High quality sleep is essential to a child’s growth and development. Obstructive sleep apnea (OSA) occurs when the muscles in the airway relax during sleep, causing a narrowing or closure of the airway, with temporary interruptions of breathing and sleep disruption. It can be either partial or full obstruction of the airway, and it leads to poor sleep quality. In Prader‑Willi syndrome (PWS), OSA is common, occurring in an average of 80% of individuals compared to only 1‑3% in the general population. OSA can have many far‑reaching consequences if not treated properly, including problems with lung function, cognition, emotion, and behavior.Adenotonsillectomy (an operation to remove both the adenoids and the tonsils) is one of the most common first treatments for OSA, and it eliminates or significantly reduces OSA in more than half of cases. However, the outcomes and potential complications after OSA surgery are not well understood in PWS, as some specific PWS features might lead to worse outcomes and potentially more complications.
Therefore, a group of researchers from Johns Hopkins University in Baltimore, MD, decided to perform a systematic review to look at all the published medical literature on OSA and adenotonsillectomy in people with PWS, to look at the complications that happened after surgery, the effectiveness of the surgery, and quality of life changes post‑surgery. The researchers found seven studies to include in this “meta‑analysis,” or review of published studies on the subject. Their findings have been published and summarized below.
Overall, all the included studies showed that people with PWS are at a higher risk for complications after adenotonsillectomy, and these patients should be monitored closely after surgery. The most common complication was velopharyngeal insufficiency, where the soft palate doesn’t close against the back of the throat, leading to air coming out of the nose during speech.
Looking at an outcome of the surgery, AHI (Apnea–Hypopnea Index, an index to look at the severity of sleep apnea), there was variable improvement. Before surgery, the mean AHI was 10.7, and after surgery, the mean AHI was 3.8, which reflects an important reduction in apnea events. Overall in these 7 studies, 67% of people improved from severe/moderate OSA to mild/resolved OSA after surgery. Furthermore, only 20% had completely resolved OSA after surgery. The overall conclusion is that patients with PWS generally benefitted from the surgery, but they need to be evaluated for residual OSA after their surgery. Two of the seven studies in this meta‑analysis looked at quality of life post‑surgery, and both found improvement after surgery based on parent feedback.
Overall, adenotonsillectomy seems to result in substantial improvement in OSA and quality of life, but may not completely resolve OSA in people with PWS. However, people with PWS have a higher risk of complications post‑surgery, including velopharyngeal insufficiency, and should be monitored closely after surgery for any acute complications. People with PWS should have their sleep symptoms and AHI re‑evaluated after adenotonsillectomy, as there could still be some residual OSA after surgery.