Abstract

Objective: To evaluate the prevalence of obstructive sleep apnea in a large population of children with achondroplasia and to evaluate the effectiveness of adenoidectomy and/or tonsillectomy as treatment. Methods: Retrospective review of 95 children with achondroplasia. Results: Thirty –six patients (38%) had clinical evidence of obstructive sleep apnea. Thirty-four patients underwent surgery, with more than 1 procedure required in 10 children (29%). Adenotonsillectomy was the initial procedure for 22 of 34 patients, and further therapy was required in only 18% of this group. Adenoidectomy was the initial procedure for 10 of 34, with 90% requiring further surgery for recurrent obstructive sleep apnea. Tonsillectomy alone was performed in 2 patients: 1 was effectively treated and 1 later required adenoidectomy. Endotracheal intubation was accomplished in all patients without complication; 53% required a smaller endotracheal tube than would be predicted by their age. Eight postoperative complications were recorded. Conclusions: Obstructive sleep apnea is very common in children with achondroplasia. Surgery is effective, but recurrent symptoms are common, particularly when the initial procedure is adenoidectomy. The complication rate is higher than that observed in a general pediatric population but is readily managed with standard therapy. Anesthesia can be given safely to these patients with special consideration for limited neck extension and appropriate endotracheal tube size. (Otolaryngol Head Neck Surg 1999;120:248-54.)

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