Mother’s liver donation saves child with rare disorder

A nine-year-old girl from Srikakulam district, diagnosed with the rare congenital condition Abernethy syndrome, has been given a new lease of life after her mother donated a portion of her liver for a transplant at MGM Seven Hills Hospital.

The child, a resident of Budarayavalasa village in Merakamudidam mandal, had been suffering from persistent jaundice, fatigue, and severe growth retardation for nearly three years. Her family had sought treatment at several hospitals in Visakhapatnam and Hyderabad, but the cause of her condition remained undetected.

Doctors said the diagnosis was confirmed following advanced imaging tests, including CT angiography and Doppler studies, conducted by consultant HPB and liver transplant surgeon Dr. Usha Ramakrishna N. The tests revealed Abernethy syndrome, a rare vascular anomaly in which the portal vein is underdeveloped, leading to abnormal diversion of blood away from the liver.

“In such cases, the liver does not receive adequate blood supply, resulting in toxin build-up in the bloodstream and progressive liver dysfunction. Liver transplantation is the only definitive treatment,” doctors said.

Following counselling, the child’s mother volunteered to donate a part of her liver. A living donor transplant using the left lateral segment was planned.

The 10-hour surgery was performed by a team led by Dr Thiagarajan Srinivasan, Director of Liver Transplant and HPB Surgery, along with Dr Usha Ramakrishna and Dr Srikanth Tummala. The procedure was supported by a multidisciplinary team comprising transplant anaesthesiologists, paediatric intensivists, hepatologists, and specialised nursing staff.

Doctors said the child faced some post-operative challenges but recovered steadily and was discharged after 11 days. At a three-month follow-up, she was found to have normal liver function and is doing well.