A huge tumour weighing 4.8 kg was removed from the jaw of a tribal youth from Jharkhand in a 14-hour-long complex surgery at Amrita Institute of Medical Sciences.
Afflicted with a rare genetic abnormality resulting in the development of the tumour, 19-year-old tribal boy Amar Samad from Jharkhand had been leading a secluded life for the last 10 years.
The tumour had started growing on Amar’s upper jaw in 2008, and it later protruded, making it difficult for him to speak and swallow, not to mention the social stigma.
Dr. Subramania Iyer, head, plastic and reconstructive surgery, said the boy had also been suffering from a parathyroid adenoma (a benign tumour of the parathyroid gland), which might have triggered the problem. The dense collection of bone and fibrous tissue was diagnosed as ‘ossifying fibroma’ on initial biopsy.
The rare medical condition called hyper-parathyroidism jaw tumour syndrome arises due to genetic abnormality, said Dr. Iyer.
Unique case
Amar’s case was unique as a tumour of that size associated with the disease had never been reported in medical literature. In fact, it was the largest ever reported upper jaw tumour, he added. The humongous growth had led to grave complications. If the condition had persisted, Amar would have found it impossible to eat, and breathing would have become a struggle. Also, the calcium levels in his body had risen quite high owing to the disease, Dr. Iyer said.
The removal of the tumour was complex owing to its size and the involvement of the entire upper jaw and the left eye. The amount of blood loss was a cause for worry, but it 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 tumour and conducted a 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. His nose was reconstructed with bone, with plastic tubes acting as airways,” Dr. Iyer said.
The patient also underwent the surgical removal of parathyroid adenoma. Post-surgery, 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 had to be readjusted to make it more acceptable cosmetically, though it has limited vision left.
Amar’s case was first noticed by Dr. Sreehari Jingla from Jharkhand. With help of a visiting US doctor, he brought the patient to Amrita Hospital.
Amar was emotional after the surgery. “I could never mingle with my friends as they would be repulsed by my appearance. It is a huge relief to get the tumour off my face. It is like a second birth. I am now eager to go back home, make friends again and work in the fields,” he said.