Hemifacial microsomia has several names Including craniofacial microsomia and Goldenhar's syndrome, or even first and second brachial arch syndrome. The syndrome includes a combination of mandibular, cheekbone, ear, mouth, eye, and skull hypoplasia. In some cases, fatty deposits called epibulbar dermoids are present on the eyes, and there may be fusion of the bones in the neck.

A crooked mouth, or asymmetry in the corners of the mouth, may be seen as a result of mandibular hypoplasia. The affected half of the mandible may not grow at the same rate as the normal half. A deviated chin can also be seen.

Microtia, or inadequate development of the ear on the affected side may also be seen. The degree of involvement is variable. In mild forms the ear may simply be slightly prominent or cupped, and in more severe cases, the ear may only be a small remnant of skin and cartilage. Reconstruction of the ear can be done with many different techniques, but we favor an autologous reconstruction for many reasons. We do perform a variety of techniques, and the plan is designed for each individual patient. A complete discussion of these issues can be seen on the Microtia Page where a paper from The Journal of Craniofacial Surgery can be downloaded and printed.

Of paramount importance in these children is the choreography of the various operations. This is done to minimize the number of operations and the number of times that the child would need anesthesia.

The Pruzansky classification system is used most frequently for mandibular hypoplasia. Grade 1 is normal in shape, but reduced in size. Grade 2 demonstrates hypoplasia as well as maldevelopment of the associated condyle and coronoid processes. In the Kaban modification, Grade 2a mandibles are hypoplastic and thecondyles are malformed, but the condylar head/glenoid fossa relationship is spatially maintained. In these patients the misshapen condyle is functional and can be used in the mandibular reconstruction. Grade 2b mandibles have a hypoplastic and malformed condyle, which is displaced outside of the sagittal plane of the contralateral temporomandibular joint. Grade 3 indicates severe hypoplasia and lack a condyle, coronoid process and glenoid fossa. The treatment plan designed for these children depends on the age of the patient and the degree of skeletal hypoplasia. Most frequently, Distraction Osteogenesis or bone grafting are utilized to reconstruct the mandible.

When there is very significant hypoplasia of the facial bones, patients may develop airway obstruction called sleep apnea. Untreated, this can lead to poor weight gain, small stature, cerebral hypoxia, and even death. For such patients, sleep studies can be obtained at the Children's Hospital. If there is evidence of sleep apnea is discovered, then appropriate steps are immediately taken to insure the childs safety and optimize future development.