Severity
The clinical presentation of hemifacial microsomia(HFM) can be quite variable. In some children, just the ear is affected(microtia or a small, underdeveloped ear), and in other children, multiple parts of the face and body can be affected. While there is no grading scale that is in use universally, the OMENS scale (standing for Orbital, Mandible, Ear, Nerves and Soft tissue)was developed to help describe the heterogeneous phenotype that makes up this malformation. Children with HFM are at higher risk for hearing loss, and should have their hearing tested. A proportion of children with HFM will have extra-cranial anomalies, including abnormalities of the kidneys and cervical spine, which are important to detect early in childhood. Cognition and development is usually typical in children with HFM.
Some have hypothesized that the severity of hemifacial microsomia depends on the extent of the physical injury (the area with a short supply of blood) and the gestational age of the fetus – the earlier the injury, the greater the chance of wide-scale problems. This has not yet been proven in an animal or human model.
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