19th century original observations…

1. Snoring and Obstructive Sleep Apnea Syndromein Children2013Gerald M. LoughlinWeill Cornell Medical CollegeKomansky Center for Child HealthNew York Presbyterian Hospital

2. 19th century original observations… “At night the child’s sleep is greatly disturbed; the respirations are loud and snorting, and there are sometimes prolonged pauses, followed by deep, noisy inspirations.” William Osler, 1892

4. Obstructive Sleep Apnea Syndrome • A spectrum of abnormal breathing during sleep, that in its mild form is manifested by snoring ( partial airway obstruction) with or without gas exchange abnormality and in its extreme form by snoring with intermittent complete airway obstruction (apnea) • Associated findings include increased respiratory effort on inspiration, snoring punctuated with periods of silence with continued respiratory efforts, resulting in hypoxia, hypercapnia and disruption of normal sleep patterns. • Daytime symptoms occur as a consequence of the abnormal breathing and/or sleep patterns.

5. Pathophysiology of Obstructive Sleep Apnea Increased Upper Airway Resistance Alterations in upper airway control during sleep OSAS Tonsils & adenoids Obesity Craniofacial abnormalities Airway size Allergy Inflammation Other factors: Age, Gender Behavior Environment Socioeconomic status Arousal Genetics & Race Passive smoking • Effects of age, • CNS dysfunction • (Primary vs. acquired) • Brainstem compression/injury • Respiratory depressants

6. Prevalence of OSAS in Children 10% - 12% of children snore loudly, nightly 1-3% of children with OSAS

7. Signs and Symptoms (sleep) • Snoring • During sleep - paradoxical inward rib cage motion – increased work of breathing • Apnea – obstructive and central • Disturbed sleep (movement arousals, restlessness) • Night sweats • Cyanosis (not often reported) • (?) Enuresis • ? Increased GER/aspiration

8. Associated Findings – awake • Mouth-breathing, hypo-nasal speech, chronic nasal congestion • Recurrent adenotonsillitis • Excessive daytime sleepiness (unusual) • Irritability on awakening • Morning headaches (?) • Behavioral and neurocognitive dysfunction

9. Childhood OSAS - complications • Cardiovascular • cor pulmonale, pulmonary hypertension • polycythemia • systemic diastolic hypertension • altered cardiac function during sleep • Failure to thrive – • increased caloric expenditure • decreased IGF-1 and IGFBP-3 levels • Neurocognitive dysfunction • Developmental delay • Death – uncommon in children

10. 19th century description of daytime symptoms... “The expression is dull, heavy, and apathetic… In long-standing cases the child is very stupid-looking, responds slowly to questions, and may be sullen and cross.”“Among other symptoms may be mentioned headache, which is by no means uncommon, general listlessness, and an indisposition for physical or mental exertion. The influence upon the mental development is striking.” William Osler, 1892

11. Patterns of Neuro-cognitive Dysfunction • Infancy - Developmental delay • Pre-school Chronic oppositional behavior - “difficult child” Easily fatigued, “always tired” Lethargy / sleepiness or hyperactivity • School age Abnormal shyness, social withdrawal Hyperactivity/ aggressiveness / attention problems Unexplained poor school performance Decreased executive functions, visual attention, conceptual ability and phonologic functioning

12. Patterns of Neuro-cognitive Dysfunction • School Age (continued) • Decreased executive functions, visual attention, conceptual ability and phonologic functioning • Intelligence - • Memory

13. Neurocognitive Behavioral Deficits Children and Adolescents • Decreased intelligence, memory, attention capacity • Decreased academic performance • Increased problematic behavior • Reports of social withdrawal, emotional lability , hyperactivity, conduct problems, aggressive behavior • Delinquency , destructive and disruptive behavior

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17. The association between sleep disordered breathing, academic grades, and cognitive and behavioral functioning among overweight subjects during middle to late childhood. Beebe DW et al. Sleep 2010;33:1447-1456 Study of 163 overweight adolescents divided based on AHI into 4 groups -Moderate/Severe OSA vs. mild vs. snorers without apnea vs. non snorers Measurements – PSG, neuropsych testing, parent and teacher reports of grades, sleep , behavior Findings: SDB in overwgt adolescents 10-16yrs associated with lower grades and worse behavior: Data suggests that alterations in academic performance arise from negative behaviors

18. How to make a diagnosis of OSAS? • Direct observation – good but hard to asses severity and to arrange • Polysomnographt is the “gold standard.” Is it needed in all patients? • AAP (2002) recommends testing for OSA before T&A • - although an appropriate recommendation • is not always practical or possible • Value of oximetry, video recordings, nap vs. overnight study

19. When is intervention indicated? • In 2013 – Snoring can no longer be considered normal. It is equivalent to stridor and wheeze, as a sign of airway obstruction • Need to be certain that what is being described is actually snoring • Therapy indicated in snoring child who presents with typical symptoms/complications of OSAS • Medical management ( including weight loss) can be considered for mild to moderate cases • T&A – most common surgical option • Results post T&A are inconsistent – abnormal sleep study may persist in as many as 40%

20. Treatment of obstructive SDB in children Surgical treatment for OSAS • Adenotonsillectomy (? Role for recently described intracapsular procedure) • Uvulopalatopharyngoplasty (UPPP) – not for children • Tracheostomy (rarely used now for OSAS) • Craniofacial reconstruction • Miscellaneous (brainstem decompression surgery in achondroplasia and Chiari malformation)

22. Non surgical Interventions • Rx of upper respiratory allergies/inflammation • Leukotriene antagonists • Nasal steroids • Weight loss for obese children • Nasal airway (short term) • Oral appliances (?) • Nasal strips (?) • “Follow up essential regardless treatment plan”

23. Long-term Implications of Childhood OSAS • May predict who is at risk as adults • “As the twig is bent, the tree inclines” • If untreated - may have profound effects on neurocognitive and cardiovascular function in adults In adults • Risk factor for hypertension • ? sudden death during sleep • ? myocardial infarction, ? Stroke

25. Allergy/Immunology – upper airway inflammation Anesthesiology – pre and intra-operative airway management Cardiology – cor pulmonale, ventricular dysfunction, hypertension Critical Care – post–op management (pulmonary edema, airway obstruction) Developmental and General Pediatrics – developmental delay, FTT, enuresis Endocrine – obesity, growth problems, puberty Gastroenterology –possible increase in GER Genetics – increased risk in African –Americans, gender issues Hematology – differential diagnosis ofpolycythemia Infectious Disease – recurrent adenotonsillitis Neonatology – increased risk in former premature infants Nephrology – hypertension, enuresis Neurology – neuro-cognitive problems, school problems Neuro-radiology – functional MRI Neurosurgery – brainstem compression syndromes Otolaryngology – most common indication for T&A Orthopediatics – fractures from falling out of bed Outcomes and Health Services Research – data needed on natural history, approach to diagnosis and therapy, who to treat and how Pulmonary – abnormal respiration and gas exchange Sleep Medicine – perhaps most common & severe sleep disorder in children Urology – enuresis

26. Suggested Reading Marcus Cl. Sleep-disordered Breathing in Children. Am J Resp Crit Care Med 2001; 164:16-30 Clinical Practice Guideline: Diagnosis and Management of Childhood Obstructive Sleep Apnea Syndrome. 2002; 109 704-712. Beebe DW, et.al. Theassociation between sleep disordered breathing, academic grades,and cognitive and behavioral functioning among overweight subjects during middle to late childhood. Sleep 2010;33:1447-1456. Redline S Amin R et.al. The Childhood Adenotonsillectomy Trial (CHAT): Rationale, Design, and Challenges of a Randomized Controlled Trial Evaluating a Standard Surgical Procedure in a Pediatric Population. Sleep 2011; 34: 1509-1517. .

27. Macey PM, et.al. Brain Morphology Associated with Obstructive Sleep Apnea. AJRRCM 166: 1382, 2002 Weissbluth M, et.al. Signs of airway obstruction during sleep and behavioral, developmental and academic problems. J Dev Behav Pediatr 1983; 4:119-121. Urschitz MS, et. al. Snoring, intermittent hypoxia and academic performance in primary school children. Pediatrics 2004; 114:1041-1048. Gozal D, Pope D. Sleep disordered breathing and school performance in children. Pediatrics 1998; 102: 616-620. Ali NJ, et al. Snoring, sleep disturbance and behavior in 4-5 year olds. Arch Dis Child 1993;68:360-68.

28. Neuropsychological and psychological function in children with a history of snoring or behavioral sleep problems. S. Blunden et al. J Pediatr 146:780-786, 2005. Bonuck K, et.al. Sleep-disordered breathing in a population-based cohort: Behavioral Outcomes at 4 and 7 years. Pediatrics 2102; 129:1-9. Redline S, et.al.The Childhood Adenotonsillectomy Trial (CHAT): Arationale design, and challenges of a Randomized Controlled Trial Eva;uating a Standard Surgical Procedure in a Pediatric Population. Sleep 2011; 34:1509-1517. Dillon JE, et.al. DSM-IV Diagnoses and Obstructive Sleep Apnea in Children Before and 1 year after Adenotonsillectomy J Am Acad Child Adolesc Psychiatry 2007; 46: 1425-1436.