Romberg’s hemifacial atrophy

Romberg’s hemifacial atrophy is an acquired condition characterised by the deterioration of the skin and soft tissue of one half of the face (usually the left side).

More common in females than males, this deterioration causes initial changes in the tissues above the upper jaw, or between the nose and the upper corner of the lip. It then progresses to the mouth, the area surrounding the eye, the brow, the ear and the neck. It may also affect the tongue, the soft palate, and the gums. The eye and cheek of the affected side may become sunken, and facial hair may turn white and fall out.

This condition may also be accompanied by neurological abnormalities including seizures and episodes of severe facial pain. The onset of the disease usually begins between the ages of five and 15 years, and the progression of the atrophy often lasts from two to ten years, after which the process enters a stable phase. Muscles in the face may atrophy and there may be bone loss in the facial bones. Problems with the retina and optic nerve may occur when the disease surrounds the eye.

What surgery is available, and what techniques are involved?

There are no treatments that can stop the progression of Romberg’s hemifacial atrophy, although reconstructive surgery may be needed to repair wasted tissue. Generally, surgeons wait until the disease has run its course before they operate; they also tend to wait until a patient’s facial growth is complete. Most surgeons will recommend a waiting period of one or two years before proceeding with reconstruction, and will use lipofilling, muscle or bone grafts to help repair the affected area
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