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Case # 1: Snoring in a 10 year old female
You are seeing a 10 year old female in the Pediatric Pulmonary Clinic at LPCH secondary to a several year
history of snoring that has worsened in the last 6 months. It is most pronounced when she is sleeping in the
supine position or during upper respiratory infections for which she tends to have 4/year; including
recurrent Strep pharyngitis.
Her mother does report witnessed mouth breathing and pauses in her breathing in the last several months
that resolve when she startles herself awake. She was a former straight A student until this past year when
she notes that she has had increasing daytime sleepiness and difficulty concentrating in school which has
resulted in a drop in her grades. She denies any other changes in her mood although her mother notes that
she is more crabby in the morning and difficult to get out of bed despite going to bed every night at 9pm
and waking up at 6am. She denies any recent changes in her sleep pattern/hygiene, enuresis, weight
changes or feelings of depression or anxiety.
Birth history, past medical and surgical histories are unremarkable aside from what is noted above. There is
a family history of seasonal allergies and eczema, but no other chronic illnesses. She is not currently taking
any medications and is otherwise healthy.
On complete review of systems, there is no concern for gastroesophageal reflux disease, restless leg
movements or allergies. Growth and developmental milestones are appropriate. Vaccination report is also
complete.
Physical examination reveals a pleasant, thin but tired appearing 10 year old female with vital signs
appropriate for her age. Her examination was notable for dark circles under her eyes and 4+ tonsillar
hypertrophy.
Questions:
1.
A) What is her diagnosis?
B) What is the definition of sleep apnea?
2. What are the red flags in this case for the diagnosis of sleep apnea?
3. What are three medical complications of sleep apnea?
4.
A) What is a polysomnograph?
B)What does it measure?
5. How do you treat sleep apnea?
Explanations:
1. Studies have shown that about 10% of children 10 years of age and younger snore. Of all children who
snore, about 20% will be found to have obstructive sleep apnea syndrome (OSAS) if sleep studies are
performed. However, some studies have indicated that a large percentage of snoring children have partial
obstruction of airways, or upper airway resistance syndrome (UARS), which can interfere with daytime
functioning. Snoring is a noise that occurs during sleep when a child breathing in and there is some
blockage of air passing through the back of the mouth. The opening and closing of the air passage causes a
vibration of the tissues in the throat. Children who are three years or older tend to snore during the deeper
stages of sleep. Nasal obstruction raises the risk of snoring. Primary snoring is defined as snoring that is not
associated with more serious problems, such as obstructive sleep apnea (OSA), frequent arousals from
sleep, or inability of the lungs to breathe in sufficient oxygen.
Loud and regular nightly snoring is usually abnormal in otherwise healthy children. It can be a sign of a
respiratory infection, a stuffy nose or allergy, or a symptom of underlying sleep apnea. Parents of a child
with sleep-disordered breathing (SDB) are much more likely to report that their child has behavioral or
emotional problems. In 2002, the American Academy of Pediatrics recommended that all children be
screened for snoring and that a diagnosis be made to determine if a child is experiencing normal
primary snoring or OSA.
By the age of two years, most children have spent more time asleep than awake. Overall, a child will spend
about 40 percent of his or her childhood asleep. Research suggests that children require an average of 9 to
10 hours of sleep each night. Sleep is especially important for children, because childhood is an important
time for the brain to mature and to develop. Thus, anything that disrupts the integrity of a child’s sleep can
result in significant impairment of mental or physical development. As mentioned before, parents of a child
with sleep-disordered breathing (SDB) are much more likely to report that their child has behavioral or
emotional problems. SDB causes sleep disruption and changes in oxygen levels that may affect how a
child’s brain develops. Sleep apnea in infants has been linked to some cases of Sudden Infant Death
Syndrome (SIDS).
Sleep apnea is a disorder in which there are pauses in breathing during sleep. Apnea is best defined as the
absence of airflow at the nostrils and mouth. The three main categories of apnea are central, obstructive,
and mixed. Central apnea occurs when respiratory effort ceases; there is no chest movement and, hence, no
airflow. Diagnosis of central apnea must take into account a multitude of factors. Apnea is ordinarily
significant when it exceeds 20 seconds in duration. Since infants normally have a more rapid baseline
respiratory rate and a reduced respiratory reserve (and, therefore, less protection from hypoxia), shorter
central events can be more clinically significant in this age group. Central apnea can yield significant
physiological compromise, such as bradycardia or color change associated with declining oxyhemoglobin
levels.
Central sleep apnea must be distinguished from other causes for respiratory pauses, including periodic
breathing and a respiratory pause following a deep yawn or sigh. In the more severe cases, central apneas
can be treated with medication. The presence of central sleep apneas is usually cause for respiratory
monitoring during sleep. Premature infants are at increased risk for central apneas; central apnea accounts
for 10% to 25% of all apnea in premature infants.6
OSA in children is a disorder of breathing during sleep characterized by prolonged, partial upper-airway
obstruction (hypopnea) and/or intermittent complete obstruction (apnea) that disrupts normal ventilation
during sleep and normal sleep patterns. Clinically, obstructive apnea is the lack or diminution of airflow,
despite the continuation of respiratory efforts. The obstruction can be functional or anatomical. When
obstruction is present, respiratory efforts continue and tugging or retraction of the skin can be seen. For
some children, obstructions lasting less than 10 seconds are important. Thus, some physicians have
suggested using a duration criterion of two respiratory cycles, and others have suggested 6 or 8 seconds as
apnea durations indicating significant upper-airway obstruction during childhood. In some infants with
clinically significant OSA, little or no snoring may be heard by the caregiver.
The most common anatomic factors leading to OSA in children include large tonsils or adenoids, obesity,
micrognathia (small jaw), and other anatomical anomalies. Muscular hypotonia is one of the most common
neurological factors contributing to OSA. Normal muscle-tone inhibition during REM sleep can change
respiratory patterns, with more breath-to-breath variability increasing the risk of full or partial airway
obstruction. Hypotonia can be commonly seen in Down syndrome, muscular dystrophy, and other genetic
disorders. Allergies or even mild upper respiratory infections can serve to swell mucous membranes and,
thereby, contribute to obstruction. Some of the less common risk factors include laryngomalacia,
pharyngeal flap surgery, sickle-cell disease, structural malformations of the brain stem, and certain
metabolic and genetic disorders.
(Scoring for tonsillar hypertrophy)
2. The following are considered red flags or warning signs for sleep apnea (the bolded findings are those
found in our patient):
Snoring or “squeaking” during sleep
Appearing to have difficulty breathing during sleep
Restlessness or sleeping in abnormal positions with the head in unusual positions
Experiencing night terrors, sleep walking, or bed wetting
Mouth breathing
Daytime hyperactivity or being irritable, agitated, aggressive, and cranky
Having problems in school
Difficulty in waking up in the morning
Appearing very sleepy or actually falling asleep during the day
Obesity
Nasal allergies
Tonsillar hypertrophy
Asthma
GERD
An abnormality in the physical structure of the face or jaw
Bed wetting
Neurological conditions
3. Undiagnosed and untreated sleep apnea may contribute to daytime sleepiness and behavioral problems,
including difficulties at school. One recent study of 866 children found that children who frequently snore
or have sleep disorders are twice as likely to have learning problems. Following a night of poor sleep,
children are also more likely to be hyperactive and to have difficulty paying attention. These are also signs
of attention-deficit hyperactivity disorder (ADHD). Failure to treat OSA or SDB puts children at risk for
long-term behavioral problems. Indeed, obstructive sleep apnea (OSA) may also be associated with
impaired growth and cardiovascular problems. Evidence suggests that growth hormone secretion is
adversely affected, thereby disturbing growth.
4. Polysomnography is a comprehensive recording of the biophysiological changes that occur during
sleep. When it is performed according to the standards accepted by the American Thoracic Society, it
provides the clinician with a more thorough evaluation of pediatric sleep and breathing disorders than a
pneumocardiogram can. Polysomnography for cardiopulmonary indications includes simultaneous
recording of physiological variables, including sleep stages, respiration, cardiac rhythm, muscle activity,
gas exchange, and snoring. The personnel in attendance during polysomnography are trained to evaluate
and document behavioral and physiological changes, as well as quality of sleep. Thus, a more accurate
diagnosis of OSA is possible with polysomnography than with the limited-channel pneumocardiogram.
Data used for the staging of infant sleep include the combined measurement of the EEG to record brain
activity, the electro-oculogram (EOG) to record bilateral eye movements, and the electromyogram (EMG)
to record facial and intercostal muscle tone. The placement of the EEG leads is based on the international
10-20 electrode placement system. EEG lead placement for scoring sleep in children is similar to that used
in an adult population. Electrodes are placed at A1, A2, O1, O2, C3, and C4, and sleep stage is determined
by the monopolar derivation C3/A2 or C4/A1. Because of the special criteria used to define sleep states in
infants less than 6 months old and the unique EEG features for this population, an extended EEG montage
or polysomnogram channel derivation is preferred. This extended montage should include bilateral EEG
electrodes using bipolar channels to evaluate the EEG of the two hemispheres of the brain more accurately.
EEG features specific to infants, such as Tracé Alternant and brushes (as well as certain epileptiform
activity), can provide useful information regarding the maturity of the brain and can alert clinicians to
potential problems in brain activity. In addition, certain normal features of the infant EEG, such as
rudimentary sleep spindles, are better seen using an extended EEG montage that includes anteriorly placed
cephalic leads.
The accurate scoring of sleep stages also requires bilateral EOG sensors to monitor the rapid movements of
the eye that normally occur in REM sleep and the slow eye movements that occur with the onset of sleep.
An EMG recording of facial muscle tone assists the clinician in more accurately determining the presence
of REM sleep when skeletal muscle tone, particularly in the muscles of the face, is normally inhibited.
In order to assess the adequacy of ventilation comprehensively and identify (and differentiate between)
central and obstructive apnea and its severity, polysomnograms should include additional parameters:
movements of the chest wall and abdomen, airflow at the nose and mouth, transcutaneous oxygen
saturation (with a validating pulse waveform), and end-tidal carbon dioxide (etco2). A pulse waveform is
necessary to assess the reliability of oxygenation data, since oxygen-saturation monitors can yield
artifactual data at times when the infant is feeding or moving. Capnography, which yields a graphic
representation of etco2, is recommended because it can assess both airflow and ventilation simultaneously.
Calibrated etco2 measurements can effectively detect carbon dioxide retention associated with apnea or
prolonged hypoventilation.
Standard polysomnograms also include parameters that can provide important information relevant to the
patient’s electrophysiological status. An electrocardiogram provides data on the cardiac rate and rhythm
and is useful in evaluating the consequences of breathing disorders on the heart. An EMG recording of the
intercostal muscles detects expansion of the chest wall, assessing the presence of respiratory effort to help
differentiate between central and obstructive apnea. A polysomnogram can include EMG of the anterior
tibialis muscles to identify periodic leg movement disorders, though these disorders are rare in infants and
leg EMGs are not routinely monitored in children less than 6 months old.
In order to assess the infant for the presence of gastroesophageal reflux and its potential cardiorespiratory
consequences, continuous esophageal pH measurement can be done in conjunction with polysomnography.
Video recording with sound is recommended because it provides invaluable information on sleep behavior,
breathing noises, respiratory effort, and sleep positions associated with a particular respiratory pattern.
5. Contrary to treatment practices in adults, surgery remains the first line of treatment for OSA in children.
The most commonly performed surgical procedure for the treatment of pediatric OSA is
adenotonsillectomy (T&A). In children the size of the airway is relatively smaller in relation to the size of
the tonsils and adenoids, as compared to adults. This increased size of the tonsils and adenoids results in a
greater role in obstruction of the airway during sleep. Indeed, the disorder is most common between 2 to 6
years of age, when the lymphoid growth is greatest in childhood. Through T&A, the removal of tissues in
the upper airway relieves obstruction and improves airflow. Following T&A, the upper airway stability is
improved. It is important to emphasize that both tonsillectomy and adenoidectomy should be performed, if
the objective of the operation is to improve airway obstruction in OSA. Although isolated adenoidectomy
or tonsillectomy does improve OSA, the improvement may be incomplete, thus leading to further surgical
procedures down the road. A sleep study should be performed after surgery as well, to determine the extent
of improvement from surgery. Although the success rate of surgery is quite high (about 80%), many
children still exhibit residual problems.
In some cases, T&A may not completely treat pediatric OSA. These cases occur when a child has
significant nasal obstruction or maxillomandibular deficiency (i.e. a small jaw). In these cases, the other
problem area or areas need to be addressed. Significant nasal obstruction can be treated in a few different
ways. If nasal allergies are a contributing factor, then inhaled nasal corticosteroids can be used to treat the
symptoms. This treatment is usually successful but not ideal, because the inhaled corticosteroids may need
to be used long-term. If nasal turbinate enlargement is a contributing factor, then turbinate reduction by
radiofrequency ablation may be performed in conjunction with T&A. Radiofrequency ablation is a
method of applying heat to a local area of soft tissue to cause scar tissue to form, thereby reducing the size
of the soft tissue. This procedure can be performed under local anesthesia.
If obstruction is caused by a narrow nasal airway or narrow upper and lower jaws, then orthodontic
expansion (i.e. maxillomandibular expansion) to widen the upper airway can be successful. The upper and
lower jaws can be expanded by orthodontic treatment, usually without the need of surgery.
Maxillomandibular expansion is often performed in children with obvious jaw deformity or when there is
residual OSA after T&A have been performed. The goal of the treatment is to widen the upper and lower
jaws with an orthodontic appliance. This treatment can be performed in children as young as 5 years old.
Occasionally, limited surgery to facilitate expansion may be necessary in teenagers when a significant
degree of jaw maturation has already occurred.
Finally, a few pediatric OSA patients may be best treated with nasal continuous positive airway pressure
(CPAP). This option may be indicated in a small portion of cases which remain unresolved after surgery or
orthodontic treatment. Another indication for CPAP would be a situation where surgery or other methods
may not be suitable for treatment.
References:
http://www.dallas-sleep.com/pediatric-sleep-apnea.php
http://physicianlink.uams.edu/casestudies/sleepapnea_children.asp
http://www.sleepreviewmag.com/issues/articles/2001-10_03.asp