Mumbai: In a complex 10-hour surgery, doctors successfully performed a living donor liver transplant on a one-year-old infant weighing just six kg, with his mother donating a portion of her liver to save his life.
Infant Diagnosed With Rare Genetic Disorder Alagille Syndrome
The procedure was performed at Narayana Health SRCC Children’s Hospital, Mumbai. The infant was suffering from advanced liver disease caused by Alagille syndrome, a rare inherited genetic disorder that affects multiple organs, including the liver. His condition was further complicated by kidney and cardiac problems, impaired blood clotting and severe nutritional deficiencies.
His symptoms began soon after birth with persistent itching and dark-coloured urine. By eight months, the itching had worsened, he was not gaining weight adequately and his abdomen had become enlarged.
Genetic Tests And Liver Biopsy Confirm Rare Disorder
Investigations revealed elevated bilirubin levels, impaired blood clotting and a nodular, scarred liver. A liver biopsy and genetic tests, including whole exome sequencing, helped doctors identify Alagille syndrome as the underlying cause. The infant also had renal tubular acidosis, mild narrowing of pulmonary arteries and mildly elevated pressure in the lungs.
Considering the multiple complications, a multidisciplinary team led by Dr Sanjay Kumar Goja and Dr Ashok Thorat, Senior Consultants in Liver Transplantation and Hepatobiliary Surgery, along with specialists in paediatric gastroenterology, anaesthesia, critical care and nutrition, prepared the infant for transplantation.
Mother Cleared As Liver Donor After Genetic Screening
Doctors first improved his nutritional status through calorie and protein supplementation and addressed vitamin deficiencies. Genetic screening confirmed that his mother did not carry the identified JAG1 gene variant. After comprehensive medical evaluation, she was cleared to donate a portion of her liver.
The infant's extremely low weight, fragile blood vessels, poor clotting and associated heart and kidney conditions made the surgery particularly challenging. Surgeons used precise microvascular techniques to connect the blood vessels and ensure adequate circulation to the transplanted liver.
Surgeons Complete Complex 10-Hour Liver Transplant Procedure
During the 10-hour procedure, doctors removed the diseased liver, implanted the donated portion and reconstructed the blood vessels and bile duct.
Following surgery, the infant spent seven days in the transplant intensive care unit and another eight days in a high-dependency ward. His mother was discharged five days after the procedure.
Doctors monitored his liver function, kidney health, blood clotting and signs of infection or organ rejection. He gradually resumed oral feeding and completed a 15-day postoperative hospital stay.
Doctors said the infant would require continued follow-up for his cardiac and kidney conditions. The case highlights the importance of early medical assessment of warning signs such as prolonged jaundice, dark urine, persistent itching and poor weight gain in infants, which may indicate serious underlying liver disease.
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