Obstructive sleep disorders in Prader-Willi syndrome: The role of surgery and growth hormone

Objective: To review the effectiveness and safety of surgical intervention for obstructive sleep apnea in Prader-Willi syndrome. Background: The muscle hypotonia and obesity associated with Prader-Willi syndrome (PWS) result in a high rate of obstructive sleep apnea (OSA). The use of growth hormone therapy in these patients has been associated with sudden death, raising concerns that such treatment may exacerbate obstructive sleep apnea. As a result, it has been suggested that children with PWS be evaluated for OSA and indications for adenotonsillectomy prior to instituting growth hormone therapy. The true effectiveness of surgical intervention in these cases, however, remains in doubt. Methods: Retrospective review of patients with a diagnosis of PWS who underwent adenoidectomy or adenotonsillectomy from January 2001 to July 2009 at a regional, tertiary care children's hospital. Patients underwent pre-operative and post-operative polysomnography. Differences between pre-operative and post-operative body-mass index (BMI), apnea-hypopnea index (AHI), and median oxygen saturation and oxygen saturation nadir were analyzed. Results: Five patients were identified during the study period. Three patients underwent adenotonsillectomy, 1 patient adenoidectomy alone, and another adenotonsillectomy with uvulopalatopharyngoplasty (UPPP). While median AHI was found to have decreased from 16.4 to 4.4, no statistically significant change could be demonstrated (p = 0.274). Mean O-2 and nadir O-2 saturation also improved, but without reaching statistical significance. No intra-operative complications were noted. Conclusions: Our series, and other small case series, have demonstrated that complete resolution of sleep apnea in PWS patients is difficult to obtain with upper airway surgery alone. It is suggested that children with PWS being considered for growth hormone therapy undergo assessment for OSA by polysomnography. Patients identified with OSA should be referred for management by tonsillectomy and/or continuous positive airway pressure (CPAP) and then reassessed for residual airway obstruction prior to instituting hormonal therapy. (C) 2010 Elsevier Ireland Ltd. All rights reserved.