Clinical presentation of hemifacial microsomia in a South African population.

Abstract

Background: Hemifacial microsomia (HFM) presentation includes gross distorted ramus, malposition temporomandibular joint, small glenoid fossa, distorted condyle and notch, malformed orbit, cupping ear or absent external ear, and facial nerve palsy. HFM is the second most prevalent congenital deformity of the face, with little literature from the South African population. This retrospective study elucidated the demographic characteristics and clinical presentations of HFM patients in a select South African population and compared it to the literature.  Methods: A retrospective study of HFM patients diagnosed through clinical presentation and confirmed by plain radiograph or computed tomography was conducted. The patient's charts were reviewed for age, sex, laterality, side, the severity of the deformity, and associated craniofacial and extra-craniofacial anomalies. The clinical presentation of malformations was categorised according to the OMENS classification, using five major craniofacial manifestations of HFM.

Results: Twenty-five patients were included, with a male-to-female ratio of 1:1.78. The population distribution is 60% Black, 32% Indian, 4% White and 4% Coloured. A right-to-left laterality ratio of 1.4:1 and 4% bilateral affectation. This study showed 100% mandibular hypoplasia, 84% ear deformity, 40% orbital deformity, 60% facial nerve defect and 100% soft tissue defect affectation with noticeable facial asymmetry. Other craniofacial anomalies were recorded in 84%, while extracraniofacial anomalies were recorded in 40% of this HFM population.

Conclusion: There is a high degree of variability in the deformities in HFM in the South African population, distinguishing it from the international population. A multidisciplinary approach is required for its treatment and management.