A Mother’s Gift of Life: Successful Living Donor Liver Transplantation for Biliary Atresia

Chief Complaints

A 1-year-2-month-old female child presented to the Liver Transplant Department at PACE Hospitals, Hitech City, Hyderabad, with complaints of jaundice and intermittent fever. The child had a 4-month history of jaundice associated with recurrent episodes of intermittent fever. 

The patient was a known case of Type 1 biliary atresia diagnosed in early infancy. She underwent Kasai procedure with duodenojejunostomy at 45 days of age as part of her initial surgical management.

Two weeks prior to presentation, she had been admitted with viral pneumonia and cholangitis. In view of persistent jaundice, recurrent fever, and recurrent post-Kasai cholangitis, she was evaluated for progressive liver dysfunction secondary to biliary atresia. 

On examination, the child was conscious and alert, with evident icterus (yellowish discoloration of the sclera). She appeared clinically unwell in the background of persistent jaundice and intermittent fever. Abdominal and systemic examination findings were assessed in view of suspected progressive liver dysfunction, post-Kasai cholangitis, and annular pancreas. No other significant examination abnormalities were documented. 

Upon admission to PACE Hospitals, following clinical assessment, the liver transplant team conducted a comprehensive evaluation based on the child’s complaints of persistent jaundice and intermittent fever. The child was a known case of Type 1 biliary atresia and had previously undergone Kasai procedure with duodenojejunostomy at 45 days of age.

A detailed medical history, physical examination, and required investigations were performed to evaluate the cause of ongoing jaundice, recurrent fever, and suspected progressive liver dysfunction. The child also had a recent history of hospital admission for viral pneumonia and cholangitis.

Imaging evaluation, such as ultrasonography, cholangiography, and other required tests, were carried out to assess liver status and identify any obstruction in the bile ducts. Based on the clinical history and investigation findings, the child was diagnosed with post-Kasai cholangitis with annular pancreas and Progressive liver dysfunction secondary to biliary atresia. The Paediatric End-Stage Liver Disease (PELD) score was 14, indicating significant paediatric liver disease requiring specialist evaluation.

Based on the confirmed diagnosis and progressive liver dysfunction, the child was advised to undergo Pediatric End-Stage Liver Disease Treatment in Hyderabad, India, under the care of the Liver Transplant Department.

After detailed evaluation by Dr. CH Madhusudhan (Senior Consultant Surgical Gastroenterologist and Liver Transplant Surgeon), along with cross-consultation with Dr. Govind Verma (Senior Consultant Interventional Gastroenterologist and Hepatologist) and Dr. Suresh Kumar (Consultant Surgical Gastroenterologist), the child’s condition was reviewed in detail. The patient had persistent jaundice, intermittent fever, post-Kasai cholangitis, annular pancreas, and progressive liver dysfunction secondary to biliary atresia, despite having undergone Kasai surgery during infancy. 

Considering the child’s worsening liver condition and the risk of further complications, the liver transplant team determined that Living Donor Liver Transplantation (LDLT) was the most appropriate treatment option to restore liver function, prevent further disease progression, and improve long-term survival and quality of life.

The child was kept on required supportive care while donor evaluation was carried out. Understanding the seriousness of the condition, the child’s mother came forward voluntarily to donate a portion of her liver. Since both the donor and recipient had the same blood group, there were no blood group incompatibility concerns.

After completion of necessary investigations, donor fitness evaluation, recipient assessment, pre-anaesthesia check-up, and required clearances, Living Donor Liver Transplantation using a left lateral segment graft was planned.

The family was counselled regarding the child’s diagnosis, the need for liver transplantation, donor evaluation, the surgical procedure, possible risks and complications, postoperative ICU care, immunosuppressive medicines, infection prevention, and the need for regular long-term follow-up after transplant.

Following the decision, the child was scheduled to undergo Living Donor Liver Transplantation (LDLT) in Hyderabad at PACE Hospitals under the expert care of the liver transplant team.

The procedure involved the following steps:

• Preoperative Preparation and Anaesthesia: After completion of the required investigations, the child was taken up for liver transplant surgery under general anaesthesia with continuous monitoring.

• Donor Evaluation and Graft Planning: The child’s mother was evaluated as the living liver donor. A left lateral segment graft was prepared for transplantation.

• Recipient Exploration and Diseased Liver Removal: The child’s diseased liver was carefully removed after controlling the major blood vessels and bile duct structures. The surgical team prepared the recipient site for implantation of the donor liver graft.

• Implantation of Left Lateral Segment Graft: The left lateral segment graft donated by the mother was implanted in the child. The graft was positioned appropriately to restore liver function and support recovery from paediatric end-stage liver disease.

• Vascular and Biliary Reconstruction: The donor liver graft blood vessels were connected to the recipient’s blood vessels to restore blood supply to the transplanted liver. Biliary reconstruction was performed to allow proper bile drainage from the new liver.

• Graft Flow Assessment and Closure: After reconstruction, liver graft blood flow was assessed using liver Doppler. The procedure was completed successfully without reported complications, and the child was shifted for close postoperative monitoring.

The postoperative period was uneventful. The child was closely monitored for transplanted liver function, graft blood flow, hemodynamic stability, and overall recovery. Liver Doppler findings supported satisfactory graft monitoring.

She developed an increased white blood cell count with high lymphocytes, which was managed conservatively. She received medicines to protect the transplanted liver and prevent rejection, prevent infection, support gastric protection, nutrition, prevent vomiting, pain relief, fever control, and other supportive care. After achieving hemodynamic stability, she was discharged with medications, counselling, and follow-up advice.

At discharge, the child was advised to take medicines to prevent graft rejection and prevent rejection, along with medicines for infection prevention, gastric protection, nutritional support, to prevent vomiting, pain relief, fever control, and other supportive care.

The parents were advised to give all medicines on time, maintain hygiene, protect the child from infections, avoid crowded places, and follow the liver transplant team’s instructions carefully. Regular follow-up was advised to monitor recovery and adjust medicines when needed.

The parents were advised to contact the emergency ward in case of the development of symptoms such as fever, vomiting, abdominal pain, poor feeding, breathing difficulty, reduced urine output, worsening jaundice, unusual drowsiness, bleeding, wound concerns, or sudden deterioration.

The child’s parents were advised to return for regular follow-up visits with the Surgical Gastroenterologist in Hyderabad at PACE Hospitals for monitoring transplanted liver function, adjusting immunosuppressive medicines, assessing recovery, and detecting any early signs of infection, rejection, or other complications.

This case highlights the successful management of a 1-year-2 months female child with post-Kasai cholangitis, annular pancreas, and progressive liver dysfunction through Living Donor Liver Transplantation using a left lateral segment graft donated by her mother. The child recovered well after surgery, with satisfactory graft monitoring on liver Doppler, and was discharged in stable condition with medications, counselling, and follow-up advice.