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Houston Cranio Facial

What Is Parry-Romberg Syndrome?

Parry–Romberg syndrome, or progressive hemifacial atrophy, is a disease characterized by localized loss of subcutaneous tissue on one side of the face and skull. Or in other words, the shrinking and degeneration of the soft and bony tissues under the skin. This is a rare neurocutaneous syndrome with an unknown etiology or cause. Typically, this affects females more commonly than males in a 3-2 ratio with usual beginning stages at around the teenage years of life, although it can also start much earlier. Some suggest this is a localized scleroderma (connective tissue disease) or autoimmune-type acquired disease, therefore it is not a genetic disease.

Associated signs and symptoms involve the eye as well as neurological problems. Some patients are affected with trigeminal neuralgia (extreme pain caused by facial movement) and/or migraines with nausea, vomiting, and visual abnormalities. Patients may develop enophthalmos (sunken eyeball), drooping of the eyelid, or paralyzed eye muscles. Exam by an ophthalmologist is necessary to make sure no eye problems need to be addressed. Some patients may develop seizures in addition to migraines, requiring evaluation by a neurologist.

Typical surgical treatment involves fat transfer or fat grafting to restore some contour to the face and reestablish normal soft tissue. However, some patients need greater volume restoration, requiring facial reconstructive procedures such as free tissue transfer with a parascapular flap (which comes from the upper back/shoulder blade area) or an anterior lateral thigh flap. This flap can involve a thin layer of muscle and fat with or without skin used to recontour the face. These procedures require microscopic surgery to reconnect blood vessels such as the artery and vein in the face. Bone grafts may be necessary in certain cases. Timing of surgical intervention is best achieved when atrophy no longer is progressing.