo
<40 breaths/min for 6–24 mo
No or mild retractions
Adequate oral intake
Initial oxygen saturation ≥94% on room air
No history of intubation or apnea
History of eczema
Reproduced with permission from Mansbach JM, Clark S, Christopher NC, et al: Prospective
multicenter study of bronchiolitis: predicting safe discharges from the emergency department. Pediatrics 121: 680, 2008.
801
ASTHMA
■ DEFINITION
The working definition of asthma provided by the NAEPP is lengthy
but is important in identifying the key features of clinical asthma: “Asthma is a chronic inflammatory disorder of the airways in which many
cells and cellular elements play a role: in particular, mast cells, eosinophils,
T lymphocytes, macrophages, neutrophils, and epithelial cells. In susceptible individuals, this inflammation causes recurrent episodes of wheezing,
breathlessness, chest tightness, and coughing, particularly at night or in
the early morning. These episodes are usually associated with widespread
but variable airflow obstruction that is often reversible either spontaneously or with treatment. The inflammation also causes an associated increase in the existing bronchial hyperresponsiveness to a variety of
stimuli. Reversibility of airflow limitation may be incomplete in some patients with asthma.”29
This section provides guidelines for assessment of asthma severity and
management of acute asthma exacerbations in the ED.
An acute asthma exacerbation consists of progressively worsening
wheezing, cough, shortness of breath, and/or chest tightness that is associated with decreased expiratory airflow. Acute severe asthma is an episode associated with dyspnea at rest that interferes with conversation
and that is associated with peak expiratory flow (PEF) of <40% of predicted value.29 Limitations of assessment in children based upon PEF are
discussed below in Clinical Assessment. Acute severe asthma may
progress to unresponsive airway obstruction (status asthmaticus), respiratory failure, and fatal asthma.30,31 The emergency medicine physician
must be prepared to identify the patient experiencing an acute severe attack and to escalate therapy to prevent respiratory failure.
■ PATHOPHYSIOLOGY
The three pathophysiologic processes of asthma that require treatment
are inflammation, bronchospasm, and airway obstruction. The inflammatory cascade either directly leads to or contributes to the severity of
bronchospasm and airway obstruction (Figure 120-2).
Multiple inflammatory pathways are activated in asthma and involve
a complex interplay of cytokines, chemokines, immunoglobulin E (IgE),
lymphocytes, mast cells, and eosinophils.
Bronchospasm is the most clinically apparent event leading to decreased
airflow and symptoms during exacerbations. Bronchospasm may be precipitated by either irritants or allergens. Irritant-induced bronchospasm
may involve non-IgE–dependent pathways (aspirin, NSAIDs), but may
also involve as yet undefined mechanisms (e.g., exercise, emotional stress).
Allergens precipitate bronchospasm as a result of IgE-dependent release of
histamine, leukotrienes, and other mediators from mast cells.
The third primary pathophysiologic process in acute asthma is airway
obstruction. Inflammation causes airway mucosal edema, but also contributes to airway obstruction indirectly as a result of mucus hypersecretion, formation of mucus plugs, and structural airway changes. These
changes include hyperplasia and hypertrophy of airway smooth muscle,
subepithelial fibrosis, and thickening of the sub-basement membrane, a
Inflammation
Airway
obstruction
Airway
hyperresponsiveness
Clinical
symptoms
FIGURE 120-2. Interplay and interaction between airway inflammation and the
clinical symptoms and pathophysiology of asthma.
802
SECTION 12: Pediatrics
TABLE 120-8 Asthma Triggers
Allergens (e.g., animal dander, dust, mold, pollen)
Air pollution (e.g., ozone, particulate matter)
Cold air or weather change
Emotional stress
Exercise
Gastroesophageal reflux
Infections (e.g., viral upper respiratory infection, pneumonia)
Tobacco smoke
Reproduced with permission from Guill MF: Asthma update: epidemiology and pathophysiology. Pediatr Rev 25: 299, 2004.
process termed airway remodeling. As a result of airway remodeling,
some asthmatic patients may experience progressive loss of lung function that may not be reversible.29
■ CLINICAL FEATURES
More than 95% of children aged 2 to 18 years with asthma present to the ED
with wheezing, shortness of breath, fever, cough, and/or dyspnea.48 Fever is
usually due to a viral illness that precipitates the asthma exacerbation. Not
only presenting signs and symptoms but also triggers of an acute exacerbation should be noted (Table 120-8). A history of past ED visits and hospitalizations, prior admissions to intensive care, and home medication use should
be elicited. A family history of asthma or eczema should also be obtained.
The child’s airway and breathing should be assessed immediately. The
degree of respiratory distress and impaired ventilation dictate the tempo
of the evaluation and interventions. Serial assessments are key to ED management, because changes in clinical status and response to treatment are
usually more relevant to outcome and need for admission than the level of
severity at presentation.
Physical signs are useful in determining the severity of airway obstruction.
Mental status changes such as agitation may indicate hypoxemia, whereas
somnolence may indicate hypercarbia. Respiratory rate (Tables 120-1 and
120-2) and air entry indicate the adequacy of gas exchange and ventilation.
Clinical Assessment The patient should be monitored continuously with
pulse oximetry. Hypoxemia is often present due to ventilation–perfusion
mismatch. Mild hypoxemia (>92% at sea level) does not directly provide
information regarding ventilation and airflow obstruction.39,49,50 Oxygen
and β2 agonists are pulmonary vasodilators and may augment perfusion
of underventilated lung units, contributing to ventilation–perfusion mismatch. This causes hypoxemia that generally resolves over the course of 1
to 2 hours as pulmonary autoregulation corrects mismatch. If oxygen saturation is <92%, worsening of the asthmatic episode, pneumothorax, or
pneumonia should be considered.
End-tidal carbon dioxide (ETCO2) monitoring is an important measure of ventilation and tissue perfusion and is easy to perform in children.
The normal level of PaCO2 in patients aged 7 to 19 years is 37 mm Hg,
and ETCO2 should be lower than normal in children with an asthma
exacerbation.51,52 Levels of ETCO2 are normally 3 to 5 mm Hg lower
than capillary or arterial PaCO2.53,54 ETCO2 monitoring should be employed in children with a severe asthma exacerbation.
Early in the course of status asthmaticus, mild hypoxemia and hypocapnia
with respiratory alkalosis are observed.39,55,56 With decreasing alveolar ventilation, increased work of breathing, and tissue hypoxia, PaCO2 increases and
metabolic acidosis begins to predominate over respiratory alkalosis. This results in a rise in PaCO2 and a decrease in serum pH. In both children and
adults, a PaCO2 of 40 to 42 mm Hg may be a sign of impending ventilatory
failure.57,58 Persons recovering from asthma exacerbations do not compensate for the initial respiratory alkalosis by hypoventilation with a rise in PaCO2
above normal. Rather, hypercarbia is the hallmark of ventilatory failure. Although ETCO2 levels above 37 to 39 mm Hg warrant concern for ventilatory failure, ETCO2 monitoring may not be sufficiently sensitive, because
inadequate alveolar emptying due to airway obstruction with gas trapping
may result in normal levels of ETCO2 in the face of rising PaCO2.
Pulsus paradoxus is an exaggeration of the difference between the decrease in left ventricular stroke volume occurring during inspiration and
the increase during expiration.59 This difference is normally <10 mm
Hg. Assessment for pulsus paradoxus in an acute asthma exacerbation is
currently recommended by NAEPP and Global Initiative for Asthma
guidelines.29,60 However, the only bedside method for pulsus paradoxus
measurement is a blood pressure cuff, and the inaccuracy and difficulty
in measurement by this method makes it impractical.61
Accessory muscle use reflects work of breathing necessary to overcome
auto-PEEP and airway resistance. In children and adults, heart rate, respiratory rate, and accessory muscle use are statistically significantly associated with diminished percentage of predicted forced expiratory volume in 1
second (% FEV1).62,63 Accessory muscle recruitment progresses in a caudal
to cephalad direction: subcostal and intercostal muscle use occurs with
mild to moderate obstruction while neck muscle use suggests severe obstruction. Wheezing is pathognomonic for airway obstruction and typically occurs more during expiration than during inspiration. However, the
quiet chest is an ominous sign of severely compromised ventilation and
indicates airflow insufficient to generate wheezing.
Measurement of PEF during asthma exacerbations is also advocated by
National Heart, Lung, and Blood Institute and Global Initiative for Asthma
guidelines.29,60 As with spirometry, PEF measurement requires patient cooperation and coordination. These studies are unable to be performed in
many children with asthma exacerbations.64 Moreover, a normal PEF can
be generated even with moderate to severe airway obstruction, and measurements are difficult to perform in children <6 years old, so this test is
generally not useful in an acute asthma exacerbation.65 However, a measurement can be useful if the child routinely measures PEF at home and can
perform the test correctly.
The severity of an acute asthma exacerbation may be assessed based
on signs and symptoms, without assigning a numeric score, and classified as “mild,” “moderate,” “severe,” or “imminent respiratory arrest”
(Table 120-2).
Use of acute asthma scoring systems is advocated as a means to assess
severity and response to treatment, and particularly to communicate
among providers. The Pediatric Respiratory Assessment Measure (Table
120-9) has been validated using an objective criterion standard for airway
obstruction (airway resistance).66–68 The performance characteristics of
TABLE 120-9 The 12-Point Pediatric Respiratory Assessment Measure
Points
Sign
0
Suprasternal
Absent
retractions
Scalene muscle Absent
contractions
Air entry*
Normal
Wheezing*
Absent
O2 saturation
≥95%
1
2
3
Present
Present
Decreased
at bases
Expiratory
only
92%–94%
Widespread
decrease
Inspiratory
and
expiratory
<92%
Absent or minimal
Audible without stethoscope or silent chest
with minimal air entry
Severity based on total score:
Mild: <5 points
Moderate: 5–9 points
Severe: ≥10 points
Clinically meaningful improvement: ≥3-point change.
*If asymmetric findings of air entry or wheezing are noted, more severe side is rated.
Reproduced with permission from Chalut DS, Ducharme FM, Davis GM: The Preschool Respiratory Assessment Measure (PRAM): a responsive index of acute asthma severity. J Pediatr
137: 762, 2000; and Ducharme FM, Chalut D, Plotnick L, et al: The Pediatric Respiratory
Assessment Measure: a valid clinical score for assessing acute asthma severity from toddlers
to teenagers. J Pediatr 152: 476, 2008.
CHAPTER 120: Wheezing in Infants and Children
803
this scoring system have been assessed in patients aged 2 to 17 years with
asthma, and it is responsive to changes in a patient’s respiratory status.
TABLE 120-11 Key Points for Managing Acute Asthma
Exacerbations in the ED
■ DIAGNOSIS
Treatment
Comments
Oxygen
Short-acting nebulized
β2 agonists
Oral or parenteral
steroids
IV magnesium sulfate
or heliox
Monitoring of response
to therapy
Prevention or minimization of relapse or
recurrence
Treats hypoxemia.
Add ipratropium bromide in moderate and severe
attacks.
Administer early in ED course to all patients requiring
>1 treatment with β agonists.
For severe exacerbations not responsive to above.
The essential diagnostic questions for the child with signs and symptoms
suggestive of acute asthma are threefold: (1) Does this patient have asthma? (2) What is the severity of airway obstruction? (3) Is there a treatable condition that precipitated this exacerbation?
For children >3 years of age who do not have a history of health professional–diagnosed asthma, a provisional diagnosis of asthma is made
when there are signs and symptoms of wheezing, shortness of breath,
cough, or dyspnea; diminished air entry or retractions; and demonstration of reversibility after treatment with albuterol. Because most children
with wheezing illnesses prior to 3 years of age do not have asthma, the diagnosis of asthma should generally not be given to children <3 years old.
It is best to demonstrate severity and reversibility of bronchospasm
with spirometry. Spirometry is the criterion method for determining the
presence of airway obstruction, indicated by the ratio of FEV1 to forced
vital capacity, and the severity of airway obstruction (% FEV1) in asthma.69 However, spirometry is often not possible in children <6 years of
age or in the presence of respiratory distress. Alternate metrics available
at the bedside include physical examination findings, (e.g., respiratory
rate, accessory muscle use, air entry, inspiratory to expiratory ratio) as
well as the NAEPP severity assessment or Pediatric Respiratory Assessment Measure score discussed earlier in Clinical Assessment.
Precipitating conditions should also be considered. Identification of
exposure to tobacco smoke, allergens, and certain other precipitants aids
in educating patients and parents to avoid unnecessary future exposures
or to identify exposures that warrant preventive measures.
Atelectasis is not uncommon in children with acute asthma exacerbations, but bacterial pneumonia is much less common. A chest radiograph for a child with acute asthma and fever is likely to demonstrate
atelectasis, and the question arises whether this finding represents pneumonia. A more reasoned approach is to obtain a chest radiograph only
for those patients who manifest localized rales or dullness that does
not resolve after bronchodilator treatment (in which case the findings
might not represent atelectasis) or who have a temperature of >39°C
(102.2°F) or respiratory distress out of proportion to the degree of airflow limitation on physical examination.70 This approach may avoid
unnecessary use of antibiotics. A chest radiograph should also be considered if fever cannot be reasonably attributed to a viral illness, if there is
significant chest pain (possible pneumothorax), or if the condition fails
to respond to bronchodilator treatment (possible foreign body or congestive heart failure) (Table 120-10).71
■ TREATMENT OF ACUTE ASTHMA EXACERBATIONS
NAEPP guidelines identify key points in management of the acute asthma exacerbation29 (Table 120-11).
There must be a reasoned, structured approach to treatment based
upon severity of illness (e.g., work of breathing, patient fatigue, oxygenation, ventilation) and upon frequent evaluations of response to treatment. The NAEPP guidelines favor assessment and treatment based upon
objective measurement of airflow using spirometrically determined FEV1
or PEF. Although there is a need for such objective measures of severity,
obtaining these measures in the ED is a great challenge in terms of feasibility and accuracy. Both spirometry and PEF determination are highly effort
dependent and require patient cooperation. With this consideration in
TABLE 120-10 Indications for Chest Radiography
in the Child with Acute Asthma
Fever not explained by apparent viral illness
Chest pain, cardiovascular instability, or absent breath sounds (rule out pneumothorax)
Poor response to treatment (consider congestive heart failure or foreign-body
aspiration)
Measure forced expiratory volume in 1 s and peak
expiratory flow.
Recommend follow-up care in 1–4 wk.
Provide ED asthma discharge plan for prescribed
medications and for symptom worsening.
Arrange for practice and review of inhaler techniques.
Consider prescribing inhaled corticosteroid.
mind, the treatment algorithm in Figure 120-3 incorporates both the
NAEPP guidelines based upon FEV1 or PEF determinations and/or Pediatric Respiratory Assessment Measure scoring. Finally, ED physicians
should partner with primary care physicians to institute anti-inflammatory therapy and implement other elements of the NAEPP guidelines.72
With these considerations in mind, severity and response to treatment
should be evaluated using the Pediatric Respiratory Assessment Measure
(Table 120-9) or other asthma score, spirometry or PEF measurement, or
the NAEPP symptom severity assessment (Table 120-2). Treatment should
be instituted and escalated in a stepped-care manner according to severity
and response to initial measures. For example, the patient presenting with a
severe exacerbation with impaired ventilation warrants rapid intensification
of treatment that may include nebulized short-acting β2 agonists, systemic
corticosteroids, and IV magnesium. Further treatments may escalate or deescalate as appropriate based upon response. Figure 120-4 presents one suggested stepped-care paradigm. We recommend a stepped-care treatment algorithm for the ED, appropriate to local resources, keeping in mind that
oxygen, short-acting β2 agonists, ipratropium, and systemic corticosteroids
are indicated for all but the mildest exacerbations. As the patient improves,
medications and other interventions should be withdrawn in a steppeddown fashion.
Medications are broadly classified as those providing long-term control (inhaled corticosteroids) and quick-relief medications (short-acting
β2 agonists). National and international guidelines recommend treatment with long-term controller medications in all children with persistent asthma.29 However, primary care physician adherence to these
guidelines has been suboptimal. Fewer than 50% of children with persistent asthma treated by pediatric emergency physicians receive prescriptions for inhaled corticosteroids at discharge, and ED physicians should
prescribe these medications in accordance with these guidelines (see discussion of steroids in the section Corticosteroids below).73 Dosages of
drugs for acute exacerbations are listed in Table 120-12.
Treatment Agents for Acute Asthma Exacerbations • Oxygen Hypoxemia
during acute asthma exacerbations is largely due to ventilation–perfusion mismatch and usually corrects with minimal supplemental oxygen
administration. The β2 agonists are pulmonary vasodilators and result in
increased perfusion of poorly ventilated alveoli, resulting in mild hypoxemia (>92%). This generally corrects within the hour after β2 agonist
treatment. The administration of 100% oxygen results in diminished
ventilation compared with administration of 28% oxygen, and it is reasonable to titrate supplemental oxygen to achieve target pulse oximetry–
measured oxygen saturation levels of ≥92%.74
Short-Acting β2-Receptor Agonists The β2 agonists have predominant specificity for the β2-receptor and are the most effective agents for relieving
acute bronchospasm. They are more effective and have fewer adverse ef-
804
SECTION 12: Pediatrics
History, physical examination (auscultation, use of accessory muscles, HR, RR), PEF or FEV1, O2 saturation & other tests
Calculate PAS & assign pathway below for clinical management
PAS
5-7
Mild Exacerbation:
FEV1 or PEF >70%
Moderate Exacerbation:
FEV1 or PEF 40-69%
PAS
8-11
Oxygen to achieve SpO2 >94%
Inhaled SABA by Neb one time or
up to 3 doses in first hour
Oral SCS
q 1-2 Repeat Assessment
Severe Exacerbation:
FEV1 or PEF <40%
PAS
12-15
Oxygen to achieve SpO2 >94%
Inhaled SABA & anticholinergic by
Neb continuously until PAS <8
Oral or IV SCS if no immediate
response or if recent SCS
IV fluids, Magnesium
Continue treatment 1-3 h, if
improvement; disposition in <4 h
Impending
Arrest
Oxygen to achieve SpO2 >94%
Inhaled SABA & anticholinergic by Neb
continuously until PAS <8
Magnesium
IV fluids, bolus 20 mL/kg
Consider NPPV
Systemic SABA
IV SCS, q6h
Consider adjunct therapies (see box)
Time
1st assessment:
2nd assessment:
Disposition Decision within
4-6 hours (NHLBI goal: 4 hours)
PAS
5-7
Discharge Home
Continue treatment with inhaled SABA
Assess PAS before discharge
Continue course of oral SCS
Continue on ICS; if not on long-term
control therapy, consider initiation of ICS
Patient education: review medications,
inhaler technique, environmental control
measures; action plan; medical follow-up
PAS
8-11
Management until ward
admission
Continue ED management until floor
team enters admission protocol orders
Oxygen to achieve SpO2 >94%
SCS
If improvement, continue current
treatment
Reassess: PAS q1-2 hours
Consider adjunct therapies (see box)
PAS
12-15
Severe Exacerbation
Oxygen to achieve SpO2 >94%
NPPV: CPAP, BiPAP
Sedation: consider for NPPV
IV fluids
Magnesium
Neb SABA & ipratropium; systemic
SABA
Consider sodium bicarbonate, pH <7.2
IV SCS
Consider adjunct therapies (see box)
unsuitable for or
failed NPPV
Adjunct Therapies
Systemic SABA:
- SC terbutaline or epinephrine
- IV terbutaline
- IV epinephrine
NPPV
Ketamine
Heliox
Nebulized steriod
Theophylline
Admit Hospital Ward Team
Use asthma inpatient admission order
set
Discharge: contact ward team
PAS
12-15
Respiratory failure:
RSI & Intubation
Mechanical ventilation
Sedation, Paralytics, Analgesics
Oxygen to achieve SpO2 >94%
Continue severe exacerbation treatment
Consider adjunct therapies (see box)
Admit to Critical Care Unit
Use ICU admission order set
FIGURE 120-3. ED management of acute asthma exacerbations, Vanderbilt asthma protocol. BiPAP = bi-level positive airway pressure; CPAP = continuous positive airway
pressure; FEV1 = forced expiratory volume in 1 second; HR = heart rate; ICS = inhaled corticosteroid; ICU = intensive care unit; Neb = nebulizer; NHLBI = National Heart,
Lung, and Blood Institute; NPPV = noninvasive positive pressure ventilation (BiPAP or CPAP); PAS = pediatric asthma score; PEF = peak expiratory flow; RR = respiratory
rate; RSI = rapid sequence intubation; SABA = short-acting β2 agonist; SCS = systemic corticosteroid; SpO2 = oxygen saturation by pulse oximetry. (Courtesy of Dominik
Aronsky, MD, PhD, and Judith Dexheimer, MPH, Department of Informatics, Vanderbilt University School of Medicine.)
CHAPTER 120: Wheezing in Infants and Children
RSI, PPV
Increasing
severity and/or
lack of
treatment
response
FIGURE 120-4. Stepped-care acute asthma
management. Dotted lines indicate the need
to progress directly to IV medications in the
presence of ventilation inadequate to deliver
nebulized medications. The ED physician
should determine the stepped-care algorithm appropriate to local resources and
availability of treatment modalities. BiPAP =
bi-level positive airway pressure; epi = epinephrine; MDI = metered dose inhaler; neb
= nebulized; PPV = positive pressure ventilation; RSI = rapid-sequence intubation.
805
Heliox
BiPAP
Ketamine
Theophylline
IV Magnesium
IV b agonists
IV/neb epi
Impaired
ventilation
Albuterol/ipratropium
(1–2 h continuous)
Systemic corticosteroid
±Oral steroid
Albuterol MDI or
one-time neb
Oxygen
fects than older, less specific agents and are the mainstay of acute asthma
treatment. They provide prompt relief of bronchospasm and acute
symptoms (cough, chest tightness, and wheezing) and prevent exerciseinduced bronchospasm.
Albuterol is currently the most widely available and inexpensive β2 agonist and should be used primarily. Albuterol relaxes airway smooth
muscle and results in increased airflow within 3 to 5 minutes after administration. Children receiving continuous administration of albuterol
(15 milligrams/h) delivered in a 70:30 helium to oxygen mixture (heliox)
have more rapid clinical improvement and reduced need for hospitalization.75 However, heliox therapy is an expensive modality not warranted
in most children with asthma exacerbations.
Albuterol is a racemic mixture of R- and S-enantiomers. The R-enantiomer is more active therapeutically than the S-enantiomer, and there
has been concern that the latter may cause more side effects, particularly
tachycardia and tremulousness. Levalbuterol is comprised only of the Renantiomer and has been marketed as the preferred formulation. However, clinical studies have not demonstrated any therapeutic advantage
of levalbuterol over the much less expensive albuterol, and it appears to
be appropriate only in those children experiencing untoward tremulousness or tachycardia from albuterol.76
The use of continuous nebulizer treatment (15 to 25 milligrams/h albuterol) with β2 agonists results in fewer hospitalizations than the use of
intermittent treatments (2.5 milligrams albuterol), especially in children
with severe airway obstruction.77 Overall costs for a 1- to 2-hour continuous treatment are less than those for multiple intermittent treatments.
These considerations warrant use of continuous nebulizer treatments in
all but mild exacerbations. Both low- and high-flow nebulizers [e.g., the
AirLife Misty Finity® (Cardinal Health, Dublin, OH)] are available with capacities for 1- to 4-hour nebulization. Equivalent bronchodilation can also
be achieved in the cooperative patient with a mild to moderate exacerbation through use of a high dose (4 to 12 actuations) of a short-acting β2 agonist using a metered dose inhaler with a valved holding chamber.29
There is no therapeutic advantage of β2 agonist administration by the
IV or SC route versus the inhaled route in the child who is ventilating
reasonably well. However, for the patient with significantly diminished
air entry, these routes may allow for critical initial bronchodilation that
permits subsequent nebulized drugs to reach distal airways (IV magnesium is also appropriate in this setting; see the section Magnesium below).78,79 Medications available in the U.S. for this purpose are
terbutaline and epinephrine. Epinephrine has α agonist properties that
facilitate vasoconstriction and rapid resolution of mucosal edema. These
agents appear to be safe in young patients without coronary artery disease. Oral albuterol is not recommended due to the delayed onset and
prominent tachycardia, tremulousness, and behavioral changes accompanying this form.
Time
Anticholinergic Agents Bronchial smooth muscle tone also is influenced by
the parasympathetic nervous system through acetylcholine binding to
muscarinic receptors. This mediates bronchoconstriction via M1 and M3
receptors or potential bronchodilation via feedback inhibition of acetylcholine release through the M2 receptor. Ipratropium binds nonselectively to these receptors and most often results in bronchodilation,
although occasionally bronchospasm may occur as a result of predominate M2 receptor activation. Addition of multiple high doses of ipratropium (0.5 milligrams) to nebulized albuterol treatments has additive
benefit and results in reduced hospitalization in children with moderate
to severe exacerbations.80
Corticosteroids Systemic corticosteroids serve two functions in asthma
management: (1) inhibition of the inflammatory cascade, and (2) enhancement of β-receptor expression, sensitivity, and function. Systemic
corticosteroids are a mainstay of acute asthma treatment except in mild,
intermittent disease. Systemic corticosteroids provide rapid beneficial
physiologic effects, usually within 4 hours, and reduce both the need for
hospitalization and the likelihood of relapse.81,82 Most patients presenting to the ED for an exacerbation should be treated immediately with
systemic corticosteroids and discharged on a 3- to 5-day course of the
drugs to prevent early relapse.83,84
The dose is prednisone, 1 to 2 milligrams/kg or equivalent, both in the
ED and daily as the discharge dose. Oral preparations are not palatable,
and adherence to the prescribed regimen is poor (50% to 60% fill rates).85
Dexamethasone phosphate given as either a single PO or IM (0.6 milligram/kg) dose or as a dose in the ED and another the following day (each
0.6 milligram/kg) is therapeutically equivalent to 5 days of prednisone.86–88 When the choice is made to use dexamethasone, however, care
must be taken to use only the phosphate form; the acetate form is a longacting, repository form that may result in prolonged adrenal suppression
and should not be used.
It is not clear whether inhaled corticosteroids provide an additional
benefit over that of systemic corticosteroids in the ED.89 However, inhaled
corticosteroids are an essential part of maintenance therapy and should be
prescribed to all patients with persistent asthma (i.e., all except those with
mild, intermittent asthma) at the time of ED discharge (Table 120-13).
Magnesium Magnesium inhibits histamine release from mast cells and
acetylcholine release from cholinergic nerve endings. However, the primary mechanism for bronchodilation appears to be inhibition of smooth
muscle contraction through competition for the calcium channel. Systematic reviews have consistently found IV magnesium to be both safe and effective for both children and adults.90–92 The benefit of IV magnesium is
particularly notable in children and in those with severe exacerbations.
Both decreased rates of hospitalization and improved pulmonary function have been demonstrated with use of magnesium. IV magnesium
806
SECTION 12: Pediatrics
TABLE 120-12 Dosages of Drugs for Asthma Exacerbations
Medication
Inhaled Short-Acting β2 Agonists
Albuterol
Nebulizer solution, 2.5 milligrams/3
mL
Pediatric Dosage
Comments
0.15 milligram/kg (minimum 2.5 milligrams) every 20
min for three doses, then 0.15–0.3 milligram/kg up to
10 milligrams every 1–4 h as needed, or 15–40 milligrams/h as continuous nebulization
4–8 puffs every 20 min for three doses, then every
1–4 h inhalation maneuver as needed
Use large-volume nebulizers for continuous administration. May mix
with ipratropium nebulizer solution.
Levalbuterol
Nebulizer solution
0.63 milligram/3 mL, 1.25 milligrams/0.5 mL, 1.25 milligrams/mL
MDI (45 micrograms/puff)
0.075 milligram/kg (minimum dose, 1.25 milligrams)
every 20 min for three doses, then 0.075–0.15 milligram/kg up to 5 milligrams every 1–4 h as needed
Levalbuterol administered in one half the milligram dose of
albuterol provides comparable efficacy and safety but offers no therapeutic advantage over albuterol. May be associated with less tachycardia and tremulousness than albuterol. Administration by
continuous nebulization has not been evaluated. More expensive
than albuterol.
Systemic β2 Agonists
Epinephrine
1:1000 (1 milligram/mL)
Terbutaline (1 milligram/mL)
SC: 0.01 milligram/kg up to 0.3–0.5 milligrams every
20 min for three doses
MDI (90 micrograms/puff)
See albuterol MDI dose
May mix in same nebulizer with albuterol. Should be added to shortacting β2 agonists for moderate-severe exacerbations.
Use with VHC as with albuterol.
May be used for up to 3 h in the initial management of severe exacerbations.
Use with VHC as with albuterol.
Total course of systemic corticosteroids for an asthma exacerbation
requiring an ED visit or hospitalization is 3–10 d. For courses <1 wk,
dose taper is not needed.
May mix IV solution with cherry syrup for PO administration.
May be used to delay or avoid endotracheal intubation or as induction agent for this procedure.
6 milligrams/kg bolus over 30 min followed by contin- Obtain serum theophylline level 30 min after bolus and every 2 h
uous infusion:
while in ED with goal of serum level of 10–4 milligrams/dL. This
drug has potential for significant toxicity.
Age 6–12 mo: 0.5 milligram/kg/h
Age 1–9 y: 0.8 milligram/kg/h
Age ≥10 y: 0.65 milligram/kg/h
Abbreviations: MDI = metered dose inhaler; VHC = valved holding chamber.
*Oral administration of systemic corticosteroids is as effective as parenteral administration.
†Acetate
Its α agonist activity may promote vasoconstriction and relieve mucosal edema, but no proven advantage of systemic therapy over aerosol.
SC: 0.01 milligram/kg every 20 min for three doses
No proven advantage of systemic therapy over aerosol if patient
then every 2–6 h as needed
ventilating sufficient for aerosol drug delivery.
IV: 10 micrograms/kg bolus over 5–10 min, then
1 microgram/kg/min; may increase 0.1 microgram/kg/
min every 10 min
Anticholinergics
Ipratropium bromide
Nebulizer solution (0.25 milligram/mL) 0.50 milligram with each continuous albuterol treatment,
or 0.25–0.50 milligram every 20 min for three doses
MDI (18 micrograms/puff)
4–8 puffs every 20 min as needed for up to 3 h
Ipratropium with albuterol
Nebulizer solution (0.5 milligram ipra- 1.5 mL every 20 min for three doses, then as needed
tropium and 2.5 milligrams albuterol
per 3-mL vial)
MDI (18 micrograms ipratropium and 4–8 puffs every 20 min as needed for up to 3 h
90 micrograms albuterol per puff)
Systemic Corticosteroids*
Prednisone (PO)
1–2 milligrams/kg/day (maximum 60 milligrams/day)
Methylprednisolone (IV)
Prednisolone (PO)
15 milligrams/5 mL, 5 milligrams/mL
Dexamethasone phosphate†
0.6 milligram/kg/d for 1 or 2 d; may administer PO, IV,
or IM
Ketamine
2 milligrams/kg followed by 2–3 milligrams/kg/h
Methylxanthines
Aminophylline (86% theophylline)
Use VHC; add mask for ages <4 y. In mild-to-moderate exacerbations, MDI plus VHC is as effective as nebulized therapy with appropriate administration technique and coaching by trained personnel.
forms of dexamethasone and other systemic corticosteroids are long acting and should be avoided.
CHAPTER 120: Wheezing in Infants and Children
807
TABLE 120-13 Estimated Comparative Daily Doses for Inhaled Corticosteroids
Ages ≥12 y
Ages 5–11 y
Drug
Low Dose
Budesonide dry powder inhaler
180 micrograms
(Pulmicort)
90, 180 micrograms/inhalation
Budesonide nebulizer suspension
0.5 milligrams
0.25, 0.5, 1 milligram/2 mL nebulizer
Fluticasone HFA (Flovent)
88–176 micrograms
44, 110, 220 micrograms/inhalation
Medium Dose
High Dose
Low Dose
Medium Dose
High Dose
360 micrograms
720 micrograms
360 micrograms
720 micrograms
1440 micrograms
1.0 milligrams
2.0 milligrams
1.0 milligrams
2.0 milligrams
2.0 milligrams
>177–352 micrograms
≥352 micrograms 88–264 micrograms >265–440 micrograms
≥440 micrograms
Note: Budesonide nebulizer suspension is the only inhaled corticosteroid with U.S. Food and Drug Administration–approved labeling for children <4 y of age.
Reproduced with permission from Expert Panel Report 3: Guidelines for the Diagnosis and Management of Asthma. National Heart, Lung, and Blood Institute, National Asthma Education and
Prevention Program. Bethesda, MD. U.S. Department of Health and Human Services, National Institutes of Health, National Heart, Lung, and Blood Institute; 2007. Available at: http://
www.nhlbi.nih.gov/guidelines/asthma/asthgdln.pdf. Accessed April 14, 2010.
therapy may serve a particular role in the patient who presents with diminished air entry and in whom nebulized medications are less effective.
The rapid bronchodilation resulting from this treatment may allow nebulized medications to more effectively reach peripheral airways and allow
for improved outcome. There is no clear dose-response effect, and a higher dose is favored (75 milligrams/kg, maximum 2.5 grams). Inhaled magnesium is not any more effective than inhaled short-acting β2 agonists.93,94
Ketamine Ketamine is a dissociative sedative agent familiar to most
emergency physicians and used frequently for both procedural sedation
and as an induction agent for endotracheal intubation. The sympathomimetic properties of ketamine also make it the ideal induction agent for
the patient with respiratory failure due to asthma. A limited case series
has reported the effectiveness of a bolus (2 milligrams/kg) followed by a
continuous infusion (2 to 3 milligrams/kg/h) of ketamine in children
with severe asthma who were approaching respiratory failure.95 Use of
ketamine resulted in prompt improvement and avoided the need for
endotracheal intubation. This is an appealing use of ketamine, because it
may allow one to avoid the hazards of endotracheal intubation and mechanical ventilation in the patient with asthma.
Heliox During normal, tidal breathing, airflow is laminar, and resistance
to flow is inversely proportional to the fourth power of airway radius.
With severe airway narrowing, gas velocity increases, and airflow becomes turbulent. Turbulent flow increases airway resistance and the
work of breathing, and results in a decreased deposition of aerosolized
medication in the distal airways. Heliox is a gaseous mixture of helium
and oxygen available in several concentrations (80:20, 70:30, 60:40). The
concept behind the use of heliox is to convert turbulent flow back to laminar flow as a result of the lower gas density of helium compared with oxygen. However, the low oxygen fraction limits its use in the hypoxemic
child, and studies to date have not demonstrated benefit in most patients
with acute asthma.96 Heliox therapy is also appreciably more expensive
than other interventions and has limited availability.
Heliox does appear to provide benefit in patients with severe airway
obstruction and is an appropriate intervention to consider in patients
approaching respiratory failure. In addition, children treated with continuously delivered albuterol with heliox as the driving gas had greater
clinical improvement than those treated with oxygen alone.75
Methylxanthines Intravenous aminophylline and oral theophylline were
mainstays of acute and chronic asthma management for many years.
However, the use of these agents has declined greatly with the advent of
effective inhaled short-acting β2 agonists over the past two decades. This
decline is attributable to the low therapeutic index of aminophylline, the
multitude of drug interactions occurring with aminophylline, and the
rapid bronchodilation achieved with short-acting β2 agonists. Toxic effects of methylxanthines include tachycardia, vomiting, and central nervous system excitation, including seizures.97,98 Aminophylline is not
recommended for treatment of acute asthma because of the limited benefit and significant side effects.29
IV aminophylline continues to be used, however, in management of
the patient with severe acute asthma who is not responding to shortacting β2 agonists and the other aforementioned medications. Other
treatment modalities, including IV β2 agonists, IV ketamine, heliox, and
BiPAP (if available), should precede use of aminophylline. Intensivists
may request that the ED physician initiate IV aminophylline in the child
with severe acute asthma who has not responded to inhaled short-acting
β2 agonists and ipratropium, systemic corticosteroids, and IV magnesium, or who appears to be approaching respiratory failure. If aminophylline is to be used, the goal is a serum theophylline level of 10 to
14 milligrams/dL. Dosages to achieve this are generally a 6 milligrams/
kg bolus over 30 minutes followed by a continuous infusion based on patient age: 6 to 12 months, 0.5 milligram/kg/h; 1 to 9 years, 0.8 milligram/
kg/h; ≥10 years, 0.65 milligram/kg/h.99 Serum theophylline level should
be measured 30 minutes after the aminophylline bolus and every 2 hours
while the patient is in the ED.
■ TREATMENT OF NEAR-FATAL ASTHMA
Patients with acute severe asthma who do not respond adequately to
maximal medical therapy and who continue to manifest severe airway
obstruction and hyperinflation are at risk for fatal asthma. One third of
pediatric deaths from asthma are in children who previously had only
mild asthma. Risk factors for potentially fatal asthma are listed in Table
120-14.56,100
Persistent airway obstruction and hyperinflation result in decreased
mechanical efficiency of the respiratory musculature and eventually a
mixed respiratory and metabolic acidosis. Hypercarbia is the hallmark of
ventilatory failure. However, children tolerate moderate levels of hypercarbia, and progression to respiratory failure is the greatest concern.101
Clinical signs of this progression include worsening hypoxemia, increasing pulsus paradoxus (although this sign may be absent in the patient too
fatigued to provide sufficient respiratory effort), increasing respiratory
rate, diaphoresis, inability to speak, somnolence, and fatigue. Therefore,
the decision to undertake endotracheal intubation is based on clinical
judgment. The ED physician must establish the point in this progression
at which assisted ventilation will be instituted, the safest manner in which
to do so, and the appropriateness of a trial of noninvasive ventilation.
Management of Ventilatory and Respiratory Failure in Children • Assisted
Ventilation The guiding principles of assisted ventilation are to minimize
hyperinflation, to avoid excess airway pressures, and to provide adequate
oxygenation. Application of positive pressure ventilation may also result
in decreased systemic venous return, decreased cardiac output, and rapid deterioration. However, positive pressure ventilation decreases left
ventricular afterload and usually improves cardiac output.
808
SECTION 12: Pediatrics
TABLE 120-14 Risk Factors for Near-Fatal Asthma
Racial/ethnic factors
Black, Hispanic, other nonwhite children
Medical factors
Prior exacerbation with:
Severe, unexpected, rapid deterioration
Respiratory failure
Loss of consciousness or seizure
Attacks precipitated by food
Psychosocial factors
Denial or failure to perceive severity of illness
Depression or other psychiatric disorder
Nonadherence with medication regimen and/or other management
Dysfunctional family dynamics
Inner-city residence
Reproduced with permission from Werner HA: Status asthmaticus in children: a review.
Chest 119: 1913, 2001.
Of much greater concern are the potential hazards of endotracheal intubation in the patient with acute severe asthma. These include sudden,
prolonged, and difficult-to-reverse bronchospasm; laryngospasm; pneumothorax; ventilator-associated pneumonia; and hemodynamic instability.102,103 Thus the decision to perform endotracheal intubation in a
patient with acute severe asthma is not a case of “if you think about it you
should do it.” Rather, the consideration of endotracheal intubation
should prompt one to think about what the decision points will be,
whether noninvasive ventilation is available and appropriate, and what
are the safest means to perform each procedure.
Noninvasive Positive Pressure Ventilation Noninvasive positive pressure ventilation is the provision of ventilatory support without the use of an
endotracheal airway. This technique requires less sedation than endotracheal intubation and does not require use of paralytics. It allows preservation of the patient’s airway reflexes and decreases the likelihood of
pneumonia. However, it does not protect the airway from aspiration.
The noninvasive modality most appropriate in the care of the patient
with severe, nonresponsive asthma is BiPAP (Figure 120-5). BiPAP consists of inspiratory (IPAP) and expiratory (EPAP) positive airway pressures. IPAP offsets auto-PEEP and unloads inspiratory muscles, decreasing
TABLE 120-15 Drugs for Rapid-Sequence Intubation
of a Child with Near-Fatal Asthma
Drug
Dose
Atropine*
0.02 milligram/kg (minimum dose, 0.5 milligram; maximum dose, 1 milligram)
1.5 milligrams/kg
2 milligrams/kg
Lidocaine*
Ketamine†
Short-term paralysis during
intubation
Succinylcholine
or
Rocuronium
Postintubation paralysis‡
Vecuronium
Postintubation sedation
Ketamine‡
or
Fentanyl
and
Midazolam
2 milligrams/kg
1 milligram/kg
0.1 milligram/kg/h
2–3 milligrams/kg/h
2 micrograms/kg bolus then 1 microgram/kg/h
0.1 milligram/kg/h
*3 min prior to endotracheal intubation.
†Immediately
prior to paralytic agent.
‡Patient-ventilator
dyssynchrony may necessitate paralysis. In most instances it is preferable
to avoid paralysis and provide sedation with fentanyl and midazolam. The use of ketamine
sedation may also obviate the need for paralysis.
the work of breathing. It also appears to have a bronchodilatory effect.104
EPAP serves to “stent” airways, thus allowing upstream airways to more
fully decompress and decreasing the air trapping and hyperinflation that
occur as a result of dynamic airway compression. The net result is to decrease the work of breathing and improve gas exchange.
Use of a well-fitting face mask and close attention to patient comfort are
essential. Most patients tolerate BiPAP well, but sedation is frequently required with low doses of benzodiazepines (0.05 to 0.1 milligram/kg of midazolam or lorazepam) or ketamine (0.5 to 1 milligram/kg followed by 0.25
milligram/kg/h). Reasonable initial applied BiPAP pressures for severe
asthma are an IPAP of 12 cm H2O and EPAP of 6 cm H2O, with ranges
of 12 to 18 cm H2O and 6 to 12 cm H2O, respectively.104–106 Aerosolized
medications can be administered through the BiPAP airway circuit.
Decreases in respiratory rate and work of breathing indicate adequate
response to treatment. The largest study of BiPAP use in pediatric patients found that 88% tolerated the intervention and demonstrated clinically significant improvements in respiratory rate and oxygenation
without adverse effects.106 BiPAP may be effective in obviating the need
for endotracheal intubation even if applied for only several hours. A
more comprehensive review of the technique of BiPAP is available.104
Endotracheal Intubation and Assisted Ventilation Approximately 0.5% of children hospitalized with acute asthma require endotracheal intubation.107
TABLE 120-16 Initial Ventilator Settings for the Intubated
Pediatric Patient with Asthma
FIGURE 120-5. Use of bi-level positive airway pressure in a young child with
severe asthma. Inspiratory positive pressure offsets auto–positive end-expiratory
pressure, and expiratory positive pressure stents airways, allowing more complete
airway decompression.
Ventilator Parameter
Setting
Mode
Synchronized intermittent mandatory ventilation/
pressure-regulated volume control
6–10 mL/kg
45 cm H2O
8–15/min
0.5–1.0 s
4–8 s
Tidal volume
Peak pressure
Respiratory rate
Inspiratory time
Expiratory time
GREEN ZONE
YELLOW ZONE
Date:
2 or
4 puffs
How much to take
5 to 60 minutes before exercise
When to take it
2 or
4 puffs, every 20 minutes for up to 1 hour
Nebulizer, once
before/
within
(oral steroid)
(short-acting beta2-agonist)
mg
4 or
Nebulizer
NOW!
(3–10) days
FIGURE 120-6. Asthma action plan. (The blank form is available from the National Heart, Lung, and Blood Institute at: http://www.nhlbi.nih.gov/health/public/lung/asthma/asthma_actplan.htm.) HEPA = high-efficiency particulate air (filter).
(phone)
6 puffs of your quick-relief medicine AND
Take
Go to the hospital or call for an ambulance
6 puffs or
Lips or fingernails are blue
4 or
mg per day For
hours after taking the oral steroid.
Then call your doctor NOW. Go to the hospital or call an ambulance if:
You are still in the red zone after 15 minutes AND
You have not reached your doctor.
(oral steroid)
(short-acting beta2-agonist)
Call the doctor
Add:
If your symptoms (and peak flow, if used) return to GREEN ZONE after 1 hour of above treatment:
Continue monitoring to be sure you stay in the green zone.
-OrIf your symptoms (and peak flow, if used) do not return to GREEN ZONE after 1 hour of above treatment:
Take:
2 or
4 puffs or
Nebulizer
(short-acting beta2-agonist)
Add: quick-relief medicine—and keep taking your GREEN ZONE medicine.
Take this medicine:
Second
First
Medicine
Take these long-term control medicines each day (include an anti-inflammatory).
Doctor:
Hospital/Emergency Department Phone Number
Trouble walking and talking due to shortness of breath
Peak flow: less than
(50 percent of my best peak flow)
DANGER SIGNS
RED ZONE
-Or-
Very short of breath, or
Quick-relief medicines have not helped, or
Cannot do usual activities, or
Symptoms are same or get worse after
24 hours in Yellow Zone
Medical Alert!
Peak flow:
to
(50 to 79 percent of my best peak flow)
-Or-
Cough, wheeze, chest tightness, or
shortness of breath, or
Waking at night due to asthma, or
Can do some, but not all, usual activities
Asthma Is Getting Worse
Before exercise
My best peak flow is:
Peak flow: more than
(80 percent or more of my best peak flow)
And, if a peak flow meter is used,
No cough, wheeze, chest tightness, or
shortness of breath during the day or night
Can do usual activities
Doing Well
For:
Doctor’s Phone Number
Asthma Action Plan
CHAPTER 120: Wheezing in Infants and Children
809
810
SECTION 12: Pediatrics
These children are in hemodynamically unstable condition, they are hypoxic, and although functional residual capacity is increased, their high
metabolic demand causes them to experience rapid desaturation. Furthermore, postintubation ventilatory management is challenging because positive pressure ventilation may worsen hyperinflation and precipitate
hypotension. Consideration must be given to the safest methods for both
endotracheal intubation and mechanical ventilation. In addition, when it
appears that the patient may be progressing toward respiratory failure, pediatric critical care colleagues should be consulted to determine preferred
ventilator settings and address other critical issues to facilitate admission to
a critical care setting.
Use of cuffed endotracheal tubes even in young children has become
acceptable in order to better regulate airway pressure. However, in order
to minimize airway resistance, the largest appropriate tube size should be
chosen, and this consideration has tempered enthusiasm for use of a
cuffed tube. The patient should be preoxygenated without providing positive pressure, bag-valve mask ventilation in order to avoid further hyperinflation. A normal saline fluid bolus (20 mL/kg) should be given prior to
intubation, if possible, to minimize risk of hypotension.
Drugs for rapid-sequence intubation must be chosen carefully (Table
120-15) and should be administered with the patient sitting up or in another comfortable position. Premedication with lidocaine may decrease
laryngospasm and bronchospasm. Atropine should be administered to
children <10 years of age. Lidocaine and atropine should be administered at least 3 minutes prior to intubation. Ketamine is the preferred induction drug because it generally provides bronchodilation and does not
directly cause hypotension. It should be administered immediately before the paralytic agent. A short-acting paralytic, either succinylcholine
or rocuronium, should be used in order to obtain optimal intubating
conditions and to minimize airway trauma. Endotracheal intubation
should be performed by the most experienced operator available. Once
the endotracheal tube is in place, an appropriate tidal volume and a sufficiently low respiratory rate to allow for expiratory emptying and avoid
hyperinflation should be provided manually.
The key pulmonary mechanics of the patient are hyperinflation, autoPEEP, and markedly prolonged expiratory time constants that result in
failure of the airways to empty. Mechanical ventilation must be tailored
to these features. The basic principles of ventilator management are thus
to use low respiratory rates and tidal volumes, long expiratory times, and
high flow rates (Table 120-16).56,108
The application of PEEP is controversial. PEEP may offset auto-PEEP
and decrease the work of breathing, although this is not relevant in the mechanically ventilated patient. And although PEEP may decrease hyperinflation by stenting airways, it also has the potential to aggravate gas
trapping and hyperinflation. Of greatest importance, the patient should be
closely monitored for such adverse events, and ventilator settings adjusted
as appropriate. Consultation with pediatric critical care colleagues or others with expertise in pediatric ventilator management is warranted.
The practice of controlled hypercapnic hypoventilation is a recommended ventilator strategy.29 Its use was first reported by Darioli and
Perret to minimize hyperinflation and airway pressures and to provide
adequate oxygenation.101 This mode of ventilation may be key to allowing for the prolonged expiratory times necessary to minimizing autoPEEP and hyperinflation. This is generally accomplished by providing
high inspiratory flow rates and decreased minute ventilation. Respiratory acidosis may be treated with IV sodium bicarbonate.29 Adequate sedation must be provided to enable the patient to tolerate this mode of
ventilation and to avoid ventilator dyssynchrony, tachypnea, and breath
stacking. Ketamine, benzodiazepines, and opiates, or a combination, are
appropriate, and paralysis is usually initially necessary. Paralytics should
be withdrawn as soon as possible to avoid myopathy and prolonged ventilator dependence due to neuromuscular blockade.109
■ DISPOSITION AND FOLLOW-UP
The decision regarding hospital admission or discharge of the child with
asthma must consider both the adequacy of response to treatment and
the ability of the patient and caretaker to provide necessary ongoing care.
The ED relapse rate of 7% to 15% reflects the potential uncertainty of
this decision.39,40,100 Clinical guidelines to guide the decision for discharge have included return of FEV1 or PEF to ≥70% of predicted value
or personal best, but the required measurements cannot be performed in
many children.29 Improvement so that only minimal symptoms remain
is a useful guide for discharge, and most patients should be observed for
30 to 60 minutes after the most recent bronchodilator dose.
A visit to the ED may be an indication of inadequate long-term asthma management and insufficient understanding of how to manage an
exacerbation. Prior to discharge from the ED or hospital, the patient
must be provided with the necessary medications and instructions on
when and how to use them. Referral for follow-up appointments and an
asthma action plan for recognizing and managing relapse or future exacerbations must also be provided (Figure 120-6).
The patient should have or be prescribed an inhaled short-acting β2
agonist. If a metered dose inhaler is to be prescribed (either short-acting
β2 agonist or inhaled corticosteroid), a valved holding chamber (e.g.,
AeroChamber®) should also be prescribed and the patient instructed in
proper metered dose inhaler use. Patients treated with systemic corticosteroids should be prescribed a 3- to 5-day oral course. Inhaled corticosteroids should be prescribed for all classes of asthma severity except
mild intermittent asthma.73 Dosages of frequently prescribed inhaled
corticosteroids are provided in Table 120-13.
Acknowledgments: The authors gratefully acknowledge the contributions of Maybelle Kou and Thom Mayer, the authors of this chapter in
the previous edition.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
121
Pneumonia in Infants
and Children
Joseph Copeland
Pneumonia is an infection of the lung and respiratory tract below the
level of the larynx. Globally it is a leading cause of morbidity and mortality, with up to 1.9 million childhood deaths per year in the developing
world.1 Even in industrialized countries, according to World Health Organization estimates, there are 4 million cases annually in children <5
years of age.2
This chapter addresses the clinical and radiographic diagnosis of
pneumonia, common viral and bacterial causes, evidence-based and historically based treatments, and appropriate consultation and follow-up
for children seen in the ED.
An exhaustive discussion of pathogens and preexisting pediatric
pathology is beyond the scope of this text. However, wherever possible,
special mention is made of unusual microbes, changing patterns of immunization and resistance, and special considerations for children with
underlying medical problems. For the clinician with less pediatric experience, a brief overview on the use and interpretation of chest radiographs in this population is included.
EPIDEMIOLOGY
In the vast majority of cases, pediatric pneumonia is caused by viral or
bacterial agents, with a smaller number due to unusual pathogens such
as Mycobacterium tuberculosis or opportunistic organisms such as fungi.
In most cases the etiologic agent is never known. Definitive microbiologic diagnosis requires invasive procedures such as bronchial lavage,
sampling of pleural effusion for culture, or lung puncture, which are un-
CHAPTER 121: Pneumonia in Infants and Children
TABLE 121-1 Age-Specific Causes of Pneumonia
in Otherwise Healthy Children
Age Group
Pathogen (in order of frequency)
1–3 mo
Pneumonitis syndrome, usually afebrile: Chlamydia trachomatis,
RSV, other respiratory viruses, Bordetella pertussis
Mild to moderate pneumonia: RSV, other respiratory viruses,
Streptococcus pneumoniae, Hib, NTHi, C. trachomatis, Mycoplasma pneumoniae
Respiratory viruses, S. pneumoniae, Hib, NTHi, M. pneumoniae,
C. pneumoniae
M. pneumoniae, S. pneumoniae, C. pneumoniae, NTHi, influenzavirus A or B, adenovirus, other respiratory viruses
Severe pneumonia requiring intensive care unit admission: S.
pneumoniae, Staphylococcus aureus, group A streptococci, Hib,
M. pneumoniae, adenovirus
1–24 mo
2–5 y
6–18 y
All ages
Abbreviations: Hib = Haemophilus influenzae type b; NTHi = nontypeable Haemophilus
influenzae; RSV = respiratory syncytial virus.
available or impractical in the ED. Although blood cultures are frequently performed for the very sick and the very young, they have limited
relevance in the ED. They are used primarily to refine treatment at a later
date, once culture results are known.
Most diagnostic algorithms estimate the likely cause of pneumonia
based on the patient’s age and the presumed incidence and prevalence of
particular microbes for each age group. Detailed summaries exist in the
literature,3 but typical causes of community-acquired pneumonia in
healthy children are listed in (Table 121-1).4 The clinician still must take
into account local and regional epidemics, individual immunization status, and underlying health problems that may influence which pathogens are likely. Infections vary considerably across the spectrum of age,
and a few general rules and specific exceptions are described below.
■ NEONATES
Neonates (0 to 30 days of age) are at risk from pathogens acquired from the
vaginal canal at or around the time of birth and require special consideration. Specific organisms of concern include group B streptococci, gramnegative enteric bacteria such as Klebsiella and Escherichia coli, and Listeria monocytogenes. Pneumonia due to Chlamydia trachomatis has been
largely eliminated in developed countries through the systematic screening and treatment of pregnant women,5 but is a consideration in children
born to women who have had little or no prenatal care. Any neonate with
pneumonia is also at risk of sepsis, and between birth and 3 months infants
with pneumonia should receive a workup for sepsis (see Chapter 113, Fever and Serious Bacterial Illness). Typical antibiotic treatment in this age
group includes ampicillin to cover Listeria and group B streptococci plus
gentamicin or cefotaxime to cover gram-negative vaginal flora.
■ INFANTS AND CHILDREN YOUNGER THAN AGE 2
Pneumonia in older infants and toddlers is typically viral rather than bacterial.6 Common causes include respiratory syncytial virus (RSV), influenza virus, parainfluenza virus, human metapneumovirus, and adenovirus.
Although viral infections of the lung do not benefit from antibiotic administration, clinicians should be on the lookout for biphasic clinical symptoms (upper respiratory tract symptoms followed by clinical worsening
with high fever and lower tract symptoms), because children with initial
viral infections may be susceptible to secondary bacterial pneumonias.2,7
In this age group, the most common bacterial cause of communityacquired pneumonia remains Streptococcus pneumoniae. Other agents
include Haemophilus influenzae type b (Hib) in nonimmunized children
and nontypeable H. influenzae in all children; less common but important pathogens include Staphylococcus aureus and Bordetella pertussis,
even among immunized children. The role of mycoplasma and Chlamydia pneumoniae in children under 2 is disputed.5,8
811
In older literature, a syndrome of afebrile pneumonitis has been described in children between 1 and 3 months of age, and up to 6 months.
Clinical findings include prolonged staccato cough, tachypnea, scattered
rales but rarely wheezing, and bilateral interstitial infiltrates on radiograph.9 These infants also show progressive respiratory distress and may
require oxygen in some cases. Because C. trachomatis and B. pertussis
have been implicated along with RSV and other respiratory viruses,10
some authors have recommended antibiotic treatment with a macrolide
for these clinical cases.4
■ TODDLERS AND SCHOOL-AGED CHILDREN
As children get older and attend day care or school, they come into contact
with many pathogens, particularly respiratory viruses. Many viral and bacterial agents that once caused significant respiratory morbidity and mortality in this age group, such as influenza virus, varicella-zoster virus,
measles virus, Hib, B. pertussis, and some strains of Pneumococcus, are
seen less frequently due to widespread vaccination and herd immunity.
In children between 2 and 5 years of age, most community-acquired
pneumonia is caused by respiratory viruses, followed by S. pneumoniae,
Hib or nontypeable H. influenzae, and possibly mycoplasma and C. pneumoniae.4,11 These latter two are thought to be less common in children <5
years of age, and macrolides are not considered first-line therapy in this
age group.12
In children from age 5 to adolescence, M. pneumoniae is thought to be
the chief cause of community-acquired pneumonia, with C. pneumoniae
and S. pneumoniae still important considerations.11,13 Less common bacterial causes include S. aureus and Legionella.6 M. tuberculosis is a rare but
important agent to consider.
■ ADOLESCENTS
By adolescence, most patients are assumed to have the same infectious
risks as healthy adults. The prevalence of specific pathogens may vary according to region, season, and cyclical epidemics in the population. In
North America, the role of atypical agents, especially mycoplasma and C.
pneumoniae, is estimated to be significant, but data and guidelines from
other parts of the industrialized world suggest regional differences14
■ UNIQUE AND HIGH-RISK GROUPS
Although uncommon in North America, tuberculosis (TB) remains a
consideration for children with known exposures and those from endemic areas such as Native reserves, Alaska and the Canadian North, and
some inner-city settings. The diagnosis should also be entertained for
immigrants from high-prevalence areas of the world, including Africa,
Asia, and parts of Eastern Europe.
Children with underlying disease are at risk for specific infections. For
example, younger children with cystic fibrosis are often infected with S.
aureus in the first years of life and later with Pseudomonas. Children with
sickle cell disease are particularly susceptible to infection with encapsulated bacteria, acute chest syndrome, and sepsis (see Chapter 135, Sickle
Cell Disease). All require early, aggressive treatment. Children with congenital or acquired immune deficiencies [e.g., human immunodeficiency virus (HIV) infection, malignancy, congenital immunodeficiencies]
are at risk for opportunistic infections with agents such as Pneumocystis,
cytomegalovirus, and fungi.
Children with incomplete immunizations or none at all should also be
approached with special care. They may be partially or fully susceptible
to pneumonia associated with B. pertussis, Hib, and all strains of Pneumococcus, as well as measles, influenza, and varicella-zoster viruses.
CLINICAL FEATURES
■ HISTORY AND COMORBIDITIES
The history at presentation may be quite varied, depending on age of the
patient, the causative agent, and the underlying health of the child. Many
complaints are nonspecific (e.g., cough and fever) and are common to
812
SECTION 12: Pediatrics
both upper and lower respiratory tract disease. The predictive value of specific signs and symptoms is discussed below (see Diagnosis section). Inquiry should be made about the presence, timing, and duration of cough,
fever, rapid breathing, and difficult breathing. The clinician should also
ask about specific exposures, sick contacts, travel, and pets, when relevant.
A history of choking or persistent or recurrent lower respiratory tract
symptoms may suggest foreign-body aspiration. Recurrent pneumonias
may signify underlying disease such as cystic fibrosis, immune disorders,
or anatomic abnormalities. Important history unique to the pediatric patient includes information in some or all of the following four areas: birth
history, medical history, social history, and immunization history.
Birth History Because neonates may acquire infections perinatally, questions should be asked about the mother’s prenatal and perinatal health,
including maternal infections (e.g., chlamydia, gonorrhea, infection with
group B streptococci, genital herpes, and HIV status if known), intrapartum or postpartum fever, and any specific antibiotic or antiviral therapy
received during labor and delivery. Additional neonatal history should
include questions about prolonged rupture of membranes, estimated
gestational age, and immediate peripartum complications. Meconium
aspiration may cause pulmonary signs and symptoms in the first 24 to 72
hours of life from chemical pneumonitis or secondary bacterial bronchopneumonia. Perinatal stays in hospital may indicate an underlying
health problem and increased risk of nosocomial infections.
Medical History The clinician should ask about major illnesses and hospitalizations since birth, especially chronic respiratory problems (e.g.,
asthma, recurrent wheezing). In the young child, there may be unrecognized or undiagnosed respiratory, cardiac, renal, or immune dysfunction. A specialist should be consulted for unusual or recurrent infections.
Children with a known history of congenital respiratory problems
(e.g., cystic fibrosis, neuromuscular disorders, immune compromise) are
at increased risk of infection with common and sometimes rare agents.
They may be at elevated risk of respiratory failure or treatment failure.
Treatment and disposition decisions should be adjusted accordingly (see
Table 121-5).
Social History The treating physician should be brief but focused in inquiring about social history, because it may influence both diagnosis and treatment. For example, children from the Far North, Native reserves, or
countries with high rates of TB may be exposed to this uncommon but serious infection. A travel history may also be relevant. Those born to HIV-positive mothers are at risk of vertical transmission and immunocompromise.
Social history may also influence treatment decisions. If the child is a
candidate for outpatient management, the clinician must ensure that caregivers are capable of understanding instructions and able to afford and
provide the required care (see Disposition and Follow-Up below).
Immunization History Do not assume that the child has had all recommended immunizations. The parent or caregiver should always be asked directly, and records reviewed for confirmation whenever possible. An
unvaccinated child is at risk of serious morbidity, and even death, from vaccine-preventable illness (see Immunization and Controversies of Treatment
below).
■ PHYSICAL EXAMINATION
The physical examination is a key element in the diagnosis of pneumonia.
For the developing world, where imaging, equipment, and laboratory tests
are limited, the World Health Organization has proposed a diagnostic algorithm based entirely on the presence or absence of tachypnea, respiratory
distress, and lower chest indrawing. Although physicians in industrialized
nations have many tools at their disposal, the diagnosis of pneumonia can
still be made clinically. No single physical finding in isolation is diagnostic
of pneumonia, and constellations of signs are more useful.15 At its most basic, the physical examination should include assessment of general appearance, respiratory rate, work of breathing, and auscultation of the chest.
Rapid respiratory rate is a simple, standardized, and sensitive screening
tool for pneumonia.4 Other signs and symptoms of disease are more variable across age groups. Tachypnea is defined as a respiratory rate >60
breaths/min for newborns <2 months old, >50 breaths/min for infants aged
2 to 11 months, and >40 breaths/min for children aged 1 to 4 years. If possible, the rate should be determined when the child is calm or sleeping, usually before the rest of the examination is performed. Breaths should be
counted for a full minute to account for natural variation in pediatric
breathing. Shorter assessments are likely to overestimate the respiratory
rate.15 Fever may increase the rate by up to 10 breaths/min for every 1°C
(1.8°F) rise in patient temperature.16 Children at very high altitudes can
have adaptive increases in resting rate, so other markers such as oxygen saturation may be more useful measures in these rare situations. Note that
children who are severely malnourished or dehydrated, or have impending
respiratory failure may not be capable of generating rapid respiratory rates.
Markers of respiratory distress include nasal flaring, tracheal tug, and
intercostal indrawing. Lower chest or “abdominal” indrawing and grunting suggest more severe pneumonia.17 Poor feeding and lethargy may be
indirect signs of respiratory compromise and should not be missed.
Cough is less likely in neonates or very young children, and productive
cough is rarely seen before late childhood. In young children, abdominal pain may be a clue to lower lobe pneumonia or effusion.
Fever is a common but nonspecific sign of both upper and lower respiratory tract infection.
The chest should be auscultated using an appropriately sized stethoscope with the chest fully exposed. All lung zones should be assessed.
The presence of localized fine crackles (rales), coarse breath sounds
(rhonchi), or diminished breath sounds may be helpful if noted, but their
identification may not be consistent across observers.15 A toxic appearance and overall impression of illness as judged by the clinician show
better diagnostic sensitivity.4
Oxygen saturation is helpful to know when available, because hypoxia on room air (arterial oxygen saturation of <90% at sea level) is
known to increase the risk of oral amoxicillin treatment failure in severe pneumonia.18 Many admit a child with an oxygen saturation of
<90% to 93% or a child who cannot maintain a satisfactory saturation
value without supplemental oxygen delivery.
Taken together, these signs and symptoms can help confirm or exclude the diagnosis of pneumonia. One study of >500 children in a North
American ED found that the combination of fever plus either tachypnea, decreased breath sounds, or fine crackles predicted x-ray–positive pneumonia with a sensitivity of 93% to 96%. The presence of fever
plus all three of the other variables increased sensitivity to 98%, and the
authors suggested that obtaining a radiograph in such cases is not warranted.19 For ruling out pneumonia, one small but frequently cited
study found that the absence of tachypnea, respiratory distress, and
rales or decreased breath sounds accurately excluded the presence of
pneumonia with 100% specificity.20
■ PATTERNS OF DISEASE
Various attempts have been made to correlate physical findings with specific pathogens. For example, “typical pneumonias” classically present
with high fever, chills, pleuritic chest pain, and productive cough, and
suggest a bacterial cause, especially S. pneumoniae. In contrast, “atypical
pneumonias” are characterized by gradual onset over days to weeks, lowgrade fever, nonproductive cough, and malaise, and suggest infection with
agents like mycoplasma or C. pneumoniae. Unfortunately, there is significant overlap in the agents causing these symptoms, and it is not possible to
pinpoint specific causative organisms by clinical findings alone.4,21 If
definitive identification is important, invasive laboratory testing must be
considered.
Despite these limitations, some patterns of disease do suggest certain etiologies. Staphylococcal pneumonia, which may follow influenza, is notorious for rapidly progressing symptoms, high fever, toxicity, and presence of
pulmonary abscesses. C. trachomatis infection in infants often presents with
a staccato cough, diffuse rales, and lack of fever, whereas older children may
complain of sore throat and dysphagia. Mycoplasma infection typically produces a hacking, dry cough and may be associated with extrapulmonary
manifestations that include arthralgias, rash, and even central nervous sys-
CHAPTER 121: Pneumonia in Infants and Children
tem symptoms.22 Paroxysmal cough leading to gasping respirations and color change is characteristic of infection due to B. pertussis (whooping cough);
there is often an upper respiratory tract prodrome and a postinfection cough
that may persist for months. TB should be considered with prolonged cough
in the setting of identified risk factors and characteristic radiographic findings. Note that classic signs and symptoms of TB may be absent in children,
especially those with immune deficiencies.23 TB in infants and young children tends to progress more rapidly from infection to clinical disease, and
hematogenous spread to distant sites is possible.24 Children with active pulmonary TB require respiratory isolation and combination therapy, and consultation with an infectious disease specialist is strongly advised.
Wheezing in a young infant with respiratory infection typically points to
bronchiolitis of viral origin. In older, school-aged children, wheezing may
suggest viral infection or mycoplasma pneumonia.5 Distinguishing between viral and bacterial causes of pneumonia is often difficult, and radiographs may be misleading.
■ WORSENING PNEUMONIA
The child with known pneumonia who returns to the ED with a worsening
clinical picture should always cause concern. If the initial treatment was
supportive care for a presumed viral pneumonia, secondary bacterial
pneumonia or an alternative diagnosis (e.g., cardiac disease, congenital anatomic anomaly, foreign body) should be considered.25 Similarly, a child
receiving antibiotics who returns with diminished breath sounds, dullness
to percussion, or worsening respiratory distress should be investigated for
pleural effusion, empyema, pneumothorax, or other secondary complications. When antibiotic-resistant pneumonia is suspected, blood or pleural
fluid culture may be necessary, because clinical findings cannot reliably
differentiate between drug-resistant and drug-sensitive pathogens.26,27
DIAGNOSIS
■ DIFFERENTIAL DIAGNOSES OF CONSEQUENCE
The differential diagnosis of pneumonia includes both infectious and
noninfectious conditions, as well as extrapulmonary disorders that may
mimic or complicate lower respiratory tract infection (Table 121-2).
This is especially important in pediatric patients, who may have undiagnosed congenital anomalies.
For children with respiratory distress but no fever, a search for causes
other than pneumonia is required. Congenital heart disease may present
with cyanosis, quiet tachypnea, or signs of respiratory distress related to
congestive heart failure (see Chapter 122A, Pediatric Heart Disease:
Congenital Heart Defects, and Chapter 122B, Pediatric Heart Disease:
Acquired Heart Disease). Respiratory distress and Kussmaul breathing
should arouse suspicion of diabetic ketoacidosis or other metabolic disease. Toddlers are particularly at risk for foreign-body aspiration and ingestion of toxins. Adolescents may intentionally or unintentionally take
drug overdoses that speed or slow breathing.
■ LABORATORY EVALUATION
Because the results of most laboratory investigations are not known in
the ED, tests are usually initiated to guide future treatment. Rapid bed-
813
side tests for specific viral infections are an exception. Nasopharyngeal
assays for RSV, influenza, and human metapneumovirus can be valuable, because they are quick and specific, and results may obviate the
need for additional invasive testing, exposure to x-rays, or antibiotic
therapy. Imaging may also be deferred and antibiotics withheld for children who do not have a toxic appearance and who have clinical bronchiolitis, especially those with a positive nasopharyngeal swab finding.3
Bacterial cultures of nasopharyngeal samples are generally not helpful,
because results are delayed and oral flora correspond poorly with the organisms causing disease in the lung. The majority of newer serologic and
polymerase chain reaction techniques to detect organisms such as H. influenzae or C. pneumoniae have not been validated in children and have
produced variable results.3,5 In patients with a toxic appearance and
those destined for admission, many clinicians draw blood samples for
culture to help narrow the spectrum of treatment in hospital, although
the yield of such cultures is low and routine use is not necessary.
When TB is suspected, obtaining induced sputum samples from older
children or gastric aspirates from infants for microscopy and confirmatory culture is helpful.28 These tests require equipment and expertise beyond the scope of the ED.
■ IMAGING
Along with the history and physical examination, the plain radiograph
(“chest x-ray”) is often used to diagnose pediatric pneumonia. Used appropriately, it can help to distinguish between pneumonia and other
types of respiratory infection, and between respiratory and nonrespiratory (e.g., cardiac) sources of symptoms. This section discusses when to
order a chest radiograph and how to recognize some common and unusual findings.
When and Why to Order a Radiograph A chest radiograph should be ordered when clinically indicated and when the results are likely to alter diagnosis, treatment, or outcome. The chest radiograph is not the gold
standard of diagnosis, because it is neither 100% sensitive nor 100% specific.29 In the ED, a small number of children may present with clinical
signs and symptoms of pneumonia before there are radiographic changes, which leads to false negative diagnoses. More commonly, an image
taken with poor inspiration or rotation may give the false impression of a
pulmonary infiltrate (Figures 121-1 and 121-2), which leads to unnecessary treatment with antibiotics. Clinicians who are unaccustomed to ordering and interpreting pediatric radiographs may find this especially
challenging.
Guidelines on the use of routine chest radiography are contradictory.3,30 There are risks and benefits to ordering chest radiograph in febrile
infants. Benefits include diagnosis or confirmation of pneumonia and
occasionally the discovery of a significant congenital abnormality. Risks
and disadvantages include cost, delay, repeated exposure to ionizing radiation, and overdiagnosis of bacterial pneumonia.31 Young children
with straightforward viral bronchiolitis may still show radiographic areas of atelectasis or patchy collapse, tempting emergency physicians to
initiate antibiotic therapy.32
Of note, a randomized trial involving children in Cape Town, South Africa, found that chest radiography had no statistically significant impact
TABLE 121-2 Differential Diagnosis of Pneumonia
Infectious Causes
Noninfectious Causes
Extrapulmonary Causes
Upper respiratory tract infection (“cold”),
otitis media
Bronchiolitis
Foreign-body aspiration
Inhalation pneumonitis (e.g., hydrocarbon inhalation, chronic gastroesophageal
reflux disease)
Intoxication (e.g., salicylate poisoning, carbon monoxide exposure)
Congenital disorders (e.g., cystic fibrosis, sickle cell disease with chest crisis)
Anatomic abnormalities (e.g., congenital lobar emphysema, pulmonary sequestration, tracheoesophageal fistula, congenital cystic adenomatous malformation)
Sepsis
Cardiac anomalies (cyanotic heart disease,
congestive heart failure, myocarditis)
Endocrinopathies (e.g., diabetic ketoacidosis)
Neuromuscular disorders
Inborn errors of metabolism
GI emergencies (e.g., appendicitis with
grunting)
Neoplasm, metastasis
Pulmonary embolism
814
SECTION 12: Pediatrics
FIGURE 121-1. Poor inspiration results in the appearance of pulmonary infiltrates
and cardiomegaly in this normal 4-month-old. (Courtesy of BC Children’s Hospital,
Vancouver, BC, Canada.)
FIGURE 121-2. The same child as in Figure 121-1, with adequate inspiration. Note
that persistent rotation (see clavicles) causes a false difference in left and right lung
density. (Courtesy of BC Children’s Hospital, Vancouver, BC, Canada.)
on clinical outcome in ambulatory children with lower respiratory tract
infections, and the authors recommended against its routine use in children >2 months of age.33 Thus some guidelines state that imaging should
not be performed routinely in children with mild, uncomplicated acute
lower respiratory tract infections.3 Most of these studies and guidelines involved the review of data obtained in ambulatory care settings in which
children with prolonged cough, severe symptoms, and other “red flag” features were excluded, so these recommendations must be applied to the ED
with caution. If imaging is deferred, or radiographs are read as “negative”
for pneumonia, follow-up at 24 to 48 hours should be arranged.
Although routine radiographs are not usually necessary, some potential indications for chest radiography include the following12,30,34,35:
1. Age of 0 to 3 months, as part of a workup for sepsis
2. Age of <5 years in a child with a temperature of >39°C (102.2°F), white
blood cell count of ≥20,000/mm3, and no clear source of infection
3. Ambiguous clinical findings
4. Suspicion of a complication, such as pleural effusion
5. Pneumonia that is prolonged or unresponsive to antibiotics
6. Suspicion of foreign-body aspiration
7. Suspected congenital lung malformation (e.g., sequestration or congenital cystic adenomatous malformation)
8. Follow-up of “round pneumonia” to ensure resolution and exclude an
underlying mass
Note that the child with a toxic appearance who has respiratory findings should always undergo chest radiography, and some infants may
merit imaging as part of a workup for sepsis, even if no clinical signs are
present.
Numerous reviews of the literature have concluded that the chest radiograph cannot reliably distinguish between bacterial and viral causes.36,37 Practically speaking, most children presenting to the ED with
clinical symptoms of pneumonia and obvious lobar or segmental consolidation receive antibiotics.
Tips on Interpreting the Pediatric Chest Radiograph Physicians accustomed to reviewing adult radiographs may find some elements of the pediatric image confusing, and a systematic and age-appropriate approach
to the pediatric chest radiograph is helpful. An exhaustive list of potential abnormalities is neither possible nor necessary, but the following dis-
cussion and images should help the emergency physician to develop
such an approach and recognize significant pathology.
Normal Neonatal Anatomy The chest of a neonate (<1 month of age) has a
more pyramidal or trapezoidal shape than the long, rectangular form of
the adult. The cardiac silhouette may occupy up to 60% or 65% of the chest
width on the posterior-anterior view and still be considered normal. In infants, bronchial branching may be seen beyond the level of the carina, giving the false impression of pathologic air bronchograms. The normal
thymus can be seen as a large, dense, anterior mediastinal “sail” until involution occurs around age 6. A normal thymus can often be recognized by
the sharp inferior edge of its silhouette and occasionally by a “wave” or
“sail” sign at the lateral edge, where the adjacent ribs indent this soft, solid
organ. Occasionally the thymus may be confused with a lobar pneumonia,
mediastinal mass, or hilar lymphadenopathy (Figure 121-3). A lateral
FIGURE 121-3. Arrows indicate a normal thymus. Rotation, apparent from the
location of the heart, trachea, and clavicles, makes this thymus appear to be far
right of midline. (Courtesy of BC Children’s Hospital, Vancouver, BC, Canada.)
CHAPTER 121: Pneumonia in Infants and Children
815
FIGURE 121-4. Lateral view confirms thymic density fully confined to the anterior
mediastinum (arrows). (Courtesy of BC Children’s Hospital, Vancouver, BC, Canada.)
view can help to confirm the anterior location of the thymus. (Figure
121-4). A silhouette that extends behind the heart shadow or posterior to
the vertical lucency of the trachea should be investigated.
Neonatal Pathology Occasionally parents may bring a very young newborn to the ED because of real or perceived breathing difficulties. Noninfectious causes of tachypnea and respiratory distress in the first few days
of life include “wet lung” or transient tachypnea of the newborn, and
chemical pneumonitis from meconium aspiration. The former may cause
increased vascular markings, linear interstitial opacities, and even pleural
effusions on radiographs due to interstitial edema. Meconium aspiration
can block small airways, leading to hyperinflation and bilateral air space
opacities on plain radiographs. A good birth history, physical examination, and chest radiograph may help to distinguish some of these noninfectious causes from infectious ones. (Signs of interstitial opacities or
edema should prompt observation and/or consultation.) Premature babies, intensive care “graduates,” and even healthy, term newborns are still
at increased risk for bacterial pneumonias. Neonatal pneumonia is always treated on an inpatient basis with parenteral antibiotics.
The Toddler In addition to contracting pneumonia from the infectious
causes noted earlier in Table 121-1, toddlers are susceptible to choking
and foreign-body aspiration from objects they can reach. These may include organic materials (e.g., food) or inorganic objects (coins, buttons,
batteries), as well as volatile toxins such as cleaning products, gasoline,
or other hydrocarbons. Retained foreign bodies can lead to respiratory
distress and febrile aspiration pneumonia. Careful review of the chest radiograph is critical, and consultation may be necessary if a foreign body
is not seen but still suspected (e.g., air trapping or segmental collapse
past an obstructed bronchus). By contrast, inhaled hydrocarbons and irritants may yield a pneumonitis with patchy lower air space opacities,
and even pneumatoceles, if the presentation is delayed. Children symptomatic from an inhalation pneumonitis require admission and/or close
follow-up. Tachypnea, respiratory distress, and fever with no radiographic findings may suggest a toxic cause such as salicylate ingestion or
a metabolic disorder, including diabetic ketoacidosis.
The Older Child By school age, the radiographic signs of chest infection in
children become more similar to the typical findings in adults. There may
be obvious lobar consolidation or patchy, multifocal findings. Younger
children can also present with “round pneumonia” (Figure 121-5), a sharp-
FIGURE 121-5. Anterior-posterior (A) and lateral views (B) shows lower lobe consolidation (arrows). (Courtesy of BC Children’s Hospital, Vancouver, BC, Canada.)
ly defined consolidation often found in the posterior lower lobe, classically
from pneumococcal infection. Children with round pneumonias should
have radiographic follow-up to confirm resolution and ensure that this
finding is not actually a mass. Cavitation and pleural effusions should
arouse suspicion of infection with S. aureus or S. pneumoniae, particularly
penicillin-resistant pneumococcus (Figure 121-6).
Patchy atelectasis and air space consolidation in the dependent zones of
the lung should raise suspicion of aspiration pneumonia or pneumonitis.
Recurrent aspiration pneumonias may occur in children with chronic gastroesophageal reflux disease, tracheoesophageal fistula, developmental delay, immobility, or neuromuscular disorders.
816
SECTION 12: Pediatrics
TREATMENT
■ STANDARD TREATMENT
FIGURE 121-6. Complicated left-sided pneumonia. Note the pleural effusion
(white arrows) and cavitation (black arrows). (Courtesy of BC Children’s Hospital,
Vancouver, BC, Canada.)
SPECIAL CONSIDERATIONS
■ TUBERCULOSIS
Children of all ages are susceptible to TB, and most have primary disease,
rather than secondary reactivation. Upper lobe infiltrates and hilar lymphadenopathy on chest radiograph both suggest the diagnosis, but classic findings can be absent in children, especially those with immune
deficiencies.23 Infants and young children tend to progress more rapidly
from infection to clinical disease, and hematogenous spread can lead to
the radiographic “snowstorm” appearance of miliary TB.24 Secondary
TB has a predilection for the superior segments of the lung, as in adults,
and may yield a cavitary lesion in some cases. M. avium lesions may be
indistinguishable from those of secondary TB.
■ OPPORTUNISTIC INFECTIONS
Nodular findings in the lungs may alert the astute clinician to the presence of other, rare infectious agents, such as Histoplasma, Aspergillus,
and Pneumocystis. Pediatric patients with acquired immunodeficiency
syndrome are at risk for infection with most of these, as well as cytomegalovirus infection, lymphomas, and lymphocytic interstitial pneumonia.
■ CYSTIC FIBROSIS
Children with cystic fibrosis have decreased mucus clearance, which
leads to small-airway obstruction. More advanced disease results in
chest radiograph findings such as peribronchial thickening and mucous
plugging with cystic or bullous lung lesions, segmental atelectasis, and
hilar adenopathy. Air trapping may be seen on expiratory views. Patients
with cystic fibrosis are also at risk for pneumothorax. If acute pneumonia is suspected, therapy must be tailored (see Table 121-5).
■ ANATOMIC ABNORMALITIES
Occasionally the initial chest radiograph may uncover an unusual or
unexpected finding. The emergency physician is not expected to recognize all possible anomalies, but when seen, they warrant discussion
with a radiologist and pediatrician to arrange appropriate evaluation
and follow-up.
The treatment of pediatric pneumonia is aimed at alleviating clinical symptoms and managing the underlying cause. General supportive measures independent of etiology include supplemental oxygen, antipyretics, oral or
parenteral fluids to offset respiratory losses, and sometimes bronchodilators
when wheezing is noted. Cough suppressants are not generally indicated in
children, who may depend on the cough to clear mucus. Over-the-counter
cough suppressants have not been shown to be effective and have been withdrawn from the market in Canada for chilren <5 years of age. Narcotic-based
preparations, on the other hand, may be effective cough suppressants and can
be used in select patients without underlying respiratory compromise.
Noninfectious pneumonitis (e.g., chemical inhalation, aspiration) and
pneumonia of suspected viral cause (e.g., age 3 months to 5 years, known
exposure, audible wheezing, positive bedside test for RSV or influenza) are
typically treated with supportive measures as outlined above. Antibiotics
are not indicated. In infants who do not appear toxic and present with RSVpositive bronchiolitis and fever, for instance, the risk of concurrent serious
bacterial infection is extremely low, in some studies as little as 1.1%.38
For children with suspected bacterial pneumonia, antibiotics should
be given promptly. Because definitive information about the causative
organism is usually unknown, the choice of antibiotic is empiric.6 Multiple guidelines exist, with variations based on best evidence, expert
opinion, and standard of care (Tables 121-3 and 121-4).4,6,12,30
In newborns, who are at risk of sepsis, ampicillin is used to cover Listeria and group B streptococcus, in conjunction with an aminoglycoside
(e.g., gentamicin) or a third-generation cephalosporin (e.g., cefotaxime)
for expanded coverage of gram-negative organisms such as E. coli. Ceftriaxone should be avoided in newborns <1 month of age because it can
displace bound bilirubin.
The syndrome of afebrile pneumonitis described in infants 1 to 3
months old (staccato cough, tachypnea, with or without progressive respiratory distress and diffuse pulmonic infiltrates) may be viral in origin,
but has also been associated with atypical bacteria, so authors have suggested empiric treatment with erythromycin or clarithromycin in this
group.4 Note that azithromycin is not included in the treatment of patients this young, because it has not been approved by the U.S. Food
and Drug Administration.
For children >3 months of age, the choice of treatment remains largely
empiric. There are some notable differences between North American
and European recommendations,14 but both presume the most frequent
cause of bacterial pneumonia to be S. pneumoniae. For this reason, highdose amoxicillin (80 to 100 milligrams/kg/d PO) or another β-lactam
antibiotic remains the drug of choice. For children 5 years of age, North
American guidelines assume that atypical or intracellular agents such as
mycoplasma and C. pneumoniae begin to play a more important role, so
macrolides are often listed as the first choice. This presumes that macrolides will treat both atypical pathogens and pneumococci. High rates
of pneumococcal resistance to macrolides in vitro are a growing concern
in many areas of the world and may lead to future changes in these recommendations (see Controversies below). In cases where resistant
pathogens or multiple pathogens are suspected, all guidelines make
provisions for double coverage with β-lactams or cephalosporins plus
macrolides. With these facts in mind, typical choices for primary or secondary bacterial pneumonia are listed in Tables 121-3 and 121-4.
The ED physician occasionally encounters a child with significant underlying illness or fulminant viral pneumonia. In these cases, special
treatments may be indicated, and consultation is strongly advised. Specific cases and treatment suggestions are listed in Table 121-5.
As with all guidelines, these serve as a template and may need to be
adapted according to formulary, familiarity, and local patterns of antibiotic resistance. For children with allergies, unusual exposures, or specific
risk factors, these choices may need to be individualized.
The choice of oral versus parenteral antibiotics depends on the clinical
picture. Oral antibiotics provide adequate coverage for most mild-to-
CHAPTER 121: Pneumonia in Infants and Children
817
TABLE 121-3 Bacterial Organisms and Empiric Treatment for Pneumonia in Otherwise Healthy Children
Age Group
Bacterial Pathogens
Hospitalized Patients
Outpatients
Newborn
Group B streptococci
Gram-negative bacilli
Listeria monocytogenes
Streptococcus pneumoniae
Chlamydia trachomatis
Haemophilus influenzae
Bordetella pertussis
Staphylococcus aureus
Ampicillin
plus
Gentamicin or cefotaxime
Afebrile pneumonitis
Erythromycin or clarithromycin
Febrile pneumonia:
Cefuroxime
± erythromycin IV or clarithromycin PO
CCU/severe: choose one of
Cefuroxime + erythromycin or clarithromycin
Cefotaxime + erythromycin
Cloxacillin† + clarithromycin
Ampicillin IV or cefuroxime IV
or amoxicillin if PO
or amoxicillin-clavulanate if PO
CCU/moderate to severe
Add erythromycin or clarithromycin
Initial outpatient management not recommended
Ampicillin IV
plus
Erythromycin or clarithromycin
Alternative
Cefuroxime
or amoxicillin-clavulanate
or erythromycin
or clarithromycin
CCU/moderate to severe:
Cefuroxime + erythromycin or clarithromycin
Erythromycin or clarithromycin#
or amoxicillin ± clavulanate
or cefuroxime axetil
1–3 mo
3 mo–5 y*
S. pneumoniae
S. aureus
H. influenzae type b‡
Nontypeable H. influenzae
C. trachomatis
Mycoplasma pneumoniae
M. pneumoniae
S. pneumoniae
C. pneumoniae
H. influenzae type b‡
S. aureus
5–18 y
Initial outpatient management not recommended
Amoxicillin
or amoxicillin-clavulanate
or cefuroxime axetil
Abbreviation: CCU = critical care unit.
*The majority of pneumonias in this age group are viral, not bacterial.
†Cloxacillin
suggested if methicillin-sensitive S. aureus is suspected.
‡Uncommon
where vaccination against H. influenzae type b is universal.
#Azithromycin
can be substituted for clarithromycin with equal safety and efficacy for treating M. pneumoniae.
Adapted with permission from Lau E (ed): Drug Handbook and Formulary 2008–2009. Toronto, The Hospital for Sick Children, 2008.
moderate cases of pneumonia. Parenteral therapy is usually limited to
neonates and severe pneumonia requiring hospitalization.4 Even for
children who are admitted, some research has shown that oral treatment
is still sufficient within the monitored setting of the hospital.41
The recommended duration of outpatient treatment is typically 7 to
10 days (5 days in cases in which azithromycin is used). This is based on
historical precedent, although some literature suggests equivalent outcomes in mild pneumonia with as few as 3 to 5 days of outpatient treatment.39,40 There are theoretical concerns, however, that shorter courses
of treatment and poor adherence may drive bacterial resistance.
In pediatrics the choice of antibiotic may also be influenced by nonmicrobial factors, such as taste, frequency of administration, availability
of a liquid formulation, the child’s ability to swallow, and cost. The simplest, most-palatable first-line antibiotic should be picked, and broadspectrum or second-line choices reserved for appropriate cases.
■ CONTROVERSIES OF TREATMENT
The use of macrolides (erythromycin, clarithromycin, azithromycin) as
first-line agents in young children is an area of controversy.5,42 These antibiotics are generally effective against atypical and intracellular agents,
which are more common in children after the age of 5, but they may be
ineffective against common S. pneumoniae. Pneumococcal resistance to
macrolides in Europe and North America ranges from 7.5% to
>50.0%.14 The indiscriminate use of azithromycin in treating upper respiratory tract infections appears to be driving streptococcal resistance in
some populations.43 The liquid suspension of azithromycin is not Food
and Drug Administration approved for children <6 months of age. Some
researchers have suggested that children who have fever of <2 days’ duration and who are <3 years of age have a very low risk of communityacquired mycoplasma infection and that macrolides can be safely excluded as first-line antibiotics.44 If a preschool child fails to improve
when taking amoxicillin or an equivalent β-lactam, the addition or substitution of a macrolide may be indicated.
Pneumococcal resistance to penicillin itself (and other β-lactams) is another area of concern around the globe. To date, most antibiotic resistance
in community-acquired streptococci has been documented in vitro, rather
than in vivo, and clinical response is still satisfactory when clinicians use
an increased dosage, as noted in Table 121-4.45 The safety and efficacy of
fluoroquinolones for treatment of respiratory infections in children is not
established, and their use is generally restricted due to the theoretical risks
of arthropathy. A novel class of antibiotics called ketolides has shown activity against both pneumococci and several atypical pathogens, but these
818
SECTION 12: Pediatrics
TABLE 121-4 Antibiotic Dosages for Bacterial Pneumonia
TABLE 121-5 Special Cases
Antibiotic
Condition
Drug
Varicella pneumonia
Respiratory syncytial virus pneumonia
Interstitial pneumonia in a patient with human
immunodeficiency virus infection
Cytomegalovirus pneumonia
Gram-negative pneumonia
Pneumonia in a patient with sickle cell disease
Acyclovir
Ribavirin, if high-risk patient
Trimethoprim-sulfamethoxazole
± prednisone*
Ganciclovir and γ-globulin
Ceftazidime
Cefotaxime ± macrolide
± vancomycin (in severely ill)
Piperacillin + tobramycin
or ceftazidime + tobramycin
Clindamycin or vancomycin
(obtain consultation)
Dosage*
Amoxicillin
Ampicillin
Azithromycin
Cefotaxime
80–100 milligrams/kg daily in three divided doses PO
200 milligrams/kg daily in four divided doses IV, IM
10 milligrams/kg daily ×1, then 5 milligrams/kg daily ×4 PO
100 milligrams/kg daily in divided doses IV
<1 mo: 150 milligrams/kg daily in three divided doses IV, IM
Ceftazidime
100 milligrams/kg daily in three divided doses IV, IM
Cefuroxime-axetil 100 milligrams/kg daily in three divided doses IV, IM
suspension
30 milligrams/kg daily in two divided doses PO
Clarithromycin
15 milligrams/kg daily in two divided doses PO
†
Doxycycline
2–4 milligrams/kg daily in two divided doses PO
Erythromycin
30–50 milligrams/kg daily in four divided doses PO, IV
Gentamicin
7.5 milligrams/kg daily in one dose IV, IM
Vancomycin‡
40–60 milligrams/kg daily in four divided doses IV
*Weight-based amounts should not exceed maximum adult doses.
†Do
not use doxycycline in children <8 y to avoid staining adult teeth.
‡Consult
infectious disease or pediatric specialist for severely ill children infected with methicillin-resistant Staphylococcus aureus.
newer agents are expensive and not yet approved for use in children.5
Doxycycline, a much older and less expensive agent, has activity against
atypical pathogens as well as streptococci, but clinicians should review its
indications and side effects before prescribing. It is generally restricted to
use in adolescents and adults, because use in young children may cause
permanent staining of the adult teeth. As resistance to amoxicillin, macrolides, and other antibiotics grows, the importance of prevention using
pneumococcal conjugate vaccines is likely to increase.
DISPOSITION AND FOLLOW-UP
The decision to admit a patient or consult with specialist physicians is
dependent on the overall appearance of the child and the findings of the
history, physical examination, and ancillary investigations.
■ INDICATIONS FOR CONSULTATION
Reasons for consultation include the following:
1. Age of birth to 3 months
2. History of severe or relevant congenital disorders
3. Immune suppression (HIV infection, sickle cell disease, malignancy)
4. Toxic appearance, dehydration, lethargy, signs of sepsis
5. Unremitting respiratory distress or hypoxia
6. Radiographic anomaly (e.g., congenital anomaly)
7. Pneumonia with complications (e.g., effusion, empyema, pneumothorax)
■ ADMISSION CRITERIA
Clinical indications for admission are similar to those for consultation
and include age <3 months, a toxic appearance, altered level of consciousness, complicated pneumonia, frank hypoxia, or unrelieved respiratory distress. There is no evidence-based definition of hypoxia, but
physicians often choose a pulse oximetry threshold of <90% to 93%
for admission. Signs of severe respiratory distress include a breathing
rate of >70 breaths/min in infants or >50 breaths/min in older children.
In infants, intermittent apnea, grunting, or an inability to feed may be
surrogate markers of dyspnea. Those too ill to take fluids by mouth may
become dehydrated, and admission for IV fluid replacement is appropriate. Young children and adolescents with underlying illnesses that predispose them to severe or atypical infections may also require inpatient
Pneumonia in a patient with cystic fibrosis†
Methicillin-resistant Staphylococcus aureus
pneumonia
*Empiric treatment for Pneumocystis infection. Early use of steroids reduces mortality.23
†Where
possible, base selection on patient’s most recent culture and sensitivity reports. Consult infectious disease specialist.
Reproduced with permission from Zar H, Madhi S: Childhood pneumonia—progress and
challenges. S Af Med J 96(9): 890, 2006.
treatment. These illnesses include severe cardiac or renal dysfunction,
cystic fibrosis, sickle cell disease, and immune suppression from HIV infection, malignancy, or active chemotherapy.
Children with complications of pneumonia may require admission for
advanced diagnostics and treatment. The finding of a pleural effusion or
pneumatocele, or findings suggestive of a bacterial infection in a child <1
year of age, suggest a pathogen other than S. pneumoniae (in particular H.
influenzae type b or S. aureus). Because such infections can progress rapidly
and are not well tolerated, hospitalization is generally required. Infants with
suspected B. pertussis infection (whooping cough) are at risk for apnea and
should also be admitted and placed in respiratory isolation. Indications for
hospitalizing patients with RSV pneumonia are the same as those for patients with RSV bronchiolitis (see Chapter 120, Wheezing in Infants and
Children). Patients of any age with suspected active pulmonary TB should
be admitted under respiratory isolation. Patients for whom outpatient antibiotic therapy has failed may need admission for more intensive treatment.
Social indications for admission include the inability of parents or
caregivers to afford, understand, or ensure outpatient treatment.3 If
there is a risk that adults cannot bring a child back for follow-up with a
health care provider in 24 to 48 hours, admission should be considered.
■ COMPLICATIONS
Most viral pneumonias resolve spontaneously without specific therapy.
Complications are similar to those for bronchiolitis and include dehydration, bronchiolitis obliterans, and apnea. Apnea is commonly seen in
very young infants with RSV, Chlamydia, or B. pertussis infection. Pleural effusions can occur with viral pneumonias but are not common.
Uncomplicated bacterial pneumonia usually responds rapidly to antibiotic therapy. A delay in improvement or a worsening condition after
therapy has begun should prompt an evaluation for possible complications. These include pleural effusion, empyema, pneumothorax, pneumatocele, dehydration, and development of additional infectious foci.
Effusions do occur in a small number of pneumococcal and mycoplasmal pneumonias, and a larger percentage of the now-rare Hib infections.
S. aureus pneumonias have a high rate of complications, including empyemas and pneumatoceles. Mycoplasma pneumonias are associated
with several extrapulmonary complications, including arthritis and
meningitis, whereas local and hematogenous spread of TB can result in
myriad manifestations both inside and outside of the lung.
Patients infected with antibiotic-resistant bacteria or unusual opportunistic pathogens may fail to improve with standard antimicrobial treat-
CHAPTER 122A: Pediatric Heart Disease: Congenital Heart Defects
ment. Whenever pneumonia is complicated or prolonged, clinicians
should consider further investigation and consultation. In these uncommon cases, radiographic follow-up is recommended to ensure complete
radiographic resolution, which may take 4 to 6 weeks or longer.
■ DISCHARGE INSTRUCTIONS
All children and families discharged from the ED with a diagnosis of
pneumonia should receive specific advice on the dosage and scheduling
of medications and the signs of worsening respiratory distress. Children
who cannot take prescribed antibiotics or adequate amounts of fluid
should return for further care. All children discharged with the diagnosis
of pneumonia should be scheduled for follow-up within 48 hours with a
primary care provider. Younger children require closer follow-up.
PRACTICE GUIDELINES AND SOCIETY
POSITION STATEMENTS
■ PREVENTION
Many factors are known to predispose children to pneumonia. Even in
the acute setting, physicians may want to quickly review the following
topics with parents and caregivers:
1. Hand washing and general hygiene to prevent transmission
2. Breastfeeding of infants, which is known to be protective
3. Avoidance of smoking (by teens) and second-hand smoke
4. Vaccination, including pneumococcal conjugate vaccine (Prevnar)
and polysaccharide vaccine (Pneumovax) in at-risk children
■ IMMUNIZATION
Annual immunization against influenza is now recommended for children ≥6 months of age and for those at high risk due to underlying health
conditions. The vaccine must be given annually to account for seasonal
antigenic changes. Priority should be given to patients with asthma, cystic fibrosis, and other pulmonary diseases; those with significant cardiac,
renal, and immune disorders; and those with diabetes. Caregivers and
health care providers should also be immunized to avoid transmission to
these at-risk children.46
Around the globe, immunization against polio, pertussis, measles, and
Hib infection has significantly lowered the risk of pneumonias and respiratory failure associated with these diseases. In some cases, simple herd
immunity has decreased the chances that a child will come into contact
with these once-feared diseases, but sporadic outbreaks exist in nonimmunized populations.
Immunization against varicella (chickenpox) should protect against the
secondary pneumonias associated with varicella-zoster virus infection, although use of the vaccine is not yet universal. Similarly, the administration
of bacille Calmette-Guérin vaccine to provide partial protection against
certain forms of TB varies by state, province, and country.
The introduction of a seven-strain pneumococcal conjugate vaccine
(Prevnar) in children <2 years of age has led to dramatic decreases in invasive disease and modest but promising trends in the reduction of pneumococcal pneumonias, especially in children <2 years.47–49 Early reports
suggest a somewhat diminished impact in HIV-positive children.50 Serotypes not covered by the vaccine remain a possible source of infection,
and the role of serotype replacement in the population is not yet known.
Expanded-spectrum vaccines (9 and 13 serotypes) are under development. Immunization coverage is universal in Canada, but varies by state
and private insurance provider in the U.S.
The older 23-valent polysaccharide vaccine is effective in children ≥2
years of age, and the emergency physician should confirm that it has
been given to children with sickle cell disease, those who have undergone
splenectomy, and others at high-risk for pneumococcal disease. A booster may be required.
Acknowledgments: The author would like to thank Drs. Kathleen
Brown and Willie Gilford, Jr., for their work on the previous edition of
819
this chapter and Dr. Gordon Culham, Department of Radiology, BC
Children’s Hospital, Vancouver, British Columbia, and Dr. James McCormack of the BC Therapeutics Initiative for their input.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com
CHAPTER
122 A
Pediatric Heart Disease:
Congenital Heart Defects
Linton L. Yee
Garth D. Meckler
Congenital heart defects can present at different ages with clinical signs
and symptoms ranging from cyanosis to cardiovascular collapse or congestive heart failure (CHF) depending on the anatomy and physiology of
the lesion. Long-term survivors are at risk for a number of postoperative
complications.
This chapter examines congenital heart defects and begins with a review of fetal and neonatal cardiac physiology, followed by a discussion of
specific lesions and their diagnosis and management organized by clinical presentation. A brief discussion of pediatric murmurs follows, and
this chapter concludes with a discussion of common surgical procedures
for repairing congenital heart defects and associated complications.
Congenital heart disease is usually classified based on physiology (presence or absence of cyanosis, with or without persistent fetal circulation)
or on the nature of the anatomic defect (shunt, obstruction, transposition,
or complex defect). Most textbooks separate cyanotic from acyanotic lesions. Cyanotic lesions result in mixing of deoxygenated and oxygenated
blood or right-to-left shunting; cyanotic lesions include the “five Ts”: tetralogy of Fallot (TOF), tricuspid anomalies including tricuspid atresia
(TA) and Ebstein anomaly, truncus arteriosus, total anomalous pulmonary venous return (TAPVR), and transposition of the great arteries
(TGA). Acyanotic lesions include those that result in pulmonary overcirculation such as ventricular septal defect (VSD), atrial septal defect
(ASD), patent ductus arteriosus (PDA), and atrioventricular (AV) canal,
as well as those with restricted pulmonary or systemic blood flow such as
pulmonary stenosis, aortic stenosis, and aortic coarctation.
It is often more useful to organize congenital heart disease by clinical
presentation (Table 122A-1). Distinct clinical presentations are discussed further in later sections, including the pathophysiology, clinical
features and treatment, and individual defects within each group. Discussion of murmurs and arrhythmias included in this chapter is limited
to those related to congenital heart disease. Rhythm disturbances are discussed in greater detail in Chapter 122B, Pediatric Heart Disease: Acquired Heart Disease, and syncope and sudden death are discussed in
Chapter 140, Syncope and Sudden Death in Children.
PHYSIOLOGY
■ FETAL CIRCULATION
Fetal circulation involves a number of shunts to bypass the liquid-filled
lungs, which are incapable of providing oxygen to circulating blood.
Blood oxygenated by the maternal lungs passes through the placenta via
the umbilical vein to the fetus. Roughly half of blood flow passes through
the liver and half through the ductus venosus to the inferior vena cava
(IVC), where it mixes with deoxygenated fetal blood returning from the
lower body. The IVC enters the right atrium, where deoxygenated blood
returning from the upper body and head via the superior vena cava mixes with that from the IVC. Blood from the right atrium travels in one of
three routes: a portion passes through the foramen ovale into the left
820
SECTION 12: Pediatrics
TABLE 122A-1 Clinical Presentations of Congenital Heart Disease
Clinical
Presentation
Causative Conditions
in Neonates
Cyanosis
Transposition of the great arteries, TOF, tricuspid atresia, truncus arteriosus, total anomalous
pulmonary venous return
Cardiovascular
Critical AS, coarctation of the
shock
aorta, HLHS
Congestive heart Rare: PDA, HLHS
failure
Murmur
PDA, valvular defects (AS, PS)
Syncope
Hypertension
Arrhythmias
—
—
—
Causative Conditions in
Infants and Children
TOF, Eisenmenger complex
lation, which can be caused by structural heart disease or noncardiac disease, including meconium aspiration, pneumonia, sepsis, and pulmonary
hypertension. Lesions that restrict pulmonary blood flow, such as critical
pulmonary stenosis, do not typically cause cyanosis without the presence
of associated defects (ASD, VSD) that allow for right-to-left shunting.
■ PATHOPHYSIOLOGY
Coarctation of the aorta
(infants)
PDA, VSD, ASD, atrioventricular canal
VSD, ASD, PDA, outflow
obstructions, valvular defects
(AS, PS)
AS, PS, Eisenmenger complex
Coarctation of the aorta
ASD, Ebstein anomaly, postsurgical complication after
repair of congenital heart
defect
Abbreviations: AS = aortic stenosis; ASD = atrial septal defect; HLHS = hypoplastic left heart
syndrome; PDA = patent ductus arteriosus; PS = pulmonic stenosis; TOF = tetralogy of Fallot; VSD = ventricular septal defect.
atrium, where it subsequently travels to the left ventricle, through the
aorta, and into the vessels supplying the fetal head and upper extremities;
most of the remainder enters the right ventricle and passes into the pulmonary artery, where the majority is shunted away from the lungs
through the ductus arteriosus connecting the pulmonary artery with the
aorta; and a small amount of blood travels to the lungs to provide oxygen
and nutrients to support fetal lung growth.
Transitional Circulation The newborn’s lungs expand and become air
filled with gradual reabsorption of fetal lung fluid. This increases the PaO2
of blood flowing through the newborn lung, which, in turn, mediates a
cascade of events that completes the transition to adult circulatory patterns. Flow through the umbilical arteries ceases, and the venous flow
through the cord slows and then stops. Pulmonary vascular resistance
falls, and pulmonary blood flow increases (pulmonary vascular resistance
continues to fall with increases in blood flow over the first 30 to 45 days of
extrauterine life). The ductus venosus and ductus arteriosus close, and decreased pulmonary arterial resistance coupled with increased systemic resistance create increased blood flow through the atria. Left atrial pressure
exceeds right atrial pressure, which leads to closure of the foramen ovale.1,2
Neonatal Cardiac Physiology Because neonates and small children have
relatively noncompliant ventricular walls, they cannot increase stroke
volume but rely on changes in heart rate to adjust cardiac output. Thus,
sinus tachycardia is usually the first response to stress in infants and young
children. The neonatal myocardium requires more oxygen than the infant’s or child’s heart and has a lower systolic reserve, which predisposes to
CHF. Although the ductus arteriosus and foramen ovale are usually functionally closed by 15 hours of life and 3 months of age, respectively, shunting may still occur through these pathways during times of stress.3 Finally,
the neonatal right ventricle is still predominant, whereas the left ventricle
is predominant in older infants and children, and pulmonary vascular resistance is relatively high and oxygen responsive.
CYANOSIS
Congenital heart defects that present with cyanosis include TGA,
TOF, TA, truncus arteriosus, and TAPVR. These lesions have in common the mixing of oxygenated and deoxygenated blood, circulation of
desaturated hemoglobin, and a cardinal manifestation as cyanotic heart
disease. Another condition resulting in cyanosis is persistent fetal circu-
Cyanosis is the bluish discoloration of the skin that occurs from the presence of deoxygenated hemoglobin (which is blue) in capillary beds. For
cyanosis to be clinically apparent, 3 to 5 milligrams/dL of deoxyhemoglobin must be present, corresponding to an oxygen saturation of 70% to
80% on room air.4–6 Compression of the placenta during birth typically
leads to polycythemia in term newborns, and, as a result, clinical cyanosis develops more readily in newborns because a smaller percentage of
circulating hemoglobin must be desaturated to manifest this sign.
Congenital heart defects can lead to cyanosis in the first weeks of life
or, for some lesions, episodically throughout childhood if uncorrected.
Lesions such as TGA are associated with mixing of oxygenated and
deoxygenated blood, usually through an associated VSD or ASD, and
produce cyanosis in the period immediately after birth. Conditions associated with persistent pulmonary hypertension allow blood to shunt
right to left through a patent foramen ovale or through a septal defect.
TOF (see the section Tetralogy of Fallot below) can produce cyanosis at
birth through mixing, but is also associated with episodic cyanosis (“tet
spells,” see the section Tet Spells below) throughout infancy and childhood if uncorrected. Large uncorrected septal defects (ASD, VSD) can
cause cyanosis in adolescents and young adults in a condition termed
Eisenmenger complex. Chronic left-to-right shunting across a nonrestrictive defect leads to hypertrophy of pulmonary arteriolar musculature that causes a gradual and irreversible rise in pulmonary vascular
resistance and right-sided heart pressures until supersystemic pressures
develop and shunting switches to right to left, which produces cyanosis.
■ DIFFERENTIAL DIAGNOSIS
Cyanosis is a physical sign with a spectrum of potential causes. As mentioned above in Pathophysiology, polycythemia, common in neonates,
makes the appearance of cyanosis relatively common. Noncardiac causes
range from benign peripheral vasoconstriction in response to cold or
crying, causing peripheral cyanosis, to sepsis with poor perfusion or even
effects of toxins such as methemoglobin.7 The most common congenital
heart defects that must be considered in the cyanotic neonate are briefly
reviewed in the sections below before return to a general approach to
evaluation and management of cyanotic congenital heart disease.
Tetralogy of Fallot TOF is the most common cyanotic congenital heart
disease manifesting in the postinfancy period and comprises as much as
10% of all congenital heart disease.8–10 There are four primary components of TOF: a large VSD, right ventricular outflow obstruction (created by valvular or supravalvular pulmonic stenosis), an overriding aorta,
and right ventricular hypertrophy.
The intensity of cyanosis depends on the amount of obstruction of the
right ventricular outflow tract. A nonrestrictive VSD balances the systolic pressures in the right and left ventricles. The amount of right ventricular outflow obstruction determines whether shunting is left to right,
bidirectional, or right to left. Severe pulmonic stenosis creates a right-toleft shunt resulting in cyanosis and decreased pulmonary blood flow.
The acyanotic form of TOF is characterized by mild pulmonic stenosis
with a left-to-right shunt. In addition to cyanosis, examination findings
can include a systolic thrill at the lower and middle left sternal border. A
loud single S2, an aortic ejection click, a loud systolic ejection murmur
(heard best at the middle to lower left sternal border), and a continuous
PDA murmur may also be apparent on examination.
Transposition of the Great Arteries Comprising about 5% to 8% of all
congenital heart disease, TGA is the most common cyanotic heart lesion
manifesting in the newborn period.10 There are many variations in TGA,
but the underlying elements are that the aorta arises from the right ventricle and the main pulmonary artery originates from the left ventricle.
CHAPTER 122A: Pediatric Heart Disease: Congenital Heart Defects
This arrangement gives rise to two distinct circulatory systems. Because
the main pulmonary artery has a higher oxygen saturation than the aorta, hyperoxemic blood goes through the pulmonary system and hypoxic
blood flows through the systemic system. Mixing of the two circulatory
systems is the only manner in which oxygenated blood enters the systemic blood flow. A VSD, ASD, or PDA must exist in order for the infant
to survive. In 20% to 40% of patients a VSD is present. The physical examination is notable for a loud and single S2, and if a VSD exists, a systolic murmur may be heard.
Total Anomalous Pulmonary Venous Return TAPVR represents 1% of
congenital heart disease.11 The pulmonary veins empty into the right atrium instead of returning blood from the lungs into the left atrium. TAPVR
is usually separated into four groups depending on where the pulmonary
veins empty. In the supracardiac type (50% of all TAPVR), the common
pulmonary vein is attached to the superior vena cava. In the cardiac type
(20%), the common pulmonary vein drains into the coronary sinus. In
the infracardiac/subdiaphragmatic type (20%), the common pulmonary
vein empties into the portal vein, ductus venosus, hepatic vein, or IVC.
Mixed lesions comprise the remaining 10%. Survival depends on the mixing of blood, so an ASD or a patent foramen ovale must be present.
When pulmonary venous return arrives in the right atrium, there is
mixing of the pulmonary and systemic circulations. In the right atrium,
blood crosses the ASD to the left atrium or crosses the tricuspid valve to
the right ventricle. Systemic arterial blood becomes desaturated because
of the mixing of pulmonary and systemic arterial flow. Pulmonary blood
flow determines the degree of desaturation of systemic arterial blood. If
there is no obstruction to pulmonary venous return, systemic blood is
minimally desaturated. Obstruction to pulmonary venous return results
in severe cyanosis. Because of the extra volume returning to the right side
of the heart, right ventricular and atrial enlargement can develop.
Although TAPVR more commonly presents with signs and symptoms
of CHF (see Congestive Heart Failure below), tachypnea, tachycardia,
hepatomegaly, and cyanosis are commonly seen. Children with pulmonary venous obstruction often have a history of frequent pneumonias
and growth retardation. The physical examination reveals a right ventricular heave and fixed split S2. A grade 2/6 to 3/6 systolic ejection murmur heard at the left upper sternal border and a mid diastolic rumble at
the left lower sternal border are also heard. TAPVR with pulmonary
venous obstruction leads to respiratory distress and cyanosis with a loud
and single S2 and a gallop but, on most occasions, no murmur.
Tricuspid Atresia TA represents 1% to 2% of congenital heart disease.12
There is no tricuspid valve, and the development of the right ventricle
and pulmonary artery is interrupted. Pulmonary blood flow is decreased.
With no flow existing between the right atrium and right ventricle, an
ASD, VSD, or PDA is required for survival, because the right atrium
needs a right-to-left shunt in order to empty. The great arteries are transposed with a VSD and pulmonic stenosis in 30% of cases. Artery anatomy is normal with a small VSD and pulmonic stenosis in half of cases.
With all of the systemic venous return shunted from the right atrium
to the left atrium, right atrial dilatation and hypertrophy occur. Increased volume from the systemic and pulmonary circulations causes
enlargement of the left atrium and left ventricle. The extent of cyanosis
and the amount of pulmonary blood flow are inversely related.
Usually patients have marked cyanosis, tachypnea, and poor feeding.
A single S2 is evident as well as a grade 2/6 or 3/6 regurgitant systolic
murmur heard best at the left lower sternal border. The continuous murmur of a PDA may also exist. Hepatomegaly is present if there is CHF.
Truncus Arteriosus In truncus arteriosus all of the pulmonary, systemic,
and coronary circulations originate from a single arterial trunk. The defect comprises <1% of all congenital heart disease.13 Associated with
truncus arteriosus are a large VSD, coronary artery irregularities, and
DiGeorge syndrome (hypocalcemia, hypoparathyroidism, absent or hypoplastic thymus, chromosomal abnormalities). Pulmonary blood flow
is determined by the type of truncus, and flow can be normal, increased,
or decreased. There is a direct relationship between the amount of pulmonary blood flow and systemic arterial oxygen saturation. Decreased
821
pulmonary blood flow creates marked cyanosis, whereas increased pulmonary blood flow produces minimal cyanosis but is associated with
CHF from left ventricular volume overload.
CHF and cyanosis typically develop within the first few weeks of life.
A loud regurgitant 2/6 to 4/6 systolic murmur at the left sternal border
may be accompanied by a high-pitched diastolic decrescendo murmur
or diastolic rumble. A single S2 is prominent.
■ EVALUATION
History The cardinal clinical presentation of cyanotic congenital heart
defects is cyanosis. The history taking should elicit details of the pregnancy, gestational age, fetal US results if applicable, and complications of
labor and delivery, including cyanosis in the period immediately after
birth. For older infants and children with known congenital heart defects, details of the anatomy and surgical procedures and current medications should be obtained. Baseline oxygen saturations may be known
by caretakers and are helpful when intercurrent illness leads to an ED
visit. A careful feeding history should be obtained focusing on changes
in oral intake, slow or difficult feeding, sweating with feeds, and growth,
and a complete review of systems should be performed.
Physical Examination Measure all vital signs, including blood pressure in
the upper and lower extremities. A difference in upper and lower extremity blood pressures may signal an obstructive lesion such as coarctation of
the aorta (see Coarctation of the Aorta below). Document weight and
growth parameters. Note if cyanosis is central (mucosal) or acral (involving digits) (Figure 122A-1). Listen for cardiac murmurs, noting location,
timing, and loudness (see Pediatric Murmurs below), and a gallop or
FIGURE 122A-1. Cyanosis of the mucous membranes (A) and nail beds (B).
[Reproduced with permission from Shah BR, Lucchesi M (eds): Atlas of Pediatric
Emergency Medicine. New York, NY, McGraw-Hill, 2006.]
822
SECTION 12: Pediatrics
fixed splitting of S2 (characteristic of ASD). Palpate the chest for heaves,
lifts, and thrills and note surgical scars. Observations of the strength,
quality, and symmetry of pulses help in the assessment of cardiac output.
Hepatomegaly and splenomegaly suggest right-sided heart failure. Observe for signs of increased work of breathing and auscultate for rales,
which suggest CHF. Neonates with cyanosis secondary to congenital
heart disease rarely have respiratory symptoms other than tachypnea.
Neonates with lung disease producing cyanosis show respiratory distress,
grunting, tachypnea, and retractions. Cyanotic infants with central nervous system disturbances or sepsis have apnea, bradycardia, lethargy, and
seizures. Neonates with methemoglobinemia show minimal distress despite their cyanotic appearance. The neurologic examination includes observation and examination of muscle tone and mental status—irritability
may be a symptom of hypoxemia. Performing a complete head-to-toe examination is important in the cyanotic patient without a known history
of congenital heart defects to exclude noncardiac causes.
Diagnostic Tests Laboratory tests are not typically helpful in the evaluation of cyanotic congenital heart defects, although they help exclude other causes of cyanosis. Results of the “hyperoxia test” (PaO2 in response to
breathing 100% oxygen) may help distinguish heart disease from other
causes of cyanosis. Neonates with cyanotic heart disease do not demonstrate an increase in PaO2 >20 mm Hg, because of the right-to-left
shunting of the circulation. Most neonates with lung disease or sepsis,
however, demonstrate an increase in PaO2 after breathing 100% oxygen
for 20 minutes. Infants with persistent pulmonary hypertension may or
may not demonstrate a significant rise in PaO2. There is no response to
oxygen in the neonate with methemoglobinemia. When a blood specimen is exposed to air, it turns pink in all the conditions described
above except in methemoglobinemia, in which the blood remains
chocolate colored. In an infant without known congenital heart defects,
results of arterial blood gas analysis with the infant breathing room air
and 100% oxygen can be compared: failure of the PaO2 to rise significantly with 100% oxygen suggests cardiac mixing or right-to-left shunting,
whereas improvement in the PaO2 in response to oxygen suggests a pulmonary cause.
The primary diagnostic tests for the patient with suspected cyanotic
congenital heart defects are chest radiography and ECG (Table 122A-2).
Chest radiographic studies are essential in assessing the size and shape of
the heart, and in evaluating pulmonary blood flow. The chest radiograph
also provides some information about the position of the aortic arch,
which should be normally left-sided. In the normal left-sided aortic arch,
there is rightward displacement of the esophagus and trachea. An abnormal position of the aortic arch may be a clue to the diagnosis of the congenital cardiac lesion. Right-sided aortic arches are seen in truncus
TABLE 122A-2 Cyanotic Congenital Cardiac Lesions: Typical
Chest Radiograph and ECG Findings
Cardiac Lesion
Chest Radiograph
Tetralogy of
Fallot
Boot-shaped heart, normalsized heart, decreased pulmonary vascular markings
Transposition of Egg-shaped heart, narrow
the great arteries mediastinum, increased pulmonary vascular marking
Total anomalous Snowman sign, significant carpulmonary
diomegaly, increased pulmovenous return
nary vascular markings
Tricuspid atresia Heart of normal to slightly
increased size, decreased pulmonary vascular markings
Truncus
arteriosus
Cardiomegaly, increased pulmonary vascular markings
ECG
Right axis deviation, right
ventricular hypertrophy
Right axis deviation, right
ventricular hypertrophy
Right axis deviation, right
ventricular hypertrophy, right
atrial enlargement
Superior QRS axis with right
atrial hypertrophy, left atrial
hypertrophy, left ventricular
hypertrophy
Biventricular hypertrophy
FIGURE 122A-2. Chest radiograph revealing the classic “boot-shaped heart” of
tetralogy of Fallot. [Reproduced with permission from Shah BR, Lucchesi M (eds):
Atlas of Pediatric Emergency Medicine. New York, NY, McGraw-Hill, 2006.]
arteriosus, TGA, TOF, TA, and TAPVR. The chest radiograph is critical
to the assessment of pulmonary vascularity. With small left-to-right
shunts, the pulmonary vascularity is normal. Pulmonary vascularity can
also be normal in conditions that cause pulmonary stenosis, such as valvular pulmonic stenosis or functional pulmonic stenosis associated with
TOF. Increased pulmonary vascularity may be seen with any cause of
left-to-right shunting or in any cause of left-sided failure, such as outflow
obstruction.
The ECG is useful to evaluate chamber size, electrical axis, and cardiac
conduction. Age-related normal values are discussed in Chapter 143D,
Pediatric Procedures: Electrocardiogram Interpretation, and should be
used as a reference to determine axis deviation, atrial enlargement, or
ventricular hypertrophy.14 The electrical axis most often defines abnormal chamber diameters and usually does not suggest cardiac ischemia as
in the adult population. Table 122A-2 lists characteristic chest radiograph and ECG findings of cyanotic congenital heart defects. Figure
122A-2 depicts the typical “boot-shaped heart” of TOF.
When available, bedside echocardiography may delineate structural
heart disease, although adequate imaging depends on the availability of
an ultrasonographer with pediatric cardiac experience.
■ TREATMENT
The management of cyanotic congenital heart defects depends on the age
of the patient, hemodynamic stability, and prior diagnosis and medical
management. Most cyanotic congenital heart defects are hemodynamically stable. With obstructive lesions, in contrast, adequate circulation often
depends on systemic or pulmonary blood flow through a PDA, and such
lesions can be fatal if patency is not maintained with prostaglandins (see
Shock below). Although central cyanosis and low oxygen saturation are
alarming and may tempt one to administer oxygen immediately, neonates
have significant amounts of oxygen-avid fetal hemoglobin and tolerate
oxygen saturation percentages in the 70s (characteristically seen in
most mixing lesions) without tissue or brain hypoxemia. Moreover, oxygen is a potent pulmonary vasodilator. Oxygen administration and pulmonary vasodilation are helpful in treating cyanotic congenital heart
defects associated with pulmonary hypertension or vasoconstriction,
but may actually lead to pulmonary vascular overcirculation or even
“steal” of systemic blood flow in patients with a PDA and ductal-dependent systemic blood flow. Oxygen administration should be reserved
for patients with signs and symptoms of inadequate tissue perfusion,
those without known heart disease in whom it may be diagnostic as well
as therapeutic, and patients with known congenital heart defects with
oxygen saturation significantly below known baseline values.
The primary management objective in the cyanotic neonate or infant is
the treatment of intercurrent illness, exclusion of noncardiac causes of cy-
CHAPTER 122A: Pediatric Heart Disease: Congenital Heart Defects
anosis, and diagnosis of cyanotic congenital heart defects in those who do
not have a previous diagnosis. Treatment thereafter involves consultation
with a pediatric cardiologist and transfer to a tertiary pediatric hospital or
clinic.
Tet Spells A tet spell is caused by right-sided outflow tract obstruction
leading to right-to-left shunting through a VSD. Hypoxia and acidosis cause pulmonary arterial vasoconstriction, increasing pulmonary
resistance and exacerbating shunting. The management goals for tet
spells are to increase pulmonary blood flow by increasing preload, provide pulmonary vasodilation, and increase afterload in order to reverse
right-to-left shunting and promote pulmonary blood flow. This is often
achieved through simple maneuvers such as administering 100% oxygen via a non-rebreathing face mask, calming the child by minimizing
stimulation and placing the child in a parent’s arms, and flexing the
child’s knees to the chest in order to increase venous return to the
heart and increase systemic vascular resistance to mitigate right-toleft shunting. Second-line intervention includes administration of morphine, 0.1 to 0.2 milligram/kg IM, SC, or IV and isotonic fluid [normal
saline (NS) 20 mL/kg bolus] to increase preload. If these measures are
unsuccessful, next options include administration of sodium bicarbonate 2 mEq/kg as an IV bolus to treat acidosis and promote pulmonary
vasodilation; propranolol, 0.2 milligram/kg IV to relieve infundibular
spasm; or phenylephrine, 2 to 10 micrograms/kg/min to increase systemic vascular resistance. Refractory spells may require neuromuscular
blockade and rapid-sequence intubation.
SHOCK
The presentation of congenital heart defects as shock or cardiovascular
collapse is dramatic. Noncardiac causes of shock and low cardiac output
states should be considered, and the patient should be treated while contemplating the possibility of congenital heart disease. Once the ductus
arteriosus begins to close, some cardiac lesions become incompatible
with life, because blood can no longer reach the lungs or distal circulation. These infants present in shock within the first 2 weeks of life. Both
cyanotic and acyanotic lesions may present in this fashion. Acyanotic lesions include severe coarctation of the aorta, critical aortic stenosis,
and hypoplastic left ventricle. TGA, pulmonary atresia, and hypoplastic right heart syndrome are examples of the cyanotic lesions that
may present with shock.
■ PATHOPHYSIOLOGY
Congenital heart defects that present as shock or cardiovascular collapse
include lesions that depend on flow through the ductus arteriosus to provide systemic or pulmonary perfusion. The classic examples are severe
coarctation of the aorta and hypoplastic left heart syndrome. In both of
these conditions, systemic blood flow is restricted by the underlying defect but maintained by flow through a PDA that bypasses the coarctation
in the former and allows blood pumped by the functional right ventricle
to perfuse the aorta in the latter. The ductus typically closes and becomes
the ligamentum arteriosum by the second or third week of life, and as it
constricts, patients with duct-dependent flow manifest poor peripheral
perfusion with increasing acidosis and eventual cardiovascular collapse.
■ DIFFERENTIAL DIAGNOSIS
Cardiogenic shock can be the final common pathway for a wide variety
of disease processes, both noncardiac and cardiac. Sepsis, hypovolemic
shock, metabolic disease, adrenal insufficiency, respiratory failure, trauma, and poisonings can all lead to cardiogenic shock and are discussed
elsewhere. Nonstructural cardiac causes of shock include dysfunctional
myocardium, which may mimic the signs and symptoms seen with
shunt-dependent anatomic lesions. Such cardiomyopathies are uncommon in pediatric patients, but can easily be confused with anatomic lesions. Cardiomyopathies in children are discussed in Chapter 122B,
Pediatric Heart Disease: Acquired Heart Disease. Duct-dependent acyanotic congenital lesions are briefly reviewed in the following sections.
823
Coarctation of the Aorta A PDA delays the obstructive effects of coarctation by allowing blood to flow distal to the obstruction. With closure
of the PDA, pulmonary hypertension occurs, leading to pulmonary
venous congestion and CHF. Blood flow distal to the aortic obstruction
is compromised. Shock, metabolic acidosis, tachypnea, and feeding difficulty are common; when CHF occurs, a loud gallop and weak pulses
with or without a murmur can usually be appreciated.
Finding decreased pulses in the lower extremities is essential to diagnosing a coarctation. Comparing right upper extremity blood pressures
and pulse oximeter readings with those of the lower extremity aids in diagnosis unless the patient is in shock, in which case pulses may be decreased all over.
Hypoplastic Left Heart Syndrome Hypoplastic left heart syndrome consists of hypoplasia of the left ventricle and ascending aorta and aortic arch.
Atresia or marked stenosis of the mitral and aortic valves and regressed development of the left atrium are also common. These combined lesions
lead to minimal left ventricular outflow.16 In utero pulmonary vascular resistance remains higher than systemic vascular resistance. The right ventricle is able to maintain normal perfusion of the body through right-toleft shunting through the PDA as a result of elevated pulmonary resistance. Systemic blood flow is based entirely on the ductus arteriosus. After
birth, major problems occur as reversal of the fetal pulmonary-systemic
pressure gradient takes place and the ductus arteriosus closes. Cardiac output collapses and aortic pressure falls, which results in circulatory shock
and metabolic acidosis. Pulmonary edema also develops because of increased pulmonary blood flow and increased left atrial pressure. Signs at
presentation include an ashen gray color, tachypnea, and listlessness, and
a single heart sound, systolic ejection murmur, and decreased pulses are
noted.
Aortic Stenosis Aortic stenosis comprises 6% of congenital heart disease
and has a 4:1 male predominance.17 Stenosis can occur at the valvular,
supravalvular, or subvalvular levels. Infants with severe obstruction
(10% to 15%) present with CHF and poor distal perfusion or shock.18
Left ventricular hypertrophy typically develops in severe stenosis. Patients with aortic stenosis who are asymptomatic in infancy can present
in childhood with syncope and hypertension.
A bicuspid aortic valve is the most common form of aortic stenosis.
Supravalvular aortic stenosis, elfin facies, mental retardation, and pulmonary artery stenosis comprise Williams syndrome. A systolic thrill
may be noticed at the right upper sternal border, suprasternal notch, or
carotid arteries along with an ejection click. There can also be a rough or
harsh grade 2/6 to 4/6 systolic murmur at the right or left sternal border
with transmission to the neck.
■ EVALUATION
History The typical history of the neonate with duct-dependent congenital heart defects is one of a day or two of poor feeding, irritability, or
lethargy followed by decreasing responsiveness, typically in the second
or third week of life. By the time the neonate arrives in the ED, he or she
is often in extremis. A complete history of the pregnancy, labor and delivery, and immediate perinatal period should be obtained and symptoms should be reviewed to rule out noncardiac causes of shock such as
vomiting, diarrhea, fever, and respiratory distress.
Physical Examination Assessment of vital signs should include fourextremity blood pressure measurement to identify a gradient between
upper and lower extremities characteristic of coarctation of the aorta.
Pulse oximetry measurements in the right (preductal) and left (postductal) upper extremities may also reveal a difference suggesting
duct-dependent flow. Tachycardia is usually severe, and tachypnea
may reflect profound metabolic acidosis or may be a manifestation of
CHF. Hypoxemia and cyanosis may accompany cyanotic lesions with
duct-dependent flow. An ashen or gray color is characteristic of the infant with left-sided outflow obstruction in systemic shock, and extremities may be cold and mottled with severely delayed capillary refill. A
single heart sound is characteristic of hypoplastic left heart syndrome,
and a harsh systolic murmur transmitted to the neck may be heard in
824
SECTION 12: Pediatrics
patients with aortic stenosis. A gallop rhythm may be appreciated when
CHF accompanies shock. Pulses are typically thready and may be absent in the lower extremities with significant delay between right brachial and femoral pulses. The lung examination may reveal rales,
tachypnea, and retractions or grunting in neonates with CHF. The infant
may be limp and lethargic.
Diagnostic Tests Blood work that may aid in the diagnosis and management of duct-dependent congenital heart defects includes arterial blood
gas analysis, which often reveals profound metabolic acidosis. Other
electrolyte abnormalities are rare, although renal insufficiency from hypoperfusion may accompany severe shock. A complete blood count is
not routinely helpful but may be obtained to rule out noncardiac causes
of shock, such as sepsis.
Imaging ECG and chest radiography are typically performed and may be
useful in narrowing the differential diagnosis of suspected congenital heart
defects. Table 122A-3 lists the characteristic findings in duct-dependent
acyanotic lesions.
Radiographs are less helpful in duct-dependent acyanotic congenital
heart defects than in cyanotic heart disease, but may be useful when clinical symptoms and signs of CHF exist. Signs of aortic stenosis outside of
infancy are cardiomegaly and posterior rib notching of the third to
eighth ribs from collateral vessels. Bedside echocardiography requires an
ultrasonographer experienced in examining for pediatric heart disease.
■ TREATMENT
Although oxygen is typically administered to patients in shock in order
to increase the dissolved oxygen content of blood and enhance tissue oxygenation, oxygen is a potent pulmonary vasodilator and decreases
right-to-left flow through the ductus arteriosus, potentially worsening systemic perfusion. Oxygen is also a vasoconstrictor of the ductus
arteriosus, which further worsens perfusion. Infants requiring rapidsequence intubation are at high risk for complications, and pretreatment
with atropine is recommended (0.02 milligram/kg IV 2 minutes prior to
sedation and paralysis).
The single most important therapeutic intervention for duct-dependent
lesions is the infusion of IV prostaglandin E1 (PGE1) to restore ductal
patency and improve left-to-right shunting and systemic blood flow. The
initial dose of PGE1 is 0.1 microgram/kg/min, and improvement in peripheral perfusion typically occurs in minutes. Subsequent titration to the
lowest effective dosage is recommended, typically 0.05 microgram/kg/min.
PGE1 can be administered through an umbilical venous catheter, central
line, IO line, or peripheral IV line with equal efficacy. Side effects include
vasodilation and flushing, hyperthermia, hypotension (although blood
pressure typically improves), and apnea. Continuous monitoring of infants receiving PGE1 is therefore advised. In certain variants of TAPVR, administration of PGE1 can exacerbate the patient’s condition.
The infant should be given 10 mL/kg NS with careful reassessment after each bolus to increase preload and improve cardiac output. Infants
with severe CHF may not tolerate much volume. Sodium bicarbonate, 1
to 2 mEq/kg, can be considered for severe metabolic acidosis (pH <7.0),
but may cause paradoxical intracellular worsening of acidosis and myo-
TABLE 122A-3 Duct-Dependent Acyanotic Congenital Cardiac Lesions:
Typical Chest Radiograph and ECG Findings
Cardiac Lesion
Coarctation of the
aorta
Chest Radiograph
Cardiomegaly with pulmonary
edema (neonate)
Rib notching and collateral
vascularity (child)
Hypoplastic left heart Cardiomegaly
syndrome
Aortic stenosis
Cardiomegaly
ECG
RVH, right bundle-branch
block (neonate)
LVH (child)
Right atrial enlargement,
RVH, peaked P waves
LVH in severe cases
Abbreviations: LVH = left ventricular hypertrophy; RVH = right ventricular hypertrophy.
cardial dysfunction, and adequate ventilation must be ensured. Occasionally, pressors such as dopamine or dobutamine may be helpful after
PGE1 infusion has been initiated.
Sepsis cannot be excluded on clinical grounds, so ampicillin, 50 milligrams/kg, and gentamicin, 5 to 7.5 milligrams/kg, or cefotaxime, 50 milligrams/kg, should be given.
Consultation with a pediatric cardiologist and pediatric critical care
specialist is of great importance. Although many practitioners routinely
intubate infants receiving PGE1 prior to transport to tertiary hospitals,
infants in stable condition may safely be transported unintubated.19
CONGESTIVE HEART FAILURE
Congenital heart defects can lead to CHF because of left-sided outflow
obstruction resulting in elevated left atrial pressure (e.g., aortic stenosis)
or pulmonary overcirculation through a PDA or septal defect. Such lesions typically present later in infancy, often in the second through
fourth months of life, with failure to thrive, feeding difficulties, sweating
with feeds, and gradually increasing respiratory distress that may worsen
with respiratory infection. A number of acquired heart conditions, including myocarditis, cardiomyopathy, and arrhythmias, as well as noncardiac conditions such as sepsis, metabolic disease, or severe anemia,
can also cause CHF in infants and children (see Chapter 122B, Pediatric
Heart Disease: Acquired Heart Disease).
■ PATHOPHYSIOLOGY
Significant factors in the development of CHF include increased afterload
from left-sided obstructive lesions (e.g., coarctation or stenosis of the aorta), increased preload or pulmonary circulation from left-to-right shunts
(e.g., large VSD, ASD, PDA), decreased inotropic function (e.g., cardiomyopathy), and rhythm abnormalities (e.g., sustained tachyarrhythmias).
■ DIFFERENTIAL DIAGNOSIS
In addition to congenital structural heart disease, noncardiac disorders
and acquired heart disease should be considered as causes of CHF. Congenital structural causes of CHF not already discussed are described further in the following sections.
Atrial Septal Defects ASDs comprise 10% of congenital heart disease.20
Only 10% of infants with ASD develop clinical symptoms. Large or multiple defects can cause significant left-to-right shunting with overloading
of the pulmonary circulation. Surgical intervention is needed for larger
ASDs, whereas smaller ones may close spontaneously. Difficulty feeding
and trouble gaining weight are common with larger lesions.
Ostium secundum defects represent the majority of ASDs and result
from the incomplete adhesion of the foramen ovale and septum secundum. Ostium primum ASDs result from the insufficient merging of the
septum primum and endocardial cushion with associated abnormalities of
the mitral and tricuspid valves. Sinus venosus ASDs occur when the atrium does not merge with the sinus venosus. In these lesions, a widely split
and fixed S2 with a grade 2/6 to 3/6 systolic ejection murmur at the left sternal border can often be appreciated, along with a mid-diastolic rumble.
Ventricular Septal Defects VSDs are the most common congenital heart
defect, comprising 25% of all such defects.21 VSDs allow blood to mix in the
ventricles. The size of the VSD determines the clinical extent of disease,
with small defects having little or no effect and large defects contributing to
pulmonary hypertension and CHF. Large VSDs create volume and pressure overload in the right ventricle and volume overload in the left atrium
and left ventricle. This results in CHF and poor weight gain and may lead
to developmental delay. A grade 2/6 to 5/6 holosystolic, harsh murmur can
often best be heard at the left lower sternal border and may have an associated systolic thrill or diastolic rumble with a narrowly split S2.
Patent Ductus Arteriosus A PDA is present in 10% of cases of congenital
heart disease and occurs when the ductus arteriosus fails to close spontaneously.22 The degree of shunting through the ductus arteriosus depends
on the length and diameter of the lesion and the pulmonary vascular re-
CHAPTER 122A: Pediatric Heart Disease: Congenital Heart Defects
sistance. Symptomatic patients have large left-to-right shunts. Normally,
the ductus arteriosus closes within 15 hours of birth and seals completely
at 3 weeks of age, becoming the ligamentum arteriosum. Prematurity and
hypoxia can delay closure of the ductus arteriosus. As with all left-to-right
shunts, large PDAs present as CHF. A grade 1/6 to 4/6 continuous “machinery” or “to-and-fro” murmur may be appreciated and is loudest at
the left upper sternal border. A diastolic rumble and bounding pulses can
also be present.
Endocardial Cushion Defect (Common Atrioventricular Canal) Incorrect
development of the endocardial cushion causes defects in the atrial septum, ventricular septum, and AV valves. Complete defects involve the entire endocardial cushion and involve the atrial and ventricular septum as
well as the common AV valve. Incomplete or partial defects have atrial involvement with an intact ventricular septum. Endocardial cushion defects
represent 3% of congenital heart disease cases, with two thirds manifesting
as complete defects.22 Down syndrome and endocardial cushion defects
are strongly associated.
Typical presentations include failure to thrive and frequent respiratory infections. There is a direct relationship between left-to-right shunting and the magnitude of the defects, and complete lesions often lead to
CHF from volume overload of both ventricles early in life.
Usually there is a hyperactive precordium, a systolic thrill, a loud holosystolic regurgitant murmur, and a loud and widely split S2. The ECG
is important and demonstrates a pathognomonic superior QRS axis with
right ventricular hypertrophy, right bundle-branch block, left ventricular hypertrophy, and a prolonged PR interval.
■ EVALUATION
History The typical history of congenital heart defects presenting with
CHF depends on the pathophysiology of the underlying lesion. Cyanotic
lesions often present early in the neonatal period, whereas obstructive
duct-dependent lesions typically present in the second week of life with
feeding difficulties and shock, as previously discussed. Patients with
overcirculation from truncus arteriosus, PDA, and large VSD or ASD lesions usually present after the neonatal period with poor or prolonged feeding, diaphoresis, and respiratory distress associated with feeds, and poor
weight gain, sometimes associated with developmental delay. Parents may
notice increased work of breathing, cyanosis, or frequent respiratory infections or wheezing. Tachyarrhythmias and anemia can also lead to CHF.
Physical Examination The hallmarks of CHF with elevated left-sided
pressure are pulmonary rales and increased work of breathing. Hepatomegaly, with or without splenomegaly, and peripheral or generalized edema suggest right-sided heart failure. Jugular venous distention is often
difficult to appreciate in neonates and infants and may not be present.
Diagnostic Tests The laboratory evaluation of CHF includes measurement of electrolytes and renal function tests, which may be helpful in determining volume status. A complete blood count may reveal anemia,
and determination of red cell indices may point to a cause.
Imaging Chest radiograph may reveal a cardiac silhouette suggestive of
a particular congenital defect, cardiomegaly, and pulmonary edema or
pleural effusion. Table 122A-4 outlines characteristic chest radiograph
and ECG findings in congenital heart defects presenting as CHF. Echocardiography provides a definitive diagnosis.
■ TREATMENT
Oxygen should be administered cautiously. Oxygen saturation of >95%
may cause pulmonary vasodilation and worsen CHF in overcirculating
lesions such as VSD and PDA. The head of the bed should be elevated,
and volume expansion undertaken cautiously if at all. A bolus of 5 to 10
mL/kg of NS may improve cardiac output in some circumstances, but
may worsen failure in others.
The mainstays of CHF treatment are furosemide (1 to 2 milligrams/kg
IV) for diuresis and inotropic support. Adjustments to preload (end-diastolic volume is roughly equivalent to intravascular volume), afterload,
contractility, and heart rate can be attempted.
825
TABLE 122A-4 Acyanotic Congenital Cardiac Lesions Resulting
in Congestive Heart Failure: Typical Chest
Radiograph and ECG Findings
Cardiac Lesion
Chest Radiograph
ECG
Atrial septal
defect
VSD
Cardiomegaly with increased
vascular markings
Cardiomegaly with increased
vascular markings
Cardiomegaly with increased
vascular markings
Cardiomegaly with increased
vascular markings
Right axis deviation, RVH, RBBB
PDA
Endocardial
cushion defect
Anomalous origin of the left
coronary artery
Cardiomegaly
LAH, LVH, (RVH with larger
VSDs)
LVH, RVH with larger PDAs
Superior QRS axis with RVH,
RBBB, LVH, prolonged PR
interval
Abnormally deep and wide Q
waves with precordial ST segment changes
Abbreviations: LAH = left atrial hypertrophy; LVH = left ventricular hypertrophy; PDA =
patent ductus arteriosus; RBBB = right bundle-branch block; RVH = right ventricular hypertrophy; VSD = ventricular septal defect.
For patients in stable condition, digoxin is the inotrope of choice and
improves cardiac contractility and output. The total digitalizing dose is
30 to 40 micrograms/kg for term neonates of >2 kg and 40 to 60 micrograms/kg for infants and children >1 month of age. The total digitalizing dose is administered over 16 to 24 hours as follows: half the total
dose is given as an initial IV bolus; one fourth of the total dose is given
8 to 12 hours after the initial dose; and the remaining one fourth is
given 8 to 12 hours later.
Dopamine and dobutamine should be considered in the acutely ill patient with CHF. Dopamine increases heart rate, blood pressure, and
urine output. Dopamine is given as a continuous infusion at 5 to 10 micrograms/kg/min. Dobutamine reduces afterload through peripheral
vasodilation and improves cardiac output without increasing blood
pressure. Dobutamine is given as a continuous infusion at 5 to 10 micrograms/kg/min, but in infants <1 year of age, tachycardia can result, and
the dose may need to be lowered. CHF associated with hypotension may
require treatment with dopamine and dobutamine.23,24
Amrinone and milrinone have inotropic effects, improve diastolic relaxation, and cause vasodilation but do not augment heart rate, and are
second-line therapy for CHF.
The dose of amrinone is 0.5 milligram/kg IV administered over 3 minutes. Milrinone is given as a loading dose of 10 to 50 micrograms/kg IV
administered over 10 minutes followed by a continuous infusion of 0.5
to 1.0 microgram/kg/min.
Afterload reduction may be useful for conditions unresponsive to standard measures and in consultation with a cardiologist. Nitroprusside is a
mixed vasodilator and can be administered as an infusion of 1 to 10 micrograms/kg/min. Calcium channel blockers may be more effective in cases of
diastolic dysfunction and include diltiazem (0.2 to 0.5 milligram/kg/dose
PO or SL) and nifedipine (0.25 to 1.0 milligram/kg PO), but are contraindicated in infants <1 year of age.
A pediatric cardiologist should be consulted to help guide diagnosis and
management. Transfer to a tertiary care pediatric facility may be necessary.
PEDIATRIC MURMURS
Common benign pediatric murmurs need to be distinguished from murmurs that represent congenital heart defects. The characteristic murmurs of specific cyanotic and acyanotic congenital heart defects were
described in the preceding brief overviews of each lesion. Usually, innocent flow murmurs are of low intensity and do not radiate, are brief
murmurs, and are most often systolic.25,26 Table 122A-5 lists the most
common benign pediatric murmurs and their characteristics.
826
SECTION 12: Pediatrics
TABLE 122A-5 Normal Benign Cardiac Murmurs
Murmur
Age
Character
Positioning
Cause
Still vibratory
murmur
Most common benign
murmur in children 2–6 y,
can occur in infancy to adolescence
Childhood to young adulthood
Grade 1/6–3/6 early systolic ejection murmur, left lower sternal
border to apex, vibratory musical
quality
Grade 2/6–3/6 crescendo-decrescendo, early to midsystolic, left
upper sternal border, second
intercostal space
Grade 1/6–2/6 low pitched, early
to midsystolic ejection murmur
in pulmonic area and radiating
to axillae and back
Louder when patient supine
Postulated to be from
Ventricular septal defect murflow across valvular cordi mur is harsher
Pulmonary flow
murmur
Differential Diagnosis
Louder when patient
supine, increased on full
expiration
Turbulent flow in the pul- Atrial septal defect has fixed
monary outflow tract
split S2; pulmonic stenosis
has higher-pitched, longer
murmur, ejection click
Peripheral
Birth to 1 y
Increased with viral respi- Turbulence at peripheral Significant branch pulmopulmonic
ratory infections, lower
pulmonary artery
nary artery stenosis in Wilstenosis murmur
heart rate, decreased with branches due to acute
liams syndrome; congenital
tachycardia
angles in infants
rubella has higher-pitched
murmur, extends beyond S2,
older child
Supraclavicular
Childhood to young adult- Crescendo-decrescendo, systolic, Decreases with hyperexTurbulent flow through Idiopathic hypertrophic subor
hood
low pitched, above the clavicles, tension of shoulders and major brachiocephalic
aortic stenosis: louder with
brachiocephalic
radiating to neck, abrupt onset reclining position
vessels arising from
Valsalva maneuver and
murmur
and brief
aorta
softer with rapid squatting
Aortic stenosis: higher
pitched, ejection click
Venous hum
Childhood
Faint to grade 6, continuous, hum- Louder when sitting, look- Turbulence from interPatent ductus arteriosus has
ming, low anterior neck to lateral ing away from murmur;
nal jugular and subcla- machinery murmur, not
sternocleidomastoid muscle to
softer when lying, with com- vian veins entering
compressible, bounding
anterior chest below clavicle
pressed jugular vein or head superior vena cava
pulses
turned toward murmur
Mammary
Pregnancy or lactation,
High pitched, systole into dias- —
Plethora of vessels over Patent ductus arteriosus has
souffle
rarely adolescence
tole, anterior chest over breast,
chest wall
machinery murmur, does
varies day to day
not vary day to day
Reproduced with permission from Strange GR, Ahrens WF, Schafermeyer RW, et al (eds): Pediatric Emergency Medicine, 3rd ed. New York, McGraw-Hill Professional, 2009, Table 47-2.
INTERVENTIONAL AND SURGICAL REPAIR
OF CONGENITAL HEART DEFECTS
■ COMPLICATIONS OF CONGENITAL HEART DEFECTS
This section discusses common complications related to surgery for repair of congenital heart defects, complications of medical management,
and infectious complications in children with congenital heart defects.
Diagnosis and management typically require pediatric cardiology consultation and transfer to a tertiary care center.
Tachyarrhythmias or bradyarrhythmias may be caused by the underlying lesion, the surgical repair, or digitalis toxicity. Some lesions may recur after surgery. Temporizing palliative shunts may occlude. Children
with pulmonary artery hypertension may develop pulmonary vasospasm leading to cyanosis and lethargy. Administration of 100% oxygen
facilitates pulmonary vasodilation.
Diuretic administration or intercurrent illness can lead to dehydration and electrolyte imbalance, especially disorders of potassium balance. Hemoconcentration can lead to shunt occlusion. Children can
outgrow their diuretic dosage and develop CHF. Digoxin has a narrow
therapeutic window and can result in dysrhythmias. Anticoagulation in
children has the same adverse effects as in adults, but anticoagulation
should not be reversed with fresh frozen plasma or vitamin K without
consultation with a pediatric cardiologist.
Children with cyanotic congenital heart defects develop an increase in
hemoglobin concentration to compensate for hypoxemia. If hemoglobin
concentrations fall to normal, tachycardia, feeding difficulty, or CHF can
result. Polycythemia causes increased blood viscosity and the potential
for cerebrovascular complications.
Children with congenital heart disease are at high risk for serious complications from infection with viruses such as influenza virus, parainfluenza virus, or respiratory syncytial virus. Acute influenza should be
treated according to standard current guidelines. As of this writing,
guidelines for prophylactic treatment after exposure to influenza virus are
still being finalized. Annual influenza immunization is recommended for
all infants with congenital heart defects. Antiviral therapy for respiratory
syncytial virus infection is controversial, but prevention with virus-specific immune globulin is recommended for most.
Although occult bacteremia has the same probability of occurrence in
a child with congenital heart disease as in a child without congenital
heart defects (see Chapter 113, Fever and Serious Bacterial Illness), bacterial endocarditis is always a concern in a child with congenital heart defects and fever, and parenteral antibiotics (ceftriaxone, 50 milligrams/
kg) should be administered presumptively after appropriate specimens
for culture are obtained. A follow-up visit in 12 to 24 hours is mandatory
for those discharged home from the ED.
Uncorrected congenital heart defects are associated with a 0.1% to
0.2% annual risk of bacterial endocarditis. The risk falls to 0.02% after
correction of most lesions. The usual presentation of endocarditis is
unexplained fever in children with known congenital heart disease.
Transient iatrogenic bacteremia produced by procedures such as dental work or respiratory manipulation can lead to localized colonization
and infection. The latest endocarditis prophylaxis guidelines are reviewed in Chapter 122B, Pediatric Heart Disease: Acquired Heart Disease and may be found at http://www.americanheart.org/presenter.
jhtml?identifier=3004539.
Acknowledgment: The authors gratefully acknowledge the contributions of C. James Corrall, the author of this chapter in the previous edition.
REFERENCES
The complete reference list is available on the included DVD or online
at www.TintinalliEM.com