Sleep-disordered breathing and its management in children with achondroplasia

Sleep-disordered breathing and its management in children with achondroplasia

Tenconi R; Khirani S; Amaddeo A; Michot C; Baujat G; Couloigner V; De Sanctis L; James S; Zerah M; Cormier-Daire V; Fauroux B

American Journal of Medical Genetics Part A

2017

retrospective study; school child; preschool child; human; child; clinical article; surgery; prevalence; apnea hypopnea index; achondroplasia; adenotonsillectomy; neurosurgery; polysomnography; sleep disordered breathing; apnea monitoring; bone dysplasia; upper respiratory tract; breathing difficulties; surgical interventions; physical interventions; ENT surgery; CPAP; BiPAP; cervical decompression; obstructive sleep apnea

Sleep-disordered breathing is a common feature in children with achondroplasia. The aim of our study was to review the poly(somno)graphic (P(S)G) findings and consequent treatments in children with achondroplasia followed in the national reference center for skeletal dysplasia. A retrospective review of the clinical charts and P(S)G of 43 consecutive children (mean age 3.9 +/- 3.5 years) with achondroplasia seen over a period of 2 years was performed. Twenty four (59%) children had obstructive sleep apnea (OSA). Thirteen children had an obstructive apnea-hypopnea index (OAHI) < 5/hr, four had an OAHI between 5 and 10/hr, and seven had an OAHI > 10/hr. Ten of the 15 children who had previous upper airway surgery still had an abnormal P(S)G. All the patients with an AHI > 10/hr were under 7 years of age and none had a prior tonsillectomy. The children who underwent adeno-tonsillectomy, coupled in most cases with turbinectomy, were significantly older (mean age 7.5 +/- 3.5 vs. 3.5 +/- 1.7 years old, P = 0.015) and had significantly better P(S)G results than those who underwent only adeno-turbinectomy. No correlation was observed between the mean AHI value at the baseline P(S)G and the type of academic course (standard, supported or specialized). In conclusion, OSA is common in children with achondroplasia. The observation of a reduced prevalence of OSA after (adeno-)tonsillectomy is in favor of this type of surgery when possible. Copyright © 2017 Wiley Periodicals, Inc.

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