Adenotonsillectomy success for treating obstructive sleep apnea in children with Prader-Willi syndrome.

BACKGROUND

Prader-Willi syndrome (PWS) is a rare genetic disorder that can increase risk of pediatric obstructive sleep apnea (OSA), caused by the combination of increased viscosity of secretions, craniofacial abnormalities, hypotonia, and obesity. While first-line treatment of pediatric OSA is typically adenotonsillectomy, the complex pathophysiology of OSA in PWS patients may lead to less success with this therapy.

METHODS

The TriNetX database was queried for patients 18 years old or younger based on the diagnoses of PWS and OSA and the surgical interventions of adenotonsillectomy, tonsillectomy, and adenoidectomy. The primary endpoint was the removal of the diagnosis of OSA 6 months postoperatively. Pediatric patients without PWS were used as a control. Secondary endpoints were the risk of OSA with common medical interventions for children with PWS.

RESULTS

A total of 2163 patients were found to have PWS, with 1035 (47 %) diagnosed with OSA. PWS patients undergoing surgery had a total success rate of 39.0 %, compared to 79.6 % in controls (p < 0.001). Total success for these surgeries was also significantly lower compared to controls matched by demographics and obesity status (36.8 % versus 82.1 %, p < 0.001). Use of growth hormone (RR 1.43, p < 0.001) and testosterone (RR 1.39, p < 0.001) were both associated with increased risk of OSA.

CONCLUSIONS

Adenotonsillectomy has significantly lower rates of success at treating pediatric OSA in patients with PWS. These patients would likely benefit from multidisciplinary care to treat their OSA and mitigate the effects of untreated disease, and further studies determining best practices for caring for these patients are necessary.