Hemifacial Atrophy

Understanding Hemifacial Atrophy (Romberg Syndrome)

Hemifacial atrophy, also known as Romberg Syndrome, is a rare condition characterized by the progressive shrinking of the skin and soft tissues on one-half of the face. This condition results in a sunken appearance and typically affects only one side of the face, though occasionally, it may also impact the limbs on the same side. Hemifacial atrophy develops slowly and is considered a rare disorder.

Onset of Romberg Syndrome

Romberg syndrome usually becomes noticeable during the first decade of life or early in the second decade, with most individuals experiencing symptoms before the age of 20. The progression of the disease can continue for many years and then stabilize, with the severity varying among affected individuals.

Causes of Hemifacial Atrophy

The exact cause of Hemifacial Atrophy is unknown, and it appears to occur randomly in individuals. However, several factors have been suggested as possible contributors to the condition, including:

• Abnormal development or inflammation in the nervous system.
• Viral infections.
• Trauma.
• Abnormalities in blood vessel formation.
• Autoimmune factors.

Symptoms of Hemifacial Atrophy

The symptoms of Hemifacial Atrophy can vary in severity and may include:

• Progressive tissue wasting on one side of the face.
• A distinct demarcation line resembling a linear scar between normal and affected skin is known as the “coup de Sabre” sign.
• Shrinkage and atrophy of tissues beneath the skin, including the fat layer, underlying muscles, and bones.
• A skeleton-like appearance on the affected side of the face.
• Dark pigmentation of the skin.
• Atrophy of half of the upper lip and tongue.
• Deviation of the jaws when opening the mouth.
• Delayed eruption of teeth, malocclusion, and posterior open bite.
• Migraine headaches are accompanied by visual symptoms, light sensitivity, nausea, and vomiting.
• Epilepsy attacks and spasms of the jaws.
• Drooping of the upper eyelid and difficulty closing the eyes.

Diagnosis of Hemifacial Atrophy

Hemifacial Atrophy is typically diagnosed based on characteristic features and physical examination. The following diagnostic criteria are often considered:

• The presence of a white line or furrow on one side of the face is known as the “coup de Sabre” sign.
• Facial atrophy on the affected side.
• In some cases, an MRI of the brain may be performed if there are fits/epilepsy or other neurological symptoms.
• An eye examination should be conducted to rule out any abnormalities.
• Skin biopsy may be performed from the area of the white line if present.

Treatment of Hemifacial Atrophy

There is no medical treatment or medication available to halt or reverse the progression of Hemifacial Atrophy. Surgical intervention is the primary option for addressing this condition.

Surgical Options for Hemifacial Atrophy

Three types of surgical procedures can be performed for Hemifacial Atrophy, depending on the extent and severity of the condition:

Fat Grafting:

This technique is suitable for treating mild and smaller asymmetries of the face caused by Hemifacial Atrophy.

• a) Fat grafting can be done under local anesthesia or general anesthesia depending upon your choice and comfort level.
• b) In the first step, liposuction is done to harvest healthy fat from another area of the body (usually the belly, thighs, or buttocks).
• c) This fat is then purified, processed, and prepared for grafting.
• d) A fine needle and cannula are used to inject the healthy fat into those areas of the face that have signs of deformity.
• e) To account for the body’s natural reabsorption of the injected fat, overcorrection is done (more fat than required is injected).

The face is gently massaged to ensure the fat is evenly distributed, addressing your initial goals for the procedure

Fat grafting done in stages gives good and acceptable results in severe deformities of hemifacial atrophy. But the procedure has to be repeated 2 to 3 times, 3 to 6 months apart to match with the opposite face. Fat grafting is the best solution if cosmesis is the main concern with no extra scar elsewhere on the body.

Microvascular Free Flaps: In cases where the skin is too tight and shrunken for fat grafting to be effective, microvascular free tissue transfer is considered. This procedure involves harvesting a flap of tissue, usually from the back of the thigh, along with its artery and vein. The flap is then transferred to the face, reshaped, and secured in place. Blood vessels are connected to vessels at the recipient site.

Bony Corrections: Correction of bone deformities can be achieved through osteotomies (cutting and repositioning of bones). For instance, if the orbit is distorted, vertical repositioning of the orbit or placement of a bone graft on the orbital floor may be performed.

Preferred Technique by Dr. Amit Agarwal

Dr. Amit Agarwal typically prefers fat grafting as the primary technique for correcting Hemifacial Atrophy. This method is favored for its cosmetically pleasing results and avoids the scarring associated with flap surgery. However, flap microsurgical reconstruction is reserved for cases of severe deformities where patients are not suitable candidates for multiple staged fat grafting or have experienced failure with fat grafting.

Recovery After Fat Grafting Surgery

After fat grafting surgery for Hemifacial Atrophy, patients can expect:

• Immediate post-operative swelling, which can last for 3 to 6 days.
• Some discomfort.
• Head elevation for 2 to 3 weeks to reduce swelling.
• Walking on the same day as surgery.
• Discharge on the same day or the next day.
• A few days off from daily routines for rest.
• Post-operative care includes avoiding physical and emotional strain, maintaining head elevation, and refraining from heavy physical activity.
• Use of ice fomentation if swelling persists.
• The gradual return of sensation as swelling subsides.

Risks and Complications

Potential risks and complications associated with fat grafting for Hemifacial Atrophy may include:

• Persistent swelling and bruising.
• Infection or delayed wound healing, especially in individuals with certain medical conditions.
• Skin redness and tenderness.
• Irregular contours or lumps.
• Asymmetry due to variable fat absorption.
• Temporary numbness or altered sensation.

Consultation with Dr. Amit Agarwal

For individuals with Hemifacial Atrophy seeking treatment, consultation with Dr. Amit Agarwal is essential. The choice of treatment technique and approach will be determined based on the specific characteristics and severity of the condition, ensuring the best possible outcome for the patient.