Airway disorders in hemifacial microsomia

The anatomic components of hemifacial microsomia have been classified previously, but their relevance to functional abnormalities has not been stressed. In a recent review of the literature, we found that the frequency and severity of airway disorders, especially those leading to upper airway obstruction and/or obstructive sleep apnea, had not been reported. Accordingly, a retrospective study of 38 patients (21 male, 17 female), aged 6 months to 22 years (mean, 8 years 3 months) with hemifacial microsomia was undertaken to ascertain the frequency and severity of airway disorders in hemifacial microsomia. Upper airway disorders fell broadly within three categories: group I (n = 22, 58 percent) was asymptomatic for airway disturbances; group II (n = 7, 18 percent) had a medical history suspect for intermittent obstructive sleep apnea or had a perioperative apneic event; and group III (n = 9, 24 percent) had a definite history of obstructive sleep apnea or upper airway obstruction requiring tracheotomy or apnea surgery. Group III versus groups II and I had a higher incidence of bilateral involvement (33 percent versus 14 percent and none), a greater percentage of M2, M2a, M2b, and M3 mandibular deformities (88.9 percent versus 28.6 percent and 18.2 percent), more severe orbital involvement (33 percent O2 and O3 versus none in group II and 9 percent in group I), and more severe soft-tissue involvement (89 percent S2 and S3 versus 29 percent and 23 percent). Patients with more severe mandibular and orbital deformities, but not ear or vertebral abnormalities, appear at a greater risk for obstructive sleep apnea. The relationship of OMENS-Plus (extracraniofacial anomalies) to apnea was variable but was found more commonly in group II (86 percent) and group III (56 percent) than in group I (32 percent) patients. Group III patients had a higher frequency of cardiac anomalies (44 percent versus 29 percent in group II and 23 percent in group I). The incidence of obstructive sleep apnea in our population of patients with hemifacial microsomia approaches 24 percent. Patients with hemifacial microsomia should undergo routine screening for obstructive sleep apnea: a positive history warrants polysomnographic and anatomic workup.