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Mumbai: 17-year-old boy undergoes surgery on elephant legs caused by Noonan syndrome

Dr Nilesh Satbhai, Consultant Plastic, and Reconstructive Microsurgeon said, “The patient came to the hospital with a history of swelling in both lower limbs and groin, and was bed-ridden. We diagnosed him with a primary lymphoedema, commonly known as elephantiasis.”

Swapnil Mishra | Updated on: Tuesday, April 19, 2022, 10:48 PM IST

Mumbai: 17-year-old boy undergoes surgery on elephant legs caused by Noonan syndrome  | FPJ
Mumbai: 17-year-old boy undergoes surgery on elephant legs caused by Noonan syndrome | FPJ
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A 17-year-old boy, diagnosed with elephantiasis-leg as huge as elephant legs because of a rare syndrome called Noonan syndrome, underwent 6-hour long surgery. The boy was bedridden for a while.

Dr Nilesh Satbhai, Consultant Plastic, and Reconstructive Microsurgeon said, “The patient came to the hospital with a history of swelling in both lower limbs and groin, and was bed-ridden. We diagnosed him with a primary lymphoedema, commonly known as elephantiasis.”

He said primary lymphedema is rare, affecting 1 in 100,000 individuals while secondary lymphedema is the most common cause of the disease and affects approximately 1 in 1000 individuals.

Lymphoedema is a chronic condition that causes swelling in the body tissues. It can affect any part of the body but usually develops in the arms or legs. It develops when the lymphatic system does not work properly and the patient becomes immobile and is left untreated. The lymphatic system is a network of channels and glands throughout the body that helps fight infection and remove excess fluid. There are 2 main types of lymphoedema.

Primary lymphoedema is caused by developmental defects of the lymphatic system. It can develop at any age but usually starts during infancy, adolescence, or early adulthood. Secondary lymphoedema occurs due to damage to the lymphatic system or problems with the movement and drainage of fluid in the lymphatic system. It can be the result of cancer treatment, an infection, injury, inflammation of the limb, or a lack of limb movement. Lymphedema secondary to filarial infection is commonly called Filariasis.

Dr Satbhai added, “Puraw was advised to undergo surgery for the treatment of lymphedema. After optimal preoperative preparation, he underwent vascularized lymph node transfers (VLNT) for both the lower limbs. VLNT is an advanced microvascular procedure, in which a few normal lymph nodes are taken from a distant part (such as the neck, chest, abdomen, etc.) and they are implanted into the limb affected by lymphedema. These transferred lymph nodes then help to drain the excess fluid from the limb and reduce the limb swelling. The surgery lasted for 6 hours. The boy was discharged after 10 days. He recovered well after the microvascular surgery on both lower limbs and the swelling on both his legs reduced significantly. The surgery has helped the boy to become mobile, playful, and active again. The parents are advised to perform a special type of massage called ‘manual lymphatic drainage. The parents are relieved, as the child is no longer dependent on them for his basic hygiene and personal activities.”

This is a complex problem and has no permanent solution. There was no treatment available till a few years back. “Currently, advanced microvascular surgery (vascularized lymph node transfer) is being performed at very few centres in the country, and the Bai Jerbai Wadia Hospital for Children is one of them. It has shown good results and is a big hope for these patients who had no better solution till now. We are fortunate to help this boy who was bed-ridden to stand on his feet again, and move freely,” said Dr Minnie Bodhanwala, CEO, Wadia Hospital.

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Published on: Tuesday, April 19, 2022, 10:48 PM IST