Outcomes of Adenotonsillectomy in Patients With Prader-Willi Syndrome | Congenital Defects | JAMA Otolaryngology–Head & Neck Surgery | JAMA Network
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Original Article
Nov 2012

Outcomes of Adenotonsillectomy in Patients With Prader-Willi Syndrome

Author Affiliations

Author Affiliations: Divisions of Endocrinology (Drs Meyer, Repaske, and Zipf), Pulmonary Medicine (Dr Splaingard), and Molecular and Human Genetics (Dr Atkins), and Department of Otolaryngology (Dr Jatana), Nationwide Children's Hospital, Columbus, Ohio; and Departments of Pediatrics (Drs Splaingard, Repaske, and Atkins) and Otolaryngology–Head and Neck Surgery (Dr Jatana), Wexner Medical Center, The Ohio State University, Columbus, Ohio.

Arch Otolaryngol Head Neck Surg. 2012;138(11):1047-1051. doi:10.1001/2013.jamaoto.64

Objective To assess the efficacy of upper airway surgical intervention in patients with Prader-Willi syndrome (PWS). Due to reports of sudden death in children undergoing treatment with growth hormone for PWS, detection of sleep-disordered breathing by polysomnography (PSG) has been recommended.

Design Retrospective study.

Setting Multidisciplinary PWS Center at a tertiary care children's hospital.

Patients Thirteen pediatric patients with PWS who underwent adenotonsillectomy (T&A) with pre-PSG and post-PSG.

Main Outcome Measures Comparison of PSG results before and after T&A.

Results Six of our patients were girls (46%); 8 had genetic characteristics consistent with deletion (61%), and the remaining 5 had genetic characteristics consistent with uniparental disomy (39%). The median age at T&A was 3 years (age range, 6 months to 11 years), and the median age at start of growth hormone treatment was 8.5 months (range, 2 months to 6 years). Nine of the 13 patients had mild to moderate obstructive sleep apnea (OSA) or obstructive hypoventilation (69%); in 8 of these 9, breathing normalized after T&A. Four children had severe OSA prior to surgery (31%). Breathing normalized in 2 of these after surgery, but 2 had PSG findings of residual combined obstructive and central apneas postoperatively.

Conclusions Adenotonsillectomy, while effective in most children with PWS who demonstrate mild to moderate OSA, may not be curative in children with severe OSA. An increase in central apneas can occur in some children with PWS postoperatively, and it is important to repeat PSG after surgery. Further studies are necessary to determine optimal treatment for some children with PWS and sleep-disordered breathing.