A 19-year-old tribal boy Amar Samad, hailing from Konaroa village in Bano Block of Simdega district in Jharkhand, got afflicted with a rare genetic abnormality that resulted in a jaw tumor so large, it has not been reported in medical literature before. A few days ago a team of 12 surgeons at Kochi’s Amrita Institute of Medical Sciences (Amrita Hospital) removed the external deformity in a surgery lasting 14 hours. The hospital conducted the surgery free of cost considering the poor socio-economic background of the patient.
A football-size mass of matter, weighing 4.8 kg and measuring 20 x 20 x 20 cm, started growing on Amar’s upper jaw in 2008 and protruded out to the left. The dense collection of bone and fibrous tissue was diagnosed as “ossifying fibroma” on initial biopsy examination. This led not only to a grotesque appearance, but also difficulty in swallowing and speaking, in addition to social stigma that left the patient home-bound. He also suffered from a parathyroid adenoma (a benign tumor of the parathyroid gland) which might have initiated the problem.
Dr. Subramania Iyer, Head, Plastic & Reconstructive Surgery, Amrita Institute of Medical Sciences (Amrita Hospital) said: “Amar’s medical condition belonged to a very rare disease group called Hyper-parathyroidism Jaw Tumor Syndrome, which arises due to a genetic abnormality and causes small-size tumors on the jaw. His case is unique as a tumor of this size associated with this disease has not yet been reported in medical literature. In fact, this is the largest ever reported upper jaw tumor of its kind. The humongous growth was leading towards grave complications. If the condition had persisted, Amar would have found it impossible to eat, and breathing would have become a struggle. The calcium levels in his body had risen very high due to the disease.”
The removal of the tumor as well as reconstruction of the upper jaw was a great challenge. Added Dr. Subramania Iyer: “The tumor’s removal was complicated by its huge size and the involvement of the entire upper jaw and the left eye. The amount of blood loss was a cause of worry, but this was controlled by temporarily blocking blood vessels to the upper part of the face. Reconstruction had to be meticulously planned. We used 3D printing to construct an accurate model of Amar’s face and tumor, and conducted mock surgery for practice. Micro-surgical transfer of his leg bone was carried out to construct a new upper jaw. We had initially planned to remove his left eye, but managed to save it through meticulous surgery. His nose was reconstructed with bone, with plastic tubes acting as airways.”
The patient also underwent surgical removal of the parathyroid adenoma. After the surgical procedures, Amar’s parathyroid hormone and calcium levels have returned to normal. He now needs implants placed in his reconstructed upper jaw to act as teeth. The position of his left eye has to be readjusted to make it more acceptable cosmetically, though it has limited vision left. Both the eye and dental procedures will be conducted after six months.
Amar has two younger brothers in the family. His father passed away due to malaria in his childhood, and his mother left soon thereafter, leaving the children to be raised by their uncle. Amar used to work in the fields, but since his medical condition developed, he rarely ventured out of home and led an isolated life.
Amar’s case came to the notice of Dr. Sreehari Jingla from Jharkhand. Moved by his plight, and with the help of a visiting US doctor who knew about the head-and-neck reconstructive service available at the Amrita Hospital, he facilitated Amar’s treatment after being turned away from many reputed hospitals as the doctors were not confident they could handle the case.
After the successfully surgery, patient Amar Samad said: “Because of this huge deformity on my face, I could never mingle with other children of my age, as they used to be repulsed by my appearance. It is a huge relief to get the tumor off my face – it is almost like a second birth. I am now eager to go back home, make friends again and work in the fields. I thank the doctors of Amrita Hospital from the bottom of my heart for enabling me to lead a normal life.”
