Drug-induced sleep endoscopy in children with Prader-Willi syndrome

Abstract

In our study, partial or complete anterior-posterior tongue base collapse was associated with higher AHI values in children with PWS. Therefore, careful attention should be addressed to the management of tongue base collapse. Positional therapy could be a potential treatment for patients with PWS since it may alleviate the severity of tongue base collapse.

An updated review of pediatric drug‐induced sleep endoscopy

“…The trachea may also be assessed. Levels of obstruction are recorded as “none,” “partial,” or “complete.” A summary of the sites of obstruction most commonly reported in children undergoing DISE for OSA after T&A are documented in Table …”

Sleep Disorders in Childhood Neurogenetic Disorders

“…Notably, 65–75% of cases are caused by deletion of this region on the paternally-inherited chromosome. Furthermore, 20–30% of cases are caused by maternal uniparental disomy, 1–3% of cases are caused by a methylation and imprinting defect, and less than 1% of cases due to paternal translocation [ 34 , 35 ]. More recent data suggests that contribution of paternal deletion may be decreasing and maternal disomy may be increasing due to increased maternal age at conception [ 36 ].…”

“…Though much of the evidence is mixed regarding this association, most of the studies have included both adult and pediatric subjects [ 36 ]. Evaluation of airway obstruction with endoscopy in nine children with PWS and OSA revealed multi-level upper airway obstruction [ 35 ]. One study of 30 children (mean age seven years old) with PWS found an association between obesity and severity of obstructive apnea-hypopnea index [ 40 ], but several smaller studies did not identify this association [ 35 ].…”

“…Evaluation of airway obstruction with endoscopy in nine children with PWS and OSA revealed multi-level upper airway obstruction [ 35 ]. One study of 30 children (mean age seven years old) with PWS found an association between obesity and severity of obstructive apnea-hypopnea index [ 40 ], but several smaller studies did not identify this association [ 35 ]. Due to the increased risk for sudden death in infants and young children less than three years of age with PWS polysomnography is recommended in infancy, both prior to growth hormone treatment and monitoring during growth hormone therapy [ 37 , 38 , 49 ].…”

“…Children with PWS may be at increased risk for post-operative complications following adenotonsillectomy and should be monitored closely following surgery. Additionally, post-operative sleep study should be obtained [ 35 , 38 , 43 , 50 ]. Findings on airway evaluation that were associated with incomplete resolution of OSA following adenotonsillectomy include lingual tonsillar hypertrophy, tongue base obstruction, and airway hypotonia leading to laryngomalacia and/or pharyngomalacia [ 35 ].…”