Hyderabad: PACE Hospital’s team has successfully performed liver transplantation for a rare genetic disorder “Alagille Syndrome” in a very frail 8 year old child.
Alagille Syndrome is an extremely rare genetic disorder seen in less than one in lakh births, in which the bile ducts (canal system) inside the liver does not develop, so the bile accumulates in the liver and the blood. This leads to jaundice, severe itching. And eventually liver failure. It gets transmitted to the children even if one of the parents has a hidden gene. It is also associated with severe growth retardation.
“This child at the age of 8 years was only 12 KGS and 105 centimeters, while his actual weight should be about 28 KGS. So the child was very frail and extremely high risk for transplantation”, said Dr Govind Verma, Chief Hepatologist.
“His father came forward to donate a part of his liver to his son. It was a very difficult operation as the child had a frail built and the portal vein that supplies the liver was blocked. Needing multiple revisions and complex reconstruction.” said Dr. R Phani Krishna, transplant surgeon.
Despite a difficult operation and frail patient with dedicated efforts of our transplant team, ICU and nursing team, the child recovered smoothly and was discharged by third week. Presently, at six weeks postoperatively, he continues to do well and is completely free from itching and jaundice for the first time in his life.
“He has gained 1 kg weight in the past three weeks and we expect him to catch up his weight and height milestones in the coming years”, said Dr Dhiraj Agarwal, Consultant Hepatologist.
PACE transplant team consisted of Dr. Govind Verma, Dr. Dhiraj Agarwal, Dr. Madhusudhan, Dr. R Phani Krishna, Dr. Suresh Kumar and Dr. Santosh Kumar – Transplant surgeons and Dr. Harish, Dr. Manasa – Anesthesia and ICU team, Dr. Priyanka, Paediatrician.
“We had many apprehensions as our child was severely underweight. Whether he can tolerate such a major surgery like transplant but with the hard work and dedication of the past team of doctors and nurses, he has been giving a second life. It is like a miracle to see him jaundice free and itching free for the first time in his life.” said his mother.
