Rare liver surgeries on kids

NEW DELHI: Three liver surgeries, conducted over 16 hours by 20 doctors at Sir Ganga Ram hospital, has set medical records tumbling.

Being touted as a "breakthrough surgery" with many "firsts", the team from SGRH performed the country's first and the world's youngest domino liver transplant -- a procedure in which one patient is cured with a transplant and then his or her organ is used for a life-saving transplant into someone else.

While 22-month-old Shourya was cured of Maple Syrup Urine Disease (MSUD) -- a highly rare inherited metabolic disorder that causes mental retardation and death -- by a transplant performed using 20% of his aunt's liver, his own liver was, in turn, transplanted into three-year-old Siya, who was battling end-stage liver failure due to another rare condition called Langerhans Cell Histiocytosis (LCH).

The surgeries were conducted on January 31.

Dr A S Soin, chief liver transplant surgeon, said: "According to the World Domino registry, the youngest registered domino liver transplant recipient was a 17-year-old. Verbally, experts at University of Pittsburgh Medical College (UPMC) say the youngest could be a four-year-old."

Dr Neelam Mohan, chief paediatric hepatologist, who visited UPMC to discuss Shourya and Siya's case, said the domino transplant was possible because both the children had the same blood group (B positive). It was also beneficial that both weighed almost the same.

"Only in 1997 was it accidentally discovered that a transplant could help patients with MSUD. The biggest challenge during the surgery on Shourya was to tightly control amino acids, glucose and sodium levels in blood before and during surgery until the new liver started working. Even a slight stress in Shourya's body would have precipitated a crisis," Dr Mohan said.

Dr Soin added: "I wrote to hospitals in Kyoto, Seoul, Brazil, Minnesota and Pittsburgh, who have an active domino transplant programme, to learn their experiences and know how they manage their patients. After taking their advise, we created a strict protocol which was followed by all of us to the hilt."

While Shourya will be discharged from hospital on Friday, Siya will be on her way back home by Sunday.

Siya was diagnosed with LCH in November 2007. Subsequently, she was put on chemotherapy for a year which cured her of LCH (a disease that behaves like cancer causing body's immune cells attacking the body rather than fighting infections) but caused end-stage renal failure. Shourya's body, on the other hand, lacked a vital enzyme, BCKAD, because of which he had to be on a protein-free diet, lest it cause brain damage.

Shourya's father had to feed him special amino acid free formula milk brought from UK, that cost him Rs 50,000 a month.

"Because 12% of this enzyme is present in the liver, we decided to undertake a transplant. Because his parents carry a defective gene for MSUD, we took 20% of the liver from his aunt Mukta," Dr Soin said.

For the surgery, the team was divided into three units who prepared the donor liver, Shourya's own liver and Siya's diseased liver for removal in three separate operating theatres.

First the donor liver and then Shourya's own liver were removed and prepared for transplantation by re-constructing the vital blood vessels with complex vein grafts. Shourya was transplanted first, and then his liver was transplanted into Siya.

Dr Soin said: "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. MSUD is one such disease."

According to Dr Mohan, UPMC, which was approached by Shourya's parents, said the surgery would cost Rs 1 crore and would need them to stay back in the US for 3-6 months.

In India, the surgery cost them Rs 12-15 lakh. "Shourya will now be able to tolerate normal protein diet. In the case of Siya, she will be on low dose chemotherapy for another six months to ensure that LCH does not recur in the new liver. Relapse rate is 10%-20%," Dr Mohan added.