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  • 1. PEDIATRIC SLEEP DISORDERS (RESPIRATORY) Lee J. Brooks, MD, FCCP Associate Professor of Pediatrics University of Pennsylvania Division of Pulmonary Medicine The Children’s Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia, PA 19104 215-590-3749 215-590-2548 (fax) 1
  • 2. Pediatric Sleep Disorders (Respiratory) Pediatric sleep disorders is a downloadable PDF file that presents an overview of various sleep disorders relating to breathing in children. Topics that are covered include obstructive sleep apnea (OSA), sudden infant death syndrome (SIDS), and apparent life-threatening events (ALTE), each with diagnosis and various solutions and treatments. References are available for more in-depth papers. This information has been modified from work prepared by Lee J. Brooks, MD, FCCP, of the Children’s Hospital of Philadelphia for the American College of Chest Physicians and is published here with permission. Objectives 1. Identify children who might be at risk for obstructive sleep apnea (OSA). 2. Evaluate children with OSA. 3. Determine treatment for children with OSA. 4. Identify risk factors for sudden infant death syndrome. 5. Evaluate an infant who has had an apparent life-threatening event. Key words: sleep, children, infants, polysomnography, apnea, SIDS, ALTE OBSTRUCTIVE SLEEP APNEA Snoring is the most common presenting complaint of children with obstructive sleep apnea (OSA).1 Twelve percent of children snore “on most nights,” and a significant amount of sleep and breathing irregularities occur in almost 1% of 4-5 year olds. 2 OSA can have important effects on daytime cognitive function 3, 4 and behavior, 5 as well as cardiopulmonary (heart and lung) function. Pathophysiology Just as sucking on a wet paper drinking straw results in collapse of the straw, the tendency of the human airway is to collapse when we breathe inwards. This tendency for our airway to collapse is balanced by muscles in our airway, including the tongue, whose activity when we inhale helps maintain an open airway. Thus, any factors that decrease the size of the airway or increase the floppiness allow 2
  • 3. the individual airway to collapse and obstruct. Pediatric conditions that increase the floppiness and collapsibility of the airway include normal sleep, as well as neurological conditions including cerebral palsy. Pediatric conditions that decrease the size of the airway include enlarged adenoids and tonsils and craniofacial conditions such as Pierre Robin syndrome, achondroplasia, and midfacial hypoplasia. Some conditions such as obesity, Down’s syndrome, and Prader-Willi syndrome may result in both a small and floppy airway. 6 Airway collapse may produce complete obstruction of the airway resulting in a pause in breathing, called apnea, or less than complete obstruction, termed a partial obstruction or obstructive hypopnea. Either an apnea or a hypopnea may result in decreases in the body’s oxygen level and increases in the carbon dioxide level, which can produce brief awakenings; many of the long-term effects of OSA are a result of repetitive drops in the oxygen level or disrupted sleep (Table 1). The laboratory evaluation of patients with snoring and suspected OSA is dedicated to detecting and quantifying apneas and hypopneas and their effects on the body’s oxygen level and sleep disruption. 7 Clinical Signs and Symptoms Clinical findings may occur during both sleep and wakefulness (Table 2). It is important to obtain the clinical history from a parent or even a sibling who shares a room and might have observed the patient while sleeping; few of us are aware of (and admit to) snoring and other nighttime symptoms. Snoring is usually the presenting complaint of patients with OSA. Respiratory pauses, often ending with a snort or gasp, are also often described. The snoring may be cyclical, worsening with the decrease in motor tone associated with rapid eye movement (REM), dream sleep. During REM sleep, the body loses the tone of some muscles that assist in breathing, allowing worsening of OSA. These symptoms are often worse when sleeping on our back when gravity pulls the tongue to block the airway. Restless sleep may be a sign of frequent brief awakenings secondary to pauses in breathing. The patient does not usually know these brief awakenings are happening. It is often helpful to inquire about the condition of the bedclothes in the morning; the only clue to the extent of restless sleep may be finding the sheets and blankets all askew. 3
  • 4. As in adults, obesity may predispose the individual to OSA. 8,9 In contrast to adults, however, children with OSA may fail to thrive, perhaps because of increased difficulty breathing during sleep, burning additional energy.10 These children usually display catch-up growth after effective treatment for their sleep apnea. The frequent awakenings and poor quality sleep may result in daytime sleepiness. Sometimes this is obvious, as in the child who falls asleep in school or at the dinner table. Frequently, it is more subtle, as a short temper, behavior problems, or academic difficulty in school. These children may be misdiagnosed with attention deficit hyperactivity disorder. The teacher may report the child “daydreams” excessively or is inattentive; these episodes may represent brief “microsleeps” as the child attempts to repay his “sleep debt.” Sleepiness in the absence of snoring may suggest narcolepsy or simply inadequate sleep due to insomnia or schedule problems. Nocturnal asthma and gastroesophageal reflux (heartburn) may also disturb nocturnal sleep and result in excessive somnolence. Not all children who snore have OSA. No combination of historical or physical factors has been useful in distinguishing OSA from primary snoring. 11-14 Laboratory confirmation is essential to confirm the diagnosis and establish the severity of the disorder so that the appropriate therapy may be instituted. Laboratory Evaluation The child with suspected OSA should be evaluated with overnight polysomnography (PSG) in a sleep laboratory experienced in the care of children. This includes an atmosphere that is friendly and not threatening, resembling a home bedroom as much as possible. A parent should be encouraged to stay overnight with a young child. A nap study is inadequate since REM intensifies through the night and the severity of the problem may not be appreciated if sufficient REM sleep is not achieved. 15 Full PSG includes measures of respiratory effort, airflow, carbon dioxide, oxygen levels, electrocardiogram (heart beat), and electroencephalography (brainwaves) and electromyography (muscle movements) to analyze sleep (Table 3). In this manner, the nature and extent of any heart and breathing events, as well as their effects on sleep quality, can be assessed. 7 It is important to record the quality of sleep since a reduction 4
  • 5. of REM sleep or sleep in general may result in underestimating the severity of the disorder. Although there is some night-to-night variability in heart and breathing functions, and particularly sleep quality, this is not enough to invalidate a technically adequate study. Normal PSG values in children are not well defined. One study suggests that more than one obstructive apnea per hour of sleep is abnormal in children,16 but there are no data defining any clinical consequences of so few respiratory events. In young adults, a respiratory disturbance index (RDI, apneas plus hypopneas per hour of sleep) of up to 5 is considered normal, and there may be a marked increase in mortality in patients with apnea more then 20 per hour of sleep. 17 Since young patients may not exhibit the classical obstructive pattern seen in adults with OSA, 18 the laboratory must be experienced with children in order to detect these often-subtle events. Abnormal heart rhythms are uncommon in children, but when present, indicate the need for prompt intervention. OSA cannot be differentiated from primary snoring by history and physical findings alone; only about half of the patients who present with signs and symptoms suggestive of OSA have the syndrome, as confirmed on PSG.11-14 Although enlarged adenoids and tonsils are commonly associated with OSA, the size of the tonsils and adenoids do not predict the number of nighttime respiratory events.8 The PSG is also helpful in predicting response to treatment and the need for follow-up,13 as well as predicting postoperative risk and the extent of monitoring that is required after treatment. 19,20 More limited diagnostic methods have not been helpful in confirming the diagnosis of OSA, 21 although some show promise with careful patient selection and careful attention to technique.22 Treatment in Otherwise Normal Children Seasonal variations of the symptoms suggest the possibility of an allergic component that can be defined by a type of skin testing. A clinical trial of nasal decongestants and/or anti-inflammatory medications (nasal steroids or cromolyn) may be warranted. If this does not result in marked improvement, a sleep study is required to establish the diagnosis and severity of OSA. An x-ray picture of the side of the neck can help identify enlarged adenoids and the extent that the adenoids decrease the size of the airway. When there are enlarged tonsils and adenoids in the presence of OSA, surgically removing the tonsils 5
  • 6. and adenoids is usually sufficient to treat children with mild to moderate OSA. Surgery may not be curative in children with severe OSA (RDI>20), so follow-up PSG is usually required to insure an adequate response to surgery, even in the absence of continued snoring. 13 In patients without enlarged tonsils or adenoids, patients with an inadequate response to removal of tonsils and adenoids, or patients who would prefer to avoid surgery, nasal continuous positive airway pressure (CPAP) is a useful option. 23 CPAP is the use of air pressure to maintain an open airway using a flow generator, tubing and seal over the nose. Determining the amount of CPAP required for the individual and the type of mask that provides the most comfortable delivery of pressure is best done in a sleep laboratory experienced in CPAP titration in children. We do not recommend attempting to titrate CPAP in the home or in the latter half of a diagnostic PSG. A considerable amount of education of both the parent and child, as well as some positive reinforcement, is required to establish patient cooperation and compliance and to determine the proper mask fit and pressure. Children may require changes in mask size or pressure as they grow, so regular follow-up with an individual experienced with OSA in children is essential. Unlike adults, children may be able to discontinue CPAP with growth of the facial structures and/or maturation of neuromuscular function of the airway. A comprehensive weight loss program should be initiated in children who are obese, and this may be sufficient to normalize mild to moderate OSA. Patients with more severe OSA or symptoms of daytime sleepiness may be treated with CPAP while they lose weight. Children With Craniofacial or Neurological Disorders As many as half the children in some series have obvious abnormal facial structure or underlying neurologic disorders.24 Physicians caring for children with craniofacial or neurologic disorders should have a high index of suspicion for OSA since these children are at risk because of a small and/or floppy airway. Some medications such as benzodiazepines may also decrease airway muscle tone. Treatment is best undertaken with a team approach with the sleep physician working with the surgeons, pediatricians, and family.25 Families should be questioned about snoring or other symptoms of OSA and, if present, the children should be evaluated with a sleep study. The presence of OSA may accelerate the decision to perform craniofacial surgery. Conversely, nasal CPAP can be used while 6
  • 7. children achieve the optimal age and/or weight for surgery. Occasionally patients require a tracheostomy (small hole in the lower neck) to bypass the upper airway completely. Fortunately, this is required much less frequently with the advent of CPAP. Some procedures performed in adults that change the position of the upper and lower jaws are usually not performed in children to avoid affecting growth of the facial structures. Summary Almost 10% of children who snore have significant sleep and breathing disorders. Obstructive sleep apnea results in drops in the body’s oxygen level and disrupted sleep that can lead to poor school performance, behavior problems, high blood pressure, heart failure, and poor weight gain. Treatment should be considered only when the severity of the syndrome has been established by objective testing. An overnight sleep study in a laboratory experienced in the evaluation of children remains the gold standard for differentiating primary snoring from OSA. A sleep study can also guide the choice of treatment, intensity of monitoring, and need for follow-up. In otherwise normal children with OSA and enlarged tonsil and adenoid tissue, surgically removing the tissue is usually curative of mild to moderate OSA. In children with more severe OSA, nasal CPAP may be required. CPAP is also helpful in children with neurologic problems, or craniofacial problems in whom surgery is not yet appropriate. CPAP is best initiated by an individual with skill and experience with CPAP in children. SUDDEN INFANT DEATH SYNDROME Sudden infant death syndrome (SIDS) is defined as the sudden death of any infant or young child that is unexplained by history and in which a thorough post-mortem examination fails to demonstrate an adequate cause of death.26 It is the most common cause of postneonatal infant death, occurring in about 1 per 1,000 live births and causing about 3,000 deaths per year.27 Infants at high risk include those born prematurely, infants who have had apparent life threatening events (ALTEs), and perhaps, infants who have lost a sibling to SIDS. However, the vast majority of infants succumbing to SIDS have no known predisposing causes. Risk Factors SIDS is more common in male than female infants. It is more common in the winter than the summer. It is more common in lower socioeconomic groups. Maternal risk factors include young age, inadequate 7
  • 8. prenatal care, lack of breast-feeding, and maternal substance abuse. SIDS is more common in infants whose mothers smoke either during or after the pregnancy. The greatest risk for SIDS occurs at the age of 2 to 3 months. Over 90% of SIDS deaths occur in infants 6 months or younger. Some post mortem studies suggest low oxygen levels and/or struggles to breathe. However, these findings are not universal. Studies have been unable to confirm a relationship between apnea and SIDS 30,31 and attempts to identify individuals who will die of SIDS have not been successful. However, infants who have experienced ALTE may have several differences from controls in terms of breathing response to hypoxia and hypercapnia 32 and arousal. 33 The most significant finding in recent years has been the observation that there may be a greater risk for SIDS in infants who sleep on their belly.34 In 1992 the American Academy of Pediatrics convened a task force to review the literature and make recommendations regarding positioning and SIDS. They recommended, “The weight of evidence implicates the prone position as a significant risk factor for SIDS.” 35 Since these recommendations, and with the start of the “Back to Sleep” campaign, there has been a considerable reduction in the rate of death from SIDS in the United States.36 In the prone position infants have increased sleep, increased quiet (non-REM) sleep and fewer, shorter awakenings.37 This may increase their risk for SIDS by decreased ability to respond to stimuli that should result in awakening. Oyen et al38 calculated the effects of various interventions on the rate of SIDS deaths (38). They estimated that 74% of SIDS death could be avoided if all infants slept on their backs. Eliminating maternal smoking would prevent 47% of SIDS death, while eliminating preterm birth or low birth weight would reduce the rate of SIDS by 16%. Apparent Life Threatening Event An ALTE is defined as an episode that is frightening to the observer and is characterized by some combination of apnea (central or occasionally obstructive), color change, marked change in muscle tone, choking, or gagging. In some cases, the observer fears that the infant has died.26 The most common 8
  • 9. identifiable cause of ALTE in one study was gastroesophageal reflux followed by central nervous system abnormalities, respiratory disease, metabolic disease, and cardiovascular disease.39 In over one third of the cases, the cause was not found. Unexplained ALTE used to be referred to as “near miss SIDS.” However, the terminology was changed since the link between ALTE and SIDS has not been proven. Evaluation of an infant presenting with an ALTE is dedicated to identifying a possible underlying cause (Table 4). If the cause of the ALTE is found, it should be treated. If the cause is unknown or can not be corrected, home cardiorespiratory monitoring may be indicated. A monitor does not prevent apnea or low heart rates; it simply alerts caregivers to the event. Therefore, it is crucial that all caregivers know how to respond to the monitor and assess the infant and are proficient in infant CPR. The decision to discontinue home monitoring is based on clinical parameters. Some experts recommend the monitor may be safely stopped when the infant “has had 2 to 3 months without significant numbers of alarms or episodes of apnea…additionally assessing the infant’s ability to tolerate stress (eg, immunizations, illness) during this time is advisable, requiring one or more two-channel basic sleep study (pneumogram) with normal findings before discontinuing the monitor may prolong needlessly the monitoring period.” 26 REFERENCES 1. Brouillette R, Hanson D, David R, et al. A diagnostic approach to suspected obstructive sleep apnea in children. J Pediatri 1984; 105:10-14. 2. Ali NJ, Pitston DJ, Stradling JR. Snoring, sleep disturbance and behavior in 4-5 year olds. Arch Dis Child 1993; 68:360-366. 3. Rhodes SK, Shimoda KC, Waid R, et al. Neurocognitive deficits in morbidly obese children with obstructive sleep apnea. J Pediatr 1995; 127:741-744. 9
  • 10. 4. Gozal D, Pope DW. Snoring during early childhood and academic performance at ages thirteen to fourteen years. Pediatrics 2001; 107:1394-1399 5. Carskadon MA, Pueschel SM, Millman RP. Sleep-disordered breathing and behavior in three risk groups: preliminary findings from parental reports. Childs Nerv Syst 1993; 9:452-457. 6. Brooks LJ. Genetic syndromes affecting breathing during sleep. In: Loughlin GM, Carroll JL, Marcus CL, eds. Sleep and breathing in children: a developmental approach. New York, NY: Marcel Dekker, 2000; 737-754. 7. Brooks LJ, Ward SLD, Hoppenbrouwers T, et al. Cardiorespiratory polygraphic monitoring during sleep. In: Stocks J, Sly PD, Tepper RS, et al, eds. Infant respiratory function testing. New York, NY: Wiley-Liss, 1996; 485-519. 8. Brooks, LJ, Stephens BM, Bacevice AM. Adenoid size is related to severity but not the number of episodes of obstructive apnea in children. J Pediatr 1998; 132:682-686. 9. Mallory GB, Fiser DH, Jackson R. Sleep-associated breathing disorders in morbidly obese children and adolescents. J Pediatr 1989; 115:892-897. 10. Marcus CL, Carroll JL, Koerner CB, et al. Determinants of growth in children with the obstructive sleep apnea syndrome. J Pediatr 1994; 125:556-562. 11. Leach J, Olson J, Hermann J, et al. Polysomnographic and clinical findings in children with obstructive sleep apnea. Arch Otolaryngol Head Neck Surg 1992; 188:741-744. 12. Carroll JL, McColley SA, Marcus Cl, et al. Inability of clinical history to distinguish primary snoring from obstructive sleep apnea in children. Chest 1995; 108:610-618. 13. Suen JS, Arnold JR, Brooks LJ. Adenotonsillectomy for treatment of obstructive sleep apnea in children. Arch Otolaryngol Head Neck Surg 1995; 121:525-530. 14. Wang RC, Elkins TP, Keech D, et al. Accuracy of clinical evaluation in pediatric obstructive sleep apnea. Otolaryngol Head Neck Surg 1998; 118:69-73. 15. Marcus CL, Keens TG, Davidson-Ward SL. Comparison of nap and overnight polysomnography in children. Pediatr Pulmonol 1992; 13:16-21. 16. Marcus CL, Omlin KJ, Bainki DJ, et al. Normal polysomnographic values for children and adolescents. Am Rev Respir Dis 1992; 146:1235-1239. 17. He J, Kryger MH, Zorick FJ, et al. Mortality and apnea index in obstructive sleep apnea. Chest 1988; 94:9-14. 10
  • 11. 18. Rosen CL, D’Andrea L, Haddad G. Adult criteria for obstructive sleep apnea do not identify serious airway obstruction in children. Am Rev Respir Dis 1992; 146:1231-1234. 19. McColley SA, April MM, Carroll JL, et al. Respiratory compromise after adenotonsillectomy in children with obstructive sleep apnea. Arch Otolaryngol Head Neck Surg 1992; 118:940-943. 20. Rosen GM, Muckle RP, Mahowald MW, et al. Postoperative respiratory compromise in children with obstructive sleep apnea syndrome: can it be anticipated? Pediatrics 1994; 93:784-768. 21. Goldstein NA, Sculerati N, Walsleben JA, et al. Clinical diagnosis of pediatric obstructive sleep apnea validated by polysomnography. Otolaryngol Head Neck Surg 1994; 111:611-617. 22. Jacob SV, Morielli A, Mograss MA, et al. Home testing for pediatric obstructive sleep apnea syndrome secondary to adenotonsillar hypertrophy. Pediatr Pulmonal 1995; 20:241-252. 23. Marcus CL, Ward SLD, Mallory GB, et al. Use of nasal continuous positive airway pressure as treatment of childhood obstructive sleep apnea. J Pediatr 1995; 127:88-94. 24. Guilleminult C, Korobkin R, Winkle R. A review of 50 children with obstructive sleep apnea syndrome. Lung 1981; 159:275-287. 25. Bull MJ, Givan DC, Sadove AM, et al. Improved outcome in Pierre Robin sequence: effect of multidisciplinary evaluation and management. Pediatrics 1990; 86:294-301. 26. National Institutes of Health Consensus Development Conference on Infantile Apnea. Pediatrics 1987; 79:292-299. 27. Guyer B, Hoyert DL, Martin JA, et al. Annual summary of vital statistics. Pediatrics 1999;104:1229-1246. 28. Panigraphy A, Filiano JJ, Sleeper LA, et al. Decreased kainate receptor binding in the arcuate nucleus of the sudden infant death syndrome. Neuropathol Exp Neurol 1997; 56:1253-1261. 29. Steinschneider A. Prolonged apnea and the sudden infant death syndrome. Pediatrics 1972; 50:646- 654. 30. Southall DP, Richards JM, deSwiet M, et al. Identification of infants destined to die unexpectedly during infancy. BMJ 1983; 286:1092-1096. 31. Southall DP, Richards JM, Stebbens V, et al. Cardiorespiratory function in 16 full term infants with sudden infant death syndrome. Pediatrics 1986; 78:787-796. 11
  • 12. 32. Hunt CE. Sudden infant death syndrome. In: Beckerman RC, Brouillette RT, Hunt CE, eds. Respiratory control disorders in infants and children. Baltimore, MD: Williams & Wilkins, 1992; 190-211. 33. McCulloch K, Brouillette R, Guzzetta AJ, et al. Arousal responses in near-miss sudden infant death syndrome and in normal infants. J Pediatr 1982; 101:911-917. 34. Dwyer T, Ponsonby AB, Newman NM, et al. Prospective cohort study of prone sleeping position and sudden infant death syndrome. Lancet 1991; 337:1244-1247. 35. AAP Task Force on Infant Positioning and SIDS. Pediatrics 1992; 89:1120-1126. 36. Task Force on Infant Positioning and Sudden Infant Death Syndrome (update). Pediatrics 1996; 98:1216-1218. 37. Kahn A, Groswasser J, Sottiaux M, et al. Prone or supine body positions and sleep characteristics in infants. Pediatrics 1993; 91:1112-1115. 38. Oyen P, Makstead T, Skjaerven R, et al. Combined effects of sleeping positions and prenatal risk factors in sudden infant death syndrome. Pediatrics 1997; 100:613-621. 39. Kahn A, Rebuffat E, Sottiaux M, et al. Management of an infant with an apparent life threatening event. Pediatrician 1988; 15:204-211. 12
  • 13. Table 1.— Sequelae of OSA Results of hypoxemia/hypercarbia (low oxygen/high carbon dioxide) Hypertension (high blood pressure) Cor pulmonale (right heart failure due to chronic respiratory disease) Failure to thrive Headaches Results of sleep fragmentation Sleepiness Poor school performance Irritability, behavior changes Table 2.— Symptoms of OSA Nocturnal (Nighttime) Diurnal (Daytime) Snoring Sleepiness Respiratory pauses Behavior problems Restless sleep Poor school performance Enuresis (bedwetting) Morning headaches 13
  • 14. Table 3.— Polysomnography Sleep staging EEG, Submental EMG Is adequate sleep and REM achieved to constitute a reliable test? How do cardiorespiratory events effect arousals and sleep architecture? Respiratory effort Chest wall and abdomen RIP or strain gauges Are respiratory events central, obstructive, or mixed? Do events produce arousal and/or oxyhemoglobin saturation? Gas Exchange Pulse oximeter, end-tidal CO2 Airflow Exhaled CO2, thermisters, and/or nasal pressure transducers Table 4.— Evaluation of ALTE History and physical examination Selective tests may include: -CBC (infection, anemia) -Electrolytes, NH3 (metabolic disorders) -Chest roentgenogram (infection, aspiration, heart size) 14
  • 15. -ECG (arrhythmia, RVH) -Physiologic monitoring during sleep 15
  • 16. 16