A comparison of two classification systems for hemifacial microsomia.
The classification of hemifacial microsomia (HFM) aids in diagnosis, treatment planning, prognostic predictions and data evaluation. The aetiological and phenotypic heterogeneities of HFM, however, make its classification problematic. This study used data from 50 patients to examine the classification of HFM and to compare two systems: OMENS and SAT. The results were concordant with current literature and demonstrated the phenotypic heterogeneity of HFM. Essentially, both classifications embody the major craniofacial defects, but the OMENS system appears to be further refined by its differentiation between soft tissue and nerve defects, and between orbital and mandibular defects. Neither system, however, records deafness or grades auricular tags, although tags occurred in 34% of cases and two patients with otherwise 'normal' ears had tags. Therefore, it is suggested that auricular tags be graded as minor ear malformations. Furthermore, the OMENS system could be strengthened by the addition of an asterisk to the acronym in cases with serious non-craniofacial anomalies, for example OMENS*. This adds little complexity to the acronym, but immediately indicates when a patient's features lie towards the more generalised oculoauriculovertebral end of the phenotypic spectrum.
Cousley
R R
RR
School of Clinical Dentistry, Queen's University of Belfast, Northern Ireland.
D000015:Abnormalities, Multiple / Q000145:classification / Q000175:diagnosis*; D003391:Cranial Nerves / Q000002:abnormalities; D004431:Ear, External / Q000002:abnormalities; D005145:Face / Q000002:abnormalities; D005146:Facial Asymmetry / Q000145:classification* / Q000150:complications / Q000473:pathology; D006311:Hearing Disorders / Q000151:congenital / Q000209:etiology; D006801:Humans; D008334:Mandible / Q000002:abnormalities; D009915:Orbit / Q000002:abnormalities