Hemi facial atrophy, otherwise known as Romberg’s disease, is a disorder of unknown etiology. It is characterized by progressive wasting of skin, subcutaneous fat, cartilage, bone, muscle and hair. It was when first reported by Romberg in 1846.
The most common early sign is a painless cleft, the “coup de sabre”, near the midline of the face or forehead. A bluish hue may appear in the skin overlying atrophic fat.
The disease occurs more frequently in women and appears in the first or second decades of life. It progresses over a time course of between two and ten years, and atrophy appears to follow the distribution of one or more divisions of the trigeminal nerve.
When associated with inflammatory changes of the skin in other areas of the body, it is often diagnosed as scleroderma, and in reality they may be manifestations of the same disease.
Neurological abnormalities are common. Roughly 45% of people with Romberg syndrome are also afflicted with trigeminal neuralgia including the forehead, eye, cheek, nose, mouth and jaw and migraine.
Diagnosis can be made solely on the basis of history and physical examination in people who present with only facial asymmetry. For those who report neurological symptoms such as migraine or seizures, MRI scan of the brain is the imaging modality of choice. A diagnostic lumbar puncture and serum test for auto antibodies may also be indicated in people who present with a seizure disorder of recent onset
Management of hemi facial atrophy comprises of a long-term follow-up of somatic disorders, and prevention of psychological problems. Treatment is symptomatic and mainly consists of plastic surgery after the disease activity has stopped.
A variety of treatment techniques have been employed in an attempt to improve the atrophic deformity. These have included free fat grafts, dermis fat grafts, fascia, cartilage and bone for augmentation purposes.
(A case of hemifacial atrophy affecting an young female is shown here and the manifestations of this uncommon disorder is discussed. The various methods employed in correcting the facial deformity including rhinoplasty is being discussed.)