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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