World's youngest domino liver transplant in Delhi hospital

New Delhi | Thursday, Feb 19 2009 IST

In what is claimed to be the first of the world's youngest domino liver transplant, a unique procedure in which two life-saving liver transplants are performed using just one donor liver, two children aged 22 months and two years old and a 37-year-old woman underwent the surgery in a hospital here.

While Shourya, a boy, was cured of Maple Syrup Urine Disease(MSUD) by a transplant performed using 20 per cent of the liver of his aunty, Mukta, 37, his own liver was, in turn, tranplanted into another two-year-old girl Siya, who was battling terminal liver failure due to a rare condition called Langerhan's Cell Histiocytosis(LCH), in Sir Ganga Ram Hospital in central Delhi.

It is due to the sequential nature of the transplants that the domino transplants are so named.

Addressing a news conference here today, Dr A S Soin, the Chief Liver Transplant Surgeon and Director of Liver Transplantation of Sir Ganga Ram Hospital, 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 with passing on the disease. MUSD is one such disease.

The surgery, which was also India's first, was performed by 20 specialists on January 31 and it lasted 16 hours.

He claimed this made Shourya the country's first and the world's youngest recipient of a living donor liver transplant for MSUD and Siya the country's first and the world's youngest recipient of a domino liver transplant.

Asked if they had any chance of developing complications in the near future after the surgery, Chief Paediatric Hepatologist Neelam Mohan said they would both be normal in three months and ''we would be closely monitoring them to check if whether the disease relapses or not''.

Shourya, Dr Mohan added, was suffering from MSUD since birth and in this disease toxic level os branched chain amino acids -- leucine, isoleucine and valine build up due to the lack of the detoxifying dehydrogenase enzyme.

She said in cases of MSUD disease soon after the birth children develop metabloic crises with vomiting, poor feeding, weight loss, convulsions and coma. The high levels of acids in the urine result in a smell of burnt sugar, hence the name Maple Syrup. ''To survive, they need a special diet low in protein and devoid of these amino acids, along with regular monitoring of leucine levels.'' Shorya's parents, Prashant and Neeti, moved to the US and put him permanently on the MUSD diet which costs around Rs 60,000 to Rs 70,000 per month. MSUD threatened to damage the nervous system of Shorya. His aunty, Mukta, was found to be a suitable donor and after the transplant said, ''it is such a joy to see the child on a normal diet for the first time in his life''.

Siya was suffering from LCH that affects many organs but especially the liver and resulted in its failure. She had several courses of chemotherapy to contain the disease. Although her disease went into remission her liver had been irreversibly damaged due to which she needed a life-saving transplant.

While Siya's mother was willing to give a part of her liver for the transplant, she did not need to undergo surgery as Shourya's liver could be successfully used in this unique domono operation, Dr Mohan added.