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Adenotonsillectomy failed to benefit all children with Prader-Willi syndrome
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Adenotonsillectomy is effective in the treatment of children with Prader-Willi syndrome who have mild-to-moderate apnea-hypopnea index, but the same cannot be said for patients with severe obstructive sleep apnea.
Stacy L. Meyer, MD, endocrinology fellow at Nationwide Children’s Hospital in Columbus, Ohio, and colleagues evaluated the medical records of 13 patients (46% female) diagnosed with Prader-Willi syndrome who also underwent an adenotonsillectomy (aged 6 to 11 years) and had pre- and post-polysomnography.
Stacey L. Meyer
“Although adenotonsillectomy can sometimes be effective in treating sleep apnea in Prader-Willi syndrome, caution should be exercised especially in those with severe obstructive sleep apnea since they may continue to have sleep-disordered breathing, even after surgical intervention. Careful monitoring of these children postoperatively with polysomnography and collaboration between endocrinologist, otolaryngologist and sleep medicine is warranted to optimize outcomes,” Meyer told Endocrine Today.
Of the 13 patients’ records studied, 61% had genetics consistent with deletion and 39% had uniparental disomy as the mode of inheritance, the researchers wrote. Patients were aged 2 months to 6 years at the start of growth hormone treatment. Nine of 13 (69%) patients had mild-to-moderate obstructive sleep apnea or obstructive hypoventilation, and 89% corrected after adenotonsillectomy.
“We found that adenotonsillectomy may not always be curative in some children with Prader-Willi syndrome and obstructive sleep apnea. Some children continue to have some degree of obstructive apnea postoperatively, and a couple of children had evidence of an altered control of breathing with increased number of central apnea and periodic breathing episodes postoperatively,” Meyer said.
Four patients (31%) had severe obstructive sleep apnea before surgery, two regulated after surgery, one patient (aged 9 months) did not normalize until age 3 years, and another patient (aged 24 months) was placed on supplemental oxygen to resolve her central apnea incidents, the researchers wrote.
Meyer said an expansion of the study to a larger population of Prader-Willi syndrome patients is necessary to identify specific predictors, including greater obesity, age and other endocrine or physical abnormalities to optimize the outcome of Prader-Willi syndrome patients with obstructive sleep apnea.
For more information:
- Meyer SL. Abstract #1513.227. Presented at: Pediatric Academic Societies Annual Meeting 2012; April 28-May 1; Boston.
Disclosure: The researchers report no relevant financial disclosures.