OBSTRUCTIVE SLEEP-APNEA SYNDROME IN A FAMILY WITH CROUZONS-SYNDROME

Craniofacial anomalies are recognized causes of obstructive sleep apne a syndrome (OSAS) in children. Current literature is limited due to ra rity of cases. Furthermore, the mechanism of upper airway obstruction is not clearly understood. We would like to report a family (father an d 2 sons) who are suffering from Crouzon's syndrome. The two brothers (ages 1 and 3) were found to have significant obstructive sleep apnea syndrome (OSAS) with failure to thrive. Nasal continuous positive airw ay pressure (CPAP) markedly improved their OSAS and resulted in accele rated weight gain. The nasoendoscopy and magnetic resonance imaging (M RI) scan taken during natural sleep showed that choanal stenosis, maxi llary hypoplasia, posteriorly displaced tongue, lengthened soft palate and adenoid tissues were important in the pathogenesis of upper airwa ys obstruction in Crouzon's syndrome. Nasal CPAP improved airway obstr uction by opening a narrow slit as demonstrated by MRI. Our results su ggest that OSAS occurred in children with Crouzon's syndrome and that nasal CPAP was a useful treatment modality.