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Chapter 31
Neonatal and Pediatric
Respiratory Disorders
Copyright © 2013, 2009, 2003, 1999, 1995, 1990, 1982, 1977, 1973, 1969 by Mosby, an imprint of Elsevier Inc.
Learning Objectives




Discuss the clinical findings, radiographic
abnormalities, and treatment of patients with
respiratory distress syndrome (RDS).
Describe the clinical manifestations and treatment
of patients with transient tachypnea of the newborn
(TTN).
Describe the pathophysiology, presentation, and
treatment of meconium aspiration syndrome.
Discuss the clinical signs and symptoms
associated with bronchopulmonary dysplasia and
the approaches used to manage these infants.
Copyright © 2013, 2009, 2003, 1999, 1995, 1990, 1982, 1977, 1973, 1969 by Mosby, an imprint of Elsevier Inc.
2
Learning Objectives (cont.)




Discuss the etiology and treatment of apnea
of prematurity.
Describe the pathophysiology, diagnosis, and
treatment of persistent pulmonary
hypertension of the newborn (PPHN).
Describe the pathophysiology, diagnosis, and
treatment of congenital diaphragmatic hernia.
Describe the anatomic defects associated
with tetralogy of Fallot.
Copyright © 2013, 2009, 2003, 1999, 1995, 1990, 1982, 1977, 1973, 1969 by Mosby, an imprint of Elsevier Inc.
3
Learning Objectives (cont.)




Describe the clinical presentation of a
ventricular septal defect.
Define the epidemiologic factors associated
with increased risk of sudden infant death
syndrome (SIDS).
Discuss the respiratory problems associated
with gastroesophageal reflux disease.
Describe the clinical findings commonly
observed in patients with bronchiolitis.
Copyright © 2013, 2009, 2003, 1999, 1995, 1990, 1982, 1977, 1973, 1969 by Mosby, an imprint of Elsevier Inc.
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Learning Objectives (cont.)


Describe the clinical features and treatment
of children with croup and epiglottitis.
Describe the clinical manifestations and
treatment of cystic fibrosis.
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Lung Parenchymal Disease




Respiratory Distress Syndrome
Transient Tachypnea of the Newborn
Meconium Aspiration Syndrome
Bronchopulmonary Dysplasia
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6
Respiratory Distress Syndrome


Also called hyaline membrane disease
Disorder of prematurity characterized by:




Qualitative surfactant deficiency
• Insufficient amounts released by type II cells
• Poor quality so cannot decrease surface tension
Decreased alveolar surface area
Increased small airway compliance
Patent ductus arteriosus
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7
When persistent fetal circulation occurs in infant
respiratory distress syndrome, blood bypasses the
infant’s lungs through which fetal pathways?
A.
B.
C.
D.
ductus venosus & ductus arteriosus
ductus venosus & foramen ovale
umbilical vein & ductus arteriosus
ductus arteriosus & foramen ovale
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Respiratory Distress Syndrome
(cont.)

Lung prematurity, & particularly surfactant
deficiency, sets up following sequelae:

Alveolar instability & collapse, leads to increased
WOB
 Simultaneously increased surface tension draws
fluid into alveoli
 Combined, these cause impaired oxygenation with
hypoxemia & acidosis, which lead to increased PVR
 Increases right-to-left shunting through PDA
 Worsens acidosis, hypoxemia, & surfactant
production, which establishes downward spiral
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9
Respiratory Distress Syndrome
(cont.)
Copyright © 2013, 2009, 2003, 1999, 1995, 1990, 1982, 1977, 1973, 1969 by Mosby, an imprint of Elsevier Inc.
10
Respiratory Distress Syndrome
(cont.)

Clinical manifestations




Tachypnea occurs soon after birth
Followed by worsening retractions, paradoxical
breathing, & grunting
• Nasal flaring may be seen
Chest auscultation: fine inspiratory crackles
Diagnosis is made from chest radiography
• Diffuse, hazy reticulogranular densities, air
bronchograms, & low lung volumes are typical
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11
Respiratory Distress Syndrome
(cont.)
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12
Respiratory Distress Syndrome
(cont.)

Treatment

Noninvasive CPAP (nasal prongs) preferred
• Trial with 4 to 6 cm H2O
• Intubate & MV if PaO2 does not improve, apnea or acidosis
occurs

MV with PEEP: goals
are to prevent alveolar collapse &
.
provide adequate VA
• Optimal PEEP provides lowest PaCO2 & highest PaO2
• If high PaCO2 persists, must increase frequency or PIP
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CPAP is administered to an infant diagnosed with
respiratory distress syndrome in an effort to do all
of the following, except:
A.
B.
C.
D.
Increase the infant’s PaO2
Decrease the infant’s work of breathing
Decrease the infant’s FRC
Decrease required FiO2
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Respiratory Distress Syndrome
(cont.)

Treatment (cont.)

Surfactant replacement
• instilled into trachea indicated for all RDS patients
• surfactants have protein B & C, which is important for
decreasing surface tension
• common commercially available surfactants include
Survanta, Infasure, & Curosurf

High-frequency ventilation (HFV)
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15
Transient Tachypnea of the Newborn
(TTN)

Often called Type II RDS



may be most common newborn respiratory
disorder
Term babies with no specific predisposing
factors
Probably occurs due to delayed clearance
fetal lung fluid


Thoracic squeeze expels about two-thirds of fluid
in birth canal
• Cesarean section avoids this squeeze
Lymphatics’ absorption accounts for final third
• Immature lymphatics impairs absorption
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Transient Tachypnea of the Newborn
(cont.)

Clinical manifestations



No. 1 is tachypnea
.
VA, pH, & PaCO2 are normal
Features of chest radiograph:
• May look like pneumonia
• Hyperinflation
• Pleural effusions
• Perihilar streaking (lymphatic engorgement)
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Transient Tachypnea of the Newborn
(cont.)

Treatment

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


Responds well to low FIO2 with oxygen hood or
nasal cannula
May benefit from CPAP
May use antibiotics (TTN hard to tell from
pneumonia)
Frequent changes in infant’s position may help
speed lung fluid clearance
Most treatments have rapid response (24 to 48
hours)
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18
Meconium Aspiration Syndrome
(MAS)


Disorder of term or near-term infants, associated
with perinatal depression & asphyxia
Pathophysiology

Depression & asphyxia lead to passing of meconium
 MAS involves three primary problems:
• Pulmonary obstruction due to meconium plugging
• Lung damage due to chemical injury
• Persistent pulmonary hypertension (PPHN)
 Chemical effects include inflammatory responses,
cytokine & chemokine activations, compliment
activation, phospholipase A2 activation
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19
Meconium Aspiration Syndrome
(cont.)
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20
Meconium Aspiration Syndrome
(cont.)

Clinical manifestations




Gasping respirations, tachypnea, grunting,
retractions
Chest radiograph
• Irregular pulmonary densities (atelectasis)
• Hyperlucent areas (hyperinflation)
ABGs: hypoxemia, mixed acidosis due to PPHN
Treatment - If severe



Immediate intubation & suctioning
Mechanical ventilation
HFV, surfactant, & iNO therapy may all be used
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21
Meconium Aspiration Syndrome
(cont.)
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Bronchopulmonary Dysplasia (BPD)

Pathophysiology: Multifactorial




Atelectrauma is result & cause of lung injury
Volutrauma injures airways & lung parenchyma
Above two synergistically increase lung injury,
necessitate oxygen therapy (leads to oxygen
toxicity)
Immaturity, genetics, malnutrition, oxygen toxicity,
hypoxia, MV all contribute to BPD development
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Bronchpulmonary Dysplasia (cont.)

Clinical manifestations



Progressive respiratory distress at 2–3 weeks of
life
• If did not require O2 & MV before, will now
Chest radiograph shows diffuse areas of
atelectasis, emphysema, & fibrosis
ABGs show varying hypoxemia & hypercapnia
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24
Bronchpulmonary Dysplasia (cont.)
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Bronchopulmonary Dysplasia (cont.)

Treatment
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Prevention of atelectrauma & volutrauma is KEY
Establish optimal FRC in delivery room
Early use of surfactant
Minimize lung damage: monitor carefully & use lowest
oxygen & MV settings possible
Diuretics to treat pulmonary edema
Antibiotics for pulmonary infections
Chest physiotherapy for retained secretions
Bronchodilator therapy to decrease Raw
Steroids used cautiously
• create as many problems as they solve
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Control of Breathing

Apnea of prematurity

Common in premature infants
 Apnea is abnormal if >15 seconds OR it causes
cyanosis, pallor, hypotonia, or bradycardia
 Central apnea: no respiratory effort during apnea
 Obstructive apnea: effort but no flow
 Mixed apnea: combination of central & obstructive
apnea
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Control of Breathing (cont.)

Apnea of prematurity (cont.)

Etiology
• Immature chemo-control of respiratory drive
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Control of Breathing (cont.)
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Pulmonary Vascular Disease

Persistent pulmonary hypertension of the
newborn (PPHN)


Prolonged postpartum fetal circulation due to ⇑ PVR
Pathophysiology
• Pulmonary blood flow is low due to right-to-left shunt
through patent foramen ovale or ductus arteriosus

Three types of PPHN
1. Vascular spasm: triggered by hypoxemia or pain
2. Increased muscle wall thickness: chronic
3. Decreased cross-sectional area of vasculature

Related to lung hypoplasia & occurs with congenital
diaphragmatic hernia, Potter’s sequence, & oligohydramnios
syndromes
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Pulmonary Vascular Disease
(cont.)

Persistent pulmonary hypertension of the
newborn (cont.)

Clinical manifestations
• Suspect when rapidly changing SpO2, hypoxemia worse
than indicated on chest radiograph
• Detected by performing pre-ductal & post-ductal SpO2

Pre-ductal SpO2 > 5% post-ductal SpO2
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Pulmonary Vascular Disease
(cont.)

Persistent pulmonary hypertension of the
newborn (cont.)

Treatment
• Correct underlying problem

Hypoxemia with oxygen, surfactant for RDS
• May require intubation, MV, & sedation
• Inhaled Nitric Oxide (INO) therapy
• Often require: paralysis, HFV, worst cases ECMO
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Congenital Abnormalities Affecting
Respiration





Airway Diseases
Lung malformations
Congenital Diaphragmatic Hernia
Abdominal wall abnormalities
Neuromuscular Control
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33
Airway Diseases

Three mechanisms
1. Internal obstruction
• Laryngomalacia, tracheomalacia, laryngeal webs,
tracheal stenosis, & hemangiomas
• Stridor, gas-trapping, wheezing, accessory muscles
2. External obstruction (symptoms similar to
internal)
• Neck or thoracic mass, vascular rings
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Airway Diseases
3. Airway disruptions
• Tracheoesophageal fistula (TEF)


Five types, all manifest as difficulty swallowing, frothing at
mouth, & choking
– Esophageal atresia with proximal fistula
– Esophageal atresia with distal fistula
– Esophageal atresia with both proximal & distal fistula
– Esophageal atresia without either fistula
– Intact esophagus with H fistula
Often associated with other congenital abnormalities
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Lung Malformations

Most common is congenital pulmonary
adenomatoid malformation (CPAM)

classified into five types based on type & size of
cyst
 symptoms of volume loss
 as mass expands, normal surrounding lung is
compressed
 usual treatment is surgical resection of affected
lobe
 cardiopulmonary compromise occurs in severe
cases
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Congenital Diaphragmatic Hernia

Two types:
1. Bochdalek hernia
•
lateral & posterior defect, usually on the left
2. Morgagni hernia
•

medial & anterior, may be on either side
Combination of lung hypoplasia & abnormal
development
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Congenital Diaphragmatic Hernia
(cont.)

Physical findings:






scaphoid abdomen
decreased breath sounds
displaced heart sounds
severe cyanosis
Manifests as severe respiratory distress
Diagnosis established with chest radiography
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38
Congenital Diaphragmatic Hernia
(cont.)

Treatment



Initially intubation, paralysis, MV, continuous
gastric insufflation
Repair delayed for PVR to fall due to adequate VA
Severe cases may require HFV & ECMO
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39
Abdominal Wall Abnormalities



Affect breathing as infants are abdominal
breathers
If severe may require MV
One of most common is omphalocele
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Neuromuscular Control



Spinal muscular atrophy, congenital
myasthenia gravis
May require MV if severe
Morbidity & mortality extremely variable
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41
Survival from congenital diaphragmatic hernia is
dependent on many complex variables that include
all of the following, except:
A.
B.
C.
D.
liver herniation into the thorax
fetal head to lung ratio
gestational age at birth
initial PaO2 and PaCO2
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42
Congenital Heart Disease

Two large categories:
1. Cyanotic Heart Diease
•
Blood shunts from right to left, bypassing lungs
2. Acyanotic Heart Disease
•
Blood shunts left to right causing congestive heart
failure
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43
Congenital Heart Disease (cont.)
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Cyanotic Congenital Heart Defects

Tetralogy of Fallot:




Pulmonary stenosis, VSD, dextroposition aorta, RVH
Timing of surgical repair dependent on case
Chest radiograph shows “boot-shaped’ heart
Transposition of great arteries




Manifests as moderate to severe cyanosis at birth
Emergency atrial septostomy relieves distress
Perform arterial switch operation at 2–3 weeks of life
Chest radiograph shows “egg-shaped” heart
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45
Acyanotic Congenital Heart Defects

Ventricular septal defect (VSD):


most common
• Results in left-to-right shunt & CHF
• Appears 6–8 weeks as PVR falls
Atrial septal defect (ASD):


typically of little importance
Most common
•
incomplete closure of foramen ovale
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46
Acyanotic Congenital Heart Defects
(cont.)

Patent ductus arteriosus (PDA)




Typically occurs in premature infants
Normally closes 5 to 7 days after birth of term
infants
May act as right-to-left or left-to-right shunt
depending on SVR & PVR
Treatment is either pharmacologic (indomethacin)
or surgical (ligation)
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47
Acyanotic Congenital Heart Defects
(cont.)

Left ventricular outflow obstructions


Includes aortic stenosis, coarctation of aorta, &
hypoplastic left heart syndrome
Treatment: Depends on severity
• Initially prostaglandin E1 may be administered
• In severe cases:


Mechanical ventilation is required
Surgical procedures
– Stenosis & coarctation: Resection or graph
– Hypoplastic left heart: Norwood procedure or tranplantation
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48
Acyanotic Congenital Heart Defects
(cont.)
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49
Acyanotic heart diseases are diseases in which
blood shunts from left to right. The congenital heart
disease that is NOT considered acyanotic is:
A.
B.
C.
D.
transposition of the great arteries
ventricular septal defect
atrial septal defect
patent ductus arteriosus
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50
Pediatric Respiratory Disorders






Sudden Infant Death Syndrome (SIDS)
Gastroesophageal Reflux Disease (GERD)
Bronchiolitis
Croup
Epiglottis
Cystic Fibrosis
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51
Sudden Infant Death Syndrome
(SIDS)


Leading cause of death in infants < 1 year of
age
Prevention of SIDS

Identification of infant at risk
 Train family in apnea monitoring & CPR
 Place patient in supine or side-lying position
 Reduce soft objects in sleeping environment
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52
Sudden Infant Death Syndrome
(cont.)
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53
Gastroesophageal Reflux Disease
(GERD)

Associated respiratory problems include :







Reactive airways disease
Aspiration pneumonia
Laryngospasm
Stridor
Chronic cough
Choking
Apnea
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Gastroesophageal Reflux Disease
(GERD) (cont.)


Diagnosed with esophageal pH testing, upper
gastrointestinal contrast studies, and gastric
scintiscanning
Once diagnosed, medical and surgical
management is required
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55
Bronchiolitis


Caused by respiratory synctial virus (RSV)
Clinical manifestations






Usually follows URTI
Slight fever & cough worsen to dyspnea & tachypnea
Inspiratory & expiratory wheezing may develop
Radiograph shows hyperinflation & consolidation
Prophylaxis: passive immunization became
available for RSV
Treatment:


relief of airway obstruction & hypoxemia
MV with prolonged expiration used in most severe cases
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Croup


Also called laryngotracheobronchitis
Viral infection resulting in subglottic swelling


most common cause of obstruction in 6-month- to 6year-olds
Clinical manifestations

Follows 2–3 days of nasal congestions, fever, cough
 Progressive inspiratory & expiratory stridor
 Barking cough
 Progression to dyspnea, cyanosis, & exhaustion
 Chest radiograph shows classic “steeple sign”
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57
Croup (cont.)
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Croup (cont.)

Treatment





Mild to moderate disease treated with cool mist
therapy with or without supplemental oxygen
Corticosteroids & epinephrine shows greatest
benefit in reducing length & severity of symptoms
Addition of budesonide may help
Severe cases progressing despite therapy may
require intubation & mechanical ventilation
Heliox may be used for severe cases
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59
Epiglottis



Most commonly caused by H. influenzae type B
Life-threatening infection resulting in supraglottic swelling
Clinical manifestations





High fever, sore throat, stridor, labored breathing
NOT associated with barking cough
May have difficulty swallowing & muffled voice
Lateral neck radiograph shows “thumb sign”
Treatment: proceed with great care


Elective intubation under general anesthesia
Corticosteroids may decrease swelling
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60
Epiglottis (cont.)
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Cystic Fibrosis

Most common lethal genetic disease in white
Americans

Genetic mutation of gene coding for large protein
called cystic fibrosis transmembrane conductance
regulator (CFTR) which affects chloride movement,
particularly in exocrine glands
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62
Cystic Fibrosis (cont.)

Clinical manifestations





Most severely affected organs:
• Sweat glands, pancreas, & lungs
Skin is very salty: mother notes when kissing infant
• Sweat chloride test is diagnostic
• > 60 mEq/L indicates presence of disease
Pancreatic insufficiency leads to malnutrition,
diarrhea, & steatorrhea
Lung disease is leading cause of death
Patients produce copious amounts of thick, mucoid
sputum
• Retained secretions lead to recurrent infections, atelectasis,
pneumonia, or lung abscesses
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63
When two carriers of cystic fibrosis produce
children, the chance that CF will develop in
offspring is
A.
B.
C.
D.
1 in 2
1 in 3
2 in 3
1 in 4
Copyright © 2013, 2009, 2003, 1999, 1995, 1990, 1982, 1977, 1973, 1969 by Mosby, an imprint of Elsevier Inc.
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Cystic Fibrosis (cont.)

Monitoring of progression & response to
treatment


Routinely accomplished with spirometry & chest
films
Cultures of sputum used to monitor airway flora &
resistance
Copyright © 2013, 2009, 2003, 1999, 1995, 1990, 1982, 1977, 1973, 1969 by Mosby, an imprint of Elsevier Inc.
65
Cystic Fibrosis (cont.)

Treatment






Pancreatic enzyme supplements
Regular chest physiotherapy
Strenuous exercise, external pneumatic vests, PEEP
devices, or autogenic drainage may substitute for
regular chest physiotherapy
DNase, 7% saline preserve lung function
Antibiotics are crucial to treat bronchiectatic flare-ups
May use anti-inflammatory agents & bronchodilators
Copyright © 2013, 2009, 2003, 1999, 1995, 1990, 1982, 1977, 1973, 1969 by Mosby, an imprint of Elsevier Inc.
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