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ROMBERG DISEASE

Romberg disease also known as progressive hemi facial atrophy (PHA) is slow progressive atrophy of skin and soft tissue of one side of the face. It affects the children and young adults and the condition is more common in females than males. It usually starts from the cheek and naso-labial area then progresses to involve other structures. Skin over cheek looks hyper pigmented and depressed.
The onset is usually at ages of 5 and 15 years. The progression of the atrophy often lasts from 2 to 10 years, and then the process seems to enter 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. The exact cause of this disease is unknown.
Treatment starts after the disease becomes stable.
Small areas of skin and soft tissue involvement can be corrected with injecting patients own fat to the affected area. This can be done as day care under local anesthesia or sedation. Fat is injected more than required because some of the fat dissolves with time. Initially there may be swelling or a feeling of tightness that will go away in a few days.
Larger defects require free tissue transfer and scapular and parascapular flaps from the back of the patient are flaps of choice.
If this disease starts in early years of life it tends to be more severe and can involve bones around the eye and bones of jaws. Corrective surgeries for these bony deformities are required.

FAQ
1. What is cause of Romberg disease?
The exact cause is unknown. No hereditary or genetic link is found. Patients will frequently remember an “initiating event” in PHA and the onset of the disorder is often linked to an episode of trauma or infection.
2. Does Romberg disease run in family?
No, there is no such association found.
3. Does it affect brain?
Nervous system involvement is rare but has been reported in literature leading to fits.
4. Is it a treatable condition?
Disease itself is self-limiting but treatment is required for resultant deformities which is done after disease entered in stable phase

 

Written by : Dr. Fahmina
Copyrights: Pakistan association of Plastic Surgeons