Management of sleep-disordered breathing in children with achondroplasia

Abstract

Achondroplastic children are at risk for sleep-disordered breathing. The aim of our study was to review the poly(somno)graphic (P(S)G) findings and treatments in achondroplastic children. A review of the clinical charts and P(S)G data of children referred to our center was performed. Forty-four patients (age 3.9±3.5 years) were included in the study. Fifteen (34%) children had upper airway surgery and 6 (14%) had a craniocervical decompression surgery before the P(S)G. Twenty-five (57%) patients had an abnormal P(S)G defined by an apnea-hypopnea index (AHI) >1.5 events/h with 13 (30%), 4 (9%), and 8 (18%) patients having an AHI between 1.5 and 5 events/h, between 5 and 10 events/h, and > 10 events/h, respectively. Twenty-four patients had obstructive sleep apnea (OSA) with the only patient presenting central sleep apnea having also a Down syndrome. The children who underwent prior adeno-tonsillectomy (± turbinectomy) were significantly older (age 3.5±1.7 vs. 7.5±3.7 years, p=.015) and had significantly better P(S)G results than those who had only an adeno-turbinectomy. The following treatments were performed in the 24 patients with OSA: 14 with mild OSA were included in a watchful waiting program, 6 underwent ENT surgery, 3 were treated with NIV, 1 had ENT surgery and NIV. Follow up P(S)Gs on 13 patients showed: spontaneous improvement in 4 patients, persistence of mild OSA in 5 patients during watchful waiting, and cure of OSA in 4 patients who underwent ENT surgery. Nocturnal gas exchanges improved immediately in the 4 patients treated by NIV. In conclusion, OSA is common in achondroplasia but significantly less common after tonsillectomy. Systematic and repeated P(S)G should be included in routine care.