Pediatric OSA. Pediatric OSA: Treatment Options Beyond AT. Copyright (c) 2012 Boston Children's Hospital 1

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Pediatric OSA Treatments Options Beyond AT Report of Financial Relationships (past 12 months) with commercial entities producing, marketing, re selling, or distributing health care goods or services consumed by, or used on, patients relevant to the content I am presenting None Eliot Katz Boston Children s Hospital Harvard Medical School What is the threshold level of OSA requiring treatment in children? Question? It depends on Severity AHI, arousal index, SpO2, other? Symptoms Cognitive, EDS, mouth breathing Signs Nasal, craniofacial, lymphoid Natural history Age, underlying medical condition Trait susceptibility Inflammatory, CV, metabolic, EDS Associated with neurocognitive impairment independent of apnea or hypoxemia Guilleminault et al, CHEST 2004;126: 1396 Treatment Nasal steroids/ltx antagonists, AT, adjuvant surgeries, CPAP, trach Primary Snoring (PS): versus Controls Answer Snoring or 1 event/hour Associated with neurocognitive impairment independent of apnea or hypoxemia Urschlitz et al, AJRCCM 2003; 168: 464- Urschlitz et al, Ped 2004; 114: 1041- Blunden et al, J Clin Exp Neuro psychol 2000; 22: 554- O Brien et al, Peds 2004; 114: 44- Goodwin et al, SLEEP 2003; 26: 587- Gottlieb et al, J Peds 2004; 145: 430- Guilleminault et al, Arch Ped Adol Med 2004; 158:153- Jackman et al, Sleep Med 2012; 13: 621- Miano et al, Clin Neurophys 2010; 122; 311- Giordani et al, J Int Neuropsych Soc 2012; 18: 212- Blunden et al, 2000 J Clin Exp Neuropsych 22;554 Def n [thermistor, n = 7, referred] PS impaired attention/memory/intelligence Gottleib et al, 2004 J Peds 145; 458 Def n [thermistor, n=48, population based} PS impaired attention/memory/iq Bourke et al, 2011 Sleep Med 12; 222 [nasal pressure, n=59, referred] PS impaired attention/behavior/executive function Copyright (c) 2012 Boston Children's Hospital 1

Washtenaw County Adenotonsillectomy Cohort Baseline (5 12 yo) Apnea Index Sleep Subjective Sleepiness Giordani et al, J Int Neuropsych Soc 2012; 18: 212- Neuropsychological MSLT Behavior Attention Cognition Executive Dysfunction Washtenaw County Adenotonsillectomy Cohort Follow up at 1 year: Apnea Index Giordani et al, J Int Neuropsych Soc 2012; 18: 212- Sleep Variables Subjective Sleepiness MSLT OSA (n=40) 5.6 49% 14.7 ( ) OSA (n=40) 5.6 0.1 49% rare 15.6 17.5 Snoring (n=38) 0.2 27% 15.9 ( ) Snoring (n=38) 0.2 0.1 27% rare 15.9 17.3 Surgical Controls (n=27) 0.1 Rare 17.4 Surgical Controls (n=27) 0.1 Rare 17.4 17.3 Improvement over time. No Differences at Follow-up vs. Controls Primary Snoring (PS): versus Controls Elevated Blood Pressure Li et al, J Peds 2009; 155: 362 Endothelial Dysfunction Li et al, Int J Card 2012 Primary snoring is harmful to some children Upper airway resistance syndrome Guilleminault et al, 1982 25 snoring children; normal AHI, Po 2, Pco 2 ; Pes Symptoms Night Snoring, Sweating, hyperextended Restless, Enuresis Daytime Guilleminault et al, 1996 EDS, ADHD, Aggressive, Learning disabilities, Clumsiness 10 year retrospective, children <12 years 259 UARS, 83 OSAS, Pes crucial Pediatric OSA: Treatment Adenotonsillectomy: AHI Medical Combination of nasal steroid/leukotriene antagonist Weight loss CPAP Nasopharyngeal tube/prone positioning for infants Oxygen Dental Rapid maxillary expansion Mandibular advancement devices Surgical Adenoidectomy/tonsillectomy Turbinectomy Mandibular distraction Mid-facial advancement Tracheostomy 207 children with OSA Follow up PSG at 4 months post AT All children had improved AHI/O 2 Saturation 46% had follow up AHI >1 110 children with OSA? Follow up PSG 46% 1<AHI<5 29% AHI >5 Guilleminault et al, Oto HNS 2007; 136: 169- Tauman et al, J Peds 2006; 149: 803- Many children have residual OSA after AT Especially, Obese, Severe OSA, & <3 yo Copyright (c) 2012 Boston Children's Hospital 2

Recurrent OSA: Lingual tonsils Normal Enlarged lingual Infant OSA: CPAP Down s syndrome (17%) Obesity Dx with MRI Other: glossoptosis, hypopharyngeal collapse Resmed Infant Bubble Mask Respironics Small Child Profile Lite SleepNet Mini-Me Donnelly et al, Sem Ultra CT MRI 2010, 31: 107- CPAP CPAP: Improves Sleep and Behavioral Sx Also improved CBCL, OSA-18 Marcus et al, AJRCCM 2012; 185: 998 CPAP: Improves Sleep and Behavioral Sx CPAP: Adherence CPAP usage related to ESS Marcus et al, AJRCCM 2012; 185: 998 1/3 dropped out; 5.3 hours/night in remainder Parents over-estimate CPAP usage Marcus et al, Peds 2006; 117: e442 Copyright (c) 2012 Boston Children's Hospital 3

Long Face Syndrome Montelukast: Randomized, double blind for 6 weeks Features Vertical height Gummy smile High arched palate Steep mandibular plane Disuse atrophy of nose Etiology Nasal obstruction affects craniofacial morphology in genetically susceptible children Adenoid decreased by 25% Goldbart et al, Peds 2012; 130: e575 Intranasal Steroids: Randomized, double blind for 6 weeks Montelukast & Intranasal Steroids: Observational, non randomized for 3 mo Baseline after AT 3 months later Montelukast/ 3.9 ± 1.2 0.3 ± 0.3 Budesonide Control 3.6 ± 1.4 4.7 ± 1.5 AHI 3.7 to 1.3, no change in Control group Adenoid decreased Kheirndish Gozal et al, Peds 2008; 122: e149 Kheirndish et al, Peds 2006; 117: e61 Weight Loss: Medical Therapy Weight Loss: Medical Therapy 61 adolescents, 14.8 ± 2.3 years Mild OSA 48%, Moderate/severe OSA 13% About 1/3 previously had an adenoidectomy Admission to impatient weight loss program for 5.2 ± 0.5 months 1,400 1,600 Calories/day Exercise 10 hours/week BMI average 37.5 ± 5.7 at baseline Average weight loss 35.8% of BMI Verhulst et al, Obesity 2009; 17: 1178 62% of Patients had a resolution of OSA Verhulst et al, Obesity 2009; 17: 1178 Copyright (c) 2012 Boston Children's Hospital 4

Weight Loss: Bariatric surgery Maxillary Constriction Normal Normal High-arched Narrow maxilla 90% of Patients had a resolution of OSA Kalra et al, Obesity Res 2005; 13: 1175 Rapid Maxillary Expansion Rapid Maxillary Expansion: Children Before After 31 children (8.7 yo) with OSA and PBC AHI average 12.2 RME for 3 weeks All had AHI < 1 at 4 months Pirelli et al, Prog Ortho 2005; 6: 48 Pirelli et al, SLEEP 2004; 27: 761- Rapid Maxillary Expansion: Young adults Supraglottoplasty 10 young adults (ave 27 yo) with OSA, PBC, & EDS RME for 3 6 months (6/10 surgically assisted, ie >25 yo) AHI 19 7 9/10 EDS resolved Before After Cistulli et al, SLEEP 1998; 21: 831- Copyright (c) 2012 Boston Children's Hospital 5

Supraglottoplasty Mandibular Distraction Before After 3 hour operation Usually extubated 24 hours Distracted 0.5 4 mm/day Total distraction about 22 mm, until slight overcorrection Device removed after 3 mos Can be done at birth Avoids tracheostomy O Connor et al, Int J Ped Oto 2009; 73: 1216- Rachmiel et al, Int J Oral Maxillofac Surg 2005; 34: 9- Mandibular Distraction Pre-operative 1 month Post-op 12 months Post-op Rachmiel et al, Int J Oral Maxillofac Surg 2005; 34: 9- Copyright (c) 2012 Boston Children's Hospital 6