Adenotonsillectomy for Obstructive Sleep Apnea in Children with Prader‐Willi Syndrome

Abstract

Summary. The aim of our study was to evaluate the efficacy of adenotonsillectomy for the treatment of obstructive sleep apnea syndrome (OSA) in pediatric patients with Prader-Willi syndrome (PWS), and to describe the postoperative complications. Five patients (4 males; median age, 4.4 years; range, 1.6-14.2 years) were studied. All patients underwent an overnight cardiorespiratory sleep study. All patients had adenotonsillar hypertrophy (ATH), and two were also obese. The preoperative obstructive apnea/hypopnea index (AHI; median and range) was 12.2 (9.0-19.9) events/hr; the mean oxygen saturation was 95 (79-96)%; the nadir oxygen saturation was 71 (58-78)%; and the oxygen desaturation index (ODI) was 15.8 (11.4-35.9) events/hr. Preoperatively, patients were classified as having moderate to severe OSA. A second sleep study, performed 16 (3-43) months after adenotonsillectomy, showed a significant decrease in AHI (P ¼ 0.009) and ODI (P ¼ 0.009). Mean and nadir oxygen saturation did not differ significantly postsurgery (P ¼ 0.188, P ¼ 0.073, respectively). Four out of five children showed at least one postoperative complication. Difficult awakening from anesthesia, hemorrhages, and respiratory complications requiring reintubation and/or supplemental oxygen administration were observed. In conclusion, patients with PWS and OSA who underwent adenotonsillectomy showed a significant decrease in AHI and number of oxygen desaturations. Pediatr Pulmonol. 2006; 41:74-79.

Outcomes of Adenotonsillectomy in Patients With Prader-Willi Syndrome

“…In addition, they found that hypercapnic ventilator responses in patients with PWS showed lower increments of ventilation than was found among their peers, thus supporting the previous claim of abnormal peripheral chemoreceptor function in PWS. 18,24 Our study complements the findings of Pavone et al 25 in their review of 5 children with PWS who underwent T&A. In that report, 1 child who initially had moderate OSA normalized after surgery; 3 patients with severe OSA and 1 patient with moderate OSA by AHI score improved to mild OSA.…”

“…Our study also adds useful information about a younger age group. Pavone et al 25 studied children with a me- dian age of 4.5 years (youngest age, 1.9 years). The patients in our study had a median age of 3 years, and the youngest patient was only 6 months old.…”

The Importance and Place of Adenotonsillectomy in Syndromic Children

“…It was seen that the children with Prader-Willi syndrome that have hypotonia and obesity along with facial anatomical abnormalities benefited from adenotonsillectomy in the presence of adenotonsillar hypertrophy; but postoperative complications increased in patients especially under 3 years of age (10). As in our patient, improvement can be achieved especially in swallowing in the presence of adenotonsillar hypertrophy through adenotonsillectomy in children with a disease such as cerebral palsy, which has an abnormal motor tone.…”

Review of 64 cases of death in children with Prader–Willi syndrome (PWS)

“…This emphasizes that PWS is per se a condition with a high mortality risk which requires careful clinical management with or without GH treatment. We do not advise adenoidectomy and tonsillectomy on every child with PWS, as firstly the surgery may be dangerous [Pavone et al, 2006] and when needed, it must be performed with cardiac and respiratory monitoring for a 24-hr period. Furthermore treatment could worsen speech problems (personal experience) and it sometimes has no effect on sleep obstructive apnea [Festen et al, 2006].…”