Gradual development of facial deformity since young
The patient is a 19-year-old boy from Karaikudi in Tamil Nadu, India. His parents first noticed the development of a facial deformity when he was around 4-5 years old. This had slowly progressed to the point where there was gross deformity of the right side of his face. He had slowly lost confidence with the passage of time and became depressed because of this.
They had presented to a local surgeon when he was 14 years of age. A detailed history was obtained from the patient and he was subsequently diagnosed with neurofibromatosis of the right side of the face with a sagging lip.
Difficulties arising from the long standing neurofibromatosis
The excessive growth has caused a great deal of hardship for the patient, resulting in difficulty with eating and speech. The patient had already lost his right eye sight due to this condition.
Having confirmed the diagnosis of neurofibromatosis, the surgeon had advised to remove the excessive tissue surgically. The patient and his parents had consented to surgery and the patient underwent surgery.
However, the overgrowth recurred shortly and worsened considerably over the years. It has now reached the point where it has become a hindrance to the patient’s activities of daily living. They then made extensive inquiries regarding the best hospital to address this problem and had been referred to us.
Patient and his parents presented at our hospital
Dr SM Balaji, neurofibroma surgeon examined the patient and ordered for imaging studies including a 3D CT scan. Examination revealed that tumor grown was extensive and had caused the right side of his face to sag downwards.
The right lateral border of the lip was also sagging downwards. His eye was also infected and appeared larger in size. There were also numerous Café au lait spots seen all over the patient’s body. The patient manifested all the signs and symptoms of neurofibromatosis.
Premier treatment center for facial deformity surgery
Our hospital is a premier center for facial deformity surgery in India. Both soft tissue deformities and bony deformities are addressed at our hospital. Many patients with debilitating facial deformities have undergone surgery at our hospital, helping them to lead relatively normal lives.
Dr SM Balaji has extensive experience of over 25 years as a cosmetic surgeon and this facet has immensely helped to rehabilitate patients with gross facial deformities. Many patients have had their lives restored to complete normalcy after undergoing cosmetic facial surgery at our hospital.
Etiology and pathogenesis of neurofibromatosis
The patient has been diagnosed with plexiform neurofibromatosis. This condition causes tumors to develop in the nervous system. It can be subdivided into neurofibromatosis type I (NF1), neurofibromatosis type II (NF2) and schwannomatosis.
The first is manifested as light brown spots on the skin along with freckles in the armpit and groin. There are also small bumps within the nerves and scoliosis. Second is manifested by hearing loss, cataracts at a young age and balance problems. There are also flesh-colored skin flaps and muscle wasting.
Plexiform neurofibroma are larger, more extensive tumors that grow from nerves anywhere in the body. Unlike cutaneous neurofibroma, plexiform neurofibroma are often found in young children. They can even manifest around the time of birth.
This condition is caused by a genetic mutation in certain genes. They can be inherited from parents or can develop spontaneously during early development. The tumors affect the supporting cells of the nervous system and not the neurons.
It is very rare that cancerous tumors arise from this condition and only an extremely small percentage of the general population is afflicted by this condition.
There is no known prevention or cure. Surgery may be done to remove tumors that are causing esthetic problems or have turned cancerous.
Treatment planning explained to patient and parents in detail
The surgical procedure was explained in detail to the patient and his parents. It was explained that the overgrowth of his plexiform neurofibroma would be excised. Excision was planned very carefully keeping in mind not to cause permanent facial disfigurement. The patient and his parents gave their consent for the surgery.
Surgical excision of neurofibromatosis growth
An elliptical incision was made on the right side of the face and excess neurofibroma tissue was excised. A Weir excision was also done on the right nostril to create symmetry of the nose. Lip level was also adjusted and all incisions were closed with non resorbable sutures.
Successful surgical outcome of the surgery
The patient and his parents expressed their happiness to Dr SM Balaji. An immediate result of the surgery was the improved ability to eat and easier speech. His facial profile was also greatly improved and they were happy with the outcome of the surgery.
