Hemifacial Mircrosomia: A New Classification System?

Abstract

Objectives:

(1) Analyze associated findings in patients with hemifacial microsomia. (2) Determine if a new classification of hemifacial microsomia is possible.

Methods:

A retrospective chart of all patients diagnosed with hemifacial microsomia treated at Children’s Hospital of Minnesota. Each chart was reviewed for the presence of facial paralysis, microtia/anotia, aural atresia, mandibular hypoplasia, midface hypoplasia, dermoids, vertebral abnormalities, ocular abnormalities, sensorineural hearing loss, laterality, renal abnormalities, developmental delay, and skin tags.

Results:

A total of 75 patients with hemifacial microsomia were included in this study. There were 43 males and 32 females with an average age of 8.51 ± 5.45 years at the time of chart review. Occurrences for each defect were: facial paresis/paralysis (26.7%), microtia/anotia (73.3%), mandibular hypoplasia (72%), midface hypoplasia (57.3%), vertebral deformities (21.3%), ocular abnormalities (24%), dermoids (21.3%), renal abnormalities (12%), SNHL (17.3%), skin tags (42.7%), bilateral and developmental delay (24%). Regression models were used that showed the odds of having a spinal defect are 3.13 (P = .001) times higher for each additional craniofacial defect present; furthermore if a patient has 2 or more craniofacial defects, the odds of having spinal defect were 31.6 (P = .001) times as high as in patients with 1 or fewer. The odds of developing a renal defect were 36.0 (P = .001) times as high as in patients with 1 or fewer craniofacial defects.

Conclusions:

The number of craniofacial defects, not just type or severity, increase the risk of having extracranial abnormalities in patients with hemifacial microsomia.