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.