Rare ‘domino liver transplant' gives two kids new life

It was like a game of passing the parcel. Only, the parcel was a precious life-saver.

Doctors at New Delhi’s Sir Ganga Ram Hospital performed a rare 'domino liver transplant' on two children, believed to be the world’s youngest patients to undergo such an operation.

Domino liver transplant involves sequential surgery. About 20 specialists performed the surgery on 22-month-old boy Shourya Verma and two-year-old girl Siya Thakur on January 31. The operation lasted 16 hours.

Shourya has been cured of maple syrup urine disease (MSUD) through the transplant that used 20% of his aunt’s liver; his own liver was given to Siya, who was battling liver failure owing to a rare condition called Langerhans’s cell histiocytosis (LCH).

Siya had many sessions of chemotherapy to contain LCH, and though her disease went into remission her liver was irreversibly damaged, making a transplant the only option.

Doctors at the hospital claimed that Shourya is India's first and the world's youngest recipient of a living donor liver transplant for MSUD, while Siya is the world's youngest recipient of a domino liver transplant.

For the surgery, Shourya's aunt Mukta, Shourya, and Siya were operated upon in three different theatres. First the donor liver and then Shourya's liver were removed. Shourya got a transplant; then his liver was transplanted into Siya.

"Domino transplants are rare because there are very few conditions in which you can cure the patient with a transplant and then transplant his or her organ into someone else without passing on the disease," said Dr AS Soin, the chief surgeon who conducted the transplants and director of liver transplantation at Ganga Ram Hospital.

MSUD is caused by the lack of an enzyme in the body. Once the patient gets a liver transplant, he is able to make enough of the enzyme in the new organ to overcome the shortfall elsewhere in the body.

In turn, an MSUD patient's liver, which is otherwise normal, can be transplanted into someone else without passing on the disease as other cells in the patient can make the enzyme lacking in the liver.

Shourya was born with MSUD. The disease causes vomiting, poor feeding, weight loss, and convulsions and ultimately leads to coma. High levels of acids in the urine give it the smell of burnt sugar; hence the name Maple Syrup.

With his survival at stake, Shourya's parents moved to the US where they put him on an MSUD diet. But they realised it would severely cramp his life as he grew up, besides making it difficult for them to return to India.

"This made the family go ahead with the transplant," said Dr Neelam Mohan, chief paediatric hepatologist at the hospital. "Now, for the first time in his life, Shourya can eat like any other child."