When they lost hope in their own country, the infants from Fiji and Iraq with the rare case of Biliary Atresia found the way to their lives in India.
New Delhi: Two babies suffering from biliary atresia, a rare disease of the liver and bile ducts that occurs in infants, were successfully treated by doctors of Fortis hospitals during the last two months. Six-month-old Azhariah Malala, who came from Fiji, and 21-month-old Lilaz Bakhtiyar, who arrived here for treatment from Iraq, were treated at Fortis Hospital, Gurgaon and Fortis Escorts, Delhi respectively.
These two intensive surgeries were done by a team of doctors under the supervision of Dr Vivek Vij, Director, Liver Transplant, Fortis Healthcare. In both the cases, their parents were the donors.
“Children with the foetal form of biliary atresia are more likely to require liver transplants, usually sooner rather than later. The need for a transplant is decided by the extent of damage and how badly the liver is diseased. Paediatric patients account for about 12.5% of liver transplant recipients. Biliary atresia is reason for transplant in paediatric age group in 50% of patients,” said Dr Vivek Vij.
Dr Ajitabh Srivastava, Senior Consultant, Liver Transplant, Fortis Healthcare, added, “These surgical procedures are challenging because of the complexity of the patients who undergo liver transplant as they are already very sick because of the disease and they need to be monitored very rigorously in the post operative period as well. In case of infants, performing vascular anastomosis becomes ever more challenging. But once it’s done successfully, the baby has very normal growth like any other individual.”
Azhariah’s case was presented at FMRI, Gurgaon and diagnosed with a rare congenital defect called ‘Biliary atresia with cystic malformation of biliary tract’. This is a condition in which the bile duct has not developed completely and is blocked, thereby not draining the bile from the liver. This resulted in stretching and blockage of blood supply of liver, mainly portal vein which supplies 75% of blood to liver. The baby weighed 6 Kg, out of which liver was of 2 Kg due to accumulation of the bile in the organ. An eight-hour long liver transplant surgery was conducted in a systematic and detailed manner.
Azhariah’s father, Semi Keuva Sua, donated 25% of his liver to his son. His portal vein was atretic (poorly developed) and hence a procedure of venoplasty was done to increase the size of portal vein. Surgical incision was conducted carefully to minimize blood loss and post-operative care was provided to the infant with immunosuppression.
“There was cystic malformation in the biliary system because of the chronic blockages in the bile. With CT scan, we learnt about the large abnormal vessel from the portal vein, which was draining out of the left renal vein. We levitated the renal vein, which improved the blood flow to the portal vein and the patient did well in the post operative period, while we frequently checked him with Doppler ultrasound. The baby got discharged within two week’s time,” said Dr Srivastava.
Another baby Lilaz, also suffering from Biliary atresia, a condition in which the bile duct is not formed in the foetal stage, was operated at Fortis Escorts Heart Institute. The infant had undergone a biopsy, which helped diagnose the congenital abnormality. She underwent an ABO incompatible (donor and recipient of different blood groups) transplant where her mother, an A blood group, donated to Lilaz who has O blood group.
“One of the earliest symptoms of biliary atresia is congenital jaundice, which if persists after a week or 10 days’ time, there could be many factors involved, one of which could be biliary atresia. One of the ways to indentify it is imaging testing,” said Dr Srivastava.
Azhariah’s father Semi Keuva Sua, said, “We were vacationing in the New Zealand when our three months old got sick. “New Zealand doctors told us that the baby’s liver had failed and it was too late to perform Kasai portoenterostomy. They said they could do liver transplant but the donor needed to be of the same age as the baby. We went back to our country looking for a donor baby. There our government supported us and sent us to India for the treatment.”
Dr Srivastava said that they do close to 250 liver transplants yearly and 10% of the volume is paediatric cases.
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