Neurofibroma – Fibrous Dysplasia Debulking Surgery

Patient diagnosed with fibrous dysplasia through genetic testing

The patient is a 26-year-old man from New Delhi in India. He had been fine up until age 12 when he started developing right sided facial asymmetry. This had progressively gotten worse with gross deformity of the face. His parents had taken him to a leading facial plastic surgeon.

Extensive testing had been performed on the patient. Genetic testing had been ordered with results positive for fibrous dysplasia with associated neurofibroma. Implications of the diagnosis had been explained to his parents in detail. It was explained that the only treatment was surgical management.

Symptomatology and implications of fibrous dysplasia

Fibrous dysplasia is a rare genetic disorder. It is abnormal growth of fibrous tissue that replaces normal bone tissue. This results in asymmetrical enlargement of the affected region, which leads to gross deformity. This condition needs facial plastic surgery for reestablishment of esthetics.

There can be spontaneous remission of the condition. Fibrous dysplasia of the face leads to gross disfigurement. Surgical intervention is the only available treatment for management of fibrous dysplasia. Constant medical surveillance is advised in order to ensure that it does not affect any vital functions.

Patient undergoes surgical intervention of his condition

The patient has undergone two surgeries thus far for treatment of his condition. Both surgeries had been performed in New Delhi. Unfortunately, the results had been catastrophic for the patient. It had led to a dramatic decrease in the quality of his life.

Incisions had been poorly designed in the first surgery. This had lead to unsightly scar formation. Excess fibrous tissue had been poorly contoured leading to worsening of his facial asymmetry. The patient had undergone a second surgery a few years later, which did not lead to any significant improvement.

The patient has been depressed for a few years now over his condition. Seeing this, his parents had made enquiries about the best facial cosmetic surgeon in India. They had finally decided to come to our hospital for surgical management of his condition. Board certified plastic surgeons also perform this surgery in the European countries.

Initial examination at our hospital along with treatment planning

Dr SM Balaji, facial deformity surgeon, examined the patient and ordered for radiological studies including a 3D CT scan. This revealed overgrowth of fibrous tissue that had replaced bone on the right side of the facial skeleton. The patient had extreme facial asymmetry because of this. This was diagnosed as neurofibroma with fibrous dysplasia.

The surgical procedure was explained to the patient and his parents in detail. They were in agreement with the treatment plan and consented to undergo surgery for facial asymmetry correction.

Successful surgical removal of excess tissue for improving esthetics

The patient was administered anesthesia through bronchoscopic intubation. A nasolabial incision was then made to expose the fibrous tissue. Excess tissue was removed using a mallet and burr through the stamp cut technique. Once adequate reduction had been obtained, the incision was closed with sutures.

Attention was next turned to the right lower border of the mandible. An incision was made through a preexisting scar from a previous surgery. The lower border of the mandible was trimmed until it was symmetrical with the left side of the mandible.

The incision was closed in layers once adequate reduction had been obtained. The patient and his parents were very happy with the results of the surgery. They stated that the patient was very happy with the facial form after surgery. They expressed their total satisfaction before final discharge from the hospital.

Surgery Video