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What is PARRY-ROMBERG SYNDROME?

Parry-Romberg Syndrome is an acquired condition in which the skin, soft tissue, cartilage, and underlying bone begin to atrophy. In most cases it affects only one side of the face and is more common in females than males. It can appear in infancy or adolescence. Facial atrophy is progressive and courses along the distribution of one or more branches of the fifth cranial nerve. There is variability in severity of tissues affected and distribution, ranging from mild to severe. The classic symptom is a "coup de sabre" sign, or a linear depression of the forehead, also known as linear scleroderma.

Symptoms SYMPTOMS

Symptoms of Parry-Romberg Syndrome include atrophy of tissue in any part of the face. It usually starts as a small change and may progress to severe shrinking of the soft tissues with color changes in the skin. Any part of the face can be affected and symptoms can get worse over time. The tongue, roof of the mouth and gums may also be affected, and in some children the eyes or cheeks may appear sunken. Facial hair may turn white and fall out. The skin can also become darkly pigmented or show patches of un-pigmented skin. Patients who have Parry-Romberg Syndrome may also have neurological conditions, including seizures.

Children are usually diagnosed with Parry-Romberg Syndrome between ages 5 to 15 years. There is no clear cause for this syndrome. The skin and soft tissue atrophy can progress over 5 - 10 years or more, at which point this usually stops, called the "burnout period".

Treatment TREATMENT & MANAGEMENT

Parry-Romberg Syndrome cannot be treated with medications, and surgery is the best option. For mild cases, fat can be transferred from a different part of the body to the affected area, known as fat grafting. When there is bony loss, various bone substitute materials, implants, or bone grafting may be considered to improve the skeletal structure and contour. In severe cases, more complex procedures to move freely move a piece of tissue from a different area of the body to the affected area requiring reconnecting small blood vessels together known as microsurgery may be required. It is best to perform definitive reconstruction after the disease has stabilized. Reconstruction requires the expertise of a plastic surgeon/craniofacial surgeon who has expertise in bone and soft tissue.