Successful Living Donor Liver Transplant for a 9 Y.O. Boy with DCLD

Chief Complaints

A 9-year-old male patient with a body mass index (BMI) of 17 presented to the Liver Transplant Department at PACE Hospitals, Hitech City, Hyderabad, with chief complaints of progressively worsening yellowish discoloration of the eyes. He is a known case of Alagille syndrome with decompensated chronic liver disease (DCLD), portal hypertension, jaundice, coagulopathy, and sepsis. His Pediatric End-Stage Liver Disease (PELD) score was 54. The patient also had a recent history of fever for the past 3 days.

The patient had a history of Alagille syndrome with chronic liver disease (DCLD), associated with portal hypertension, jaundice, and coagulopathy, along with a prior episode of fever suggestive of sepsis.

On examination, the patient was conscious, coherent and oriented with evident icterus (yellowish discoloration of eyes). He appeared clinically ill with features of chronic liver disease, including pallor. Abdominal examination revealed hepatosplenomegaly with mild ascites, and signs suggestive of portal hypertension were present. There were no focal neurological deficits, and other systemic examinations were largely normal.

Upon admission to PACE Hospitals, following the clinical assessment, the liver transplant team conducted a comprehensive evaluation based on the patient’s complaints of progressively increasing yellowish discoloration of the eyes in a known case of Alagille syndrome. A detailed medical history, physical examination, and preoperative assessment were performed to evaluate the severity of underlying liver disease and overall fitness for liver transplantation, along with identification of associated complications including portal hypertension, jaundice, and coagulopathy.

Baseline investigations, including complete blood picture, liver function tests, renal function tests, serum electrolytes, coagulation profile (PT/INR, APTT), inflammatory markers (CRP, procalcitonin), ammonia levels, thyroid profile, viral screening (Hepatitis A, Hepatitis E, COVID-19, Influenza panel), blood cultures (aerobic and anaerobic), urine culture, complete urine examination, fibrinogen levels, fibrin degradation products, arterial blood gas analysis, serum calcium, magnesium, and phosphorus levels were carried out.

Imaging and diagnostic studies, including chest X-ray, HRCT chest, ultrasonography of the abdomen, CT scan, liver Doppler studies, and 2D echocardiography, were performed. These investigations revealed features consistent with chronic liver disease, including cirrhotic liver changes, splenomegaly, minimal ascites, portal hypertension.

Based on the confirmed diagnosis, the patient was advised to undergo Decompensated Chronic Liver Disease (DCLD) Treatment in Hyderabad, India, under the care of the liver transplant team, ensuring restoration of liver function and prevention of life-threatening complications.

Following the decision, the patient 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:

• Recipient Exploration and Liver Mobilization: The patient was placed under general anesthesia in the supine position. A bilateral subcostal incision was made to access the abdominal cavity. Intraoperative assessment confirmed features of chronic liver disease with portal hypertension. The native liver was mobilized by dividing the falciform ligament, right and left coronary ligaments, and diaphragmatic attachments. Careful dissection was performed to free the liver from surrounding structures while maintaining hemostasis throughout the procedure.

• Hilar Dissection and Native Liver Explantation: Detailed porta hepatis dissection was carried out, and the hepatic artery, portal vein (PV), and common bile duct (CBD) were identified. The right and left hepatic arteries were clipped and divided. The bile duct was ligated and divided appropriately. The portal vein was preserved intact initially. The hepatic veins, including the middle hepatic vein (MHV) and left hepatic vein (LHV), were meticulously dissected, looped, and prepared for subsequent vascular anastomosis. Once the donor graft was ready, the diseased native liver was explanted.

• Implantation of Donor Left Extended Hepatectomy Graft: A living donor related liver transplant using a left extended hepatectomy graft harvested from the patient's father was performed. The donor MHV-LHV venous confluence was anastomosed to the recipient MHV-LHV cuff over the inferior vena cava (IVC) using 5-0 Prolene sutures. Subsequently, the donor left portal vein (LPV) was anastomosed to the recipient main portal vein (MPV) with 6-0 Prolene sutures, restoring portal inflow to the graft. The warm ischemia time was 38 minutes, cold ischemia time was 1 hour, and the anhepatic phase duration was 54 minutes.

• Hepatic Artery and Biliary Reconstruction: After satisfactory portal reperfusion, arterial reconstruction was performed by end-to-end anastomosis of the donor left hepatic artery (LHA) to the recipient left hepatic artery using 8-0 Prolene under magnification. Biliary reconstruction was then completed by anastomosing the donor left hepatic duct (LHD) to an enterotomy created on the D1 segment of the recipient duodenum using 6-0 PDS sutures in a choledochoduodenostomy (CDD) fashion. Post-anastomotic Doppler assessment demonstrated satisfactory hepatic arterial and portal venous flow.

• Hemostasis, Drain Placement, and Closure: Following confirmation of adequate graft perfusion and hemostasis, the transplanted liver was fixed in position with sutures. One right and one left subdiaphragmatic drain were placed for postoperative monitoring. The abdomen was closed in layers after ensuring complete hemostasis. Subcuticular skin suturing was performed. The donor graft weighed 389 grams with a graft recipient weight ratio (GRWR) of 2.04. The patient tolerated the procedure well and was shifted to the intensive care unit for postoperative management.

The procedure was uneventful, and the patient was closely monitored postoperatively with regular assessment of graft function and hemodynamic status. He developed anemia and coagulopathy, which were managed with blood component support, along with measures to optimize liver perfusion. Serial monitoring showed gradual improvement in liver and renal function. 

Infection surveillance was performed with cultures and monitoring for sepsis, and appropriate infection control measures were undertaken when required. Glycemic fluctuations were managed with blood sugar control measures, and cardiac findings were addressed with supportive cardiac management and fluid optimization. Overall, the patient received comprehensive care including immunosuppression, supportive therapy, and monitoring, and was discharged in a stable condition.

At the time of discharge, the patient was advised medications for maintenance of graft function and prevention of rejection, along with prevention of bacterial and fungal infections due to immunocompromised status. He was also started on treatment for hepatic encephalopathy prevention and gut decontamination, and gastric mucosal protection.

Supportive therapy included medications for liver protection and antioxidant support, blood pressure stabilization, and cardiac management. Additional supplementation was given for calcium, magnesium, and nutritional support, along with measures for bowel regulation. The patient was also advised blood sugar control therapy.

The patient was advised to follow a high-protein diet to support recovery and promote healing. He was instructed to monitor blood glucose levels daily to ensure adequate glycemic control. Precautions to prevent infection were emphasized, including avoiding crowded places and consistently wearing a mask. In addition, proper drain care and wound care were advised to prevent postoperative complications and ensure optimal healing.

The patient was informed to contact the emergency ward at PACE Hospitals in case of any emergency or development of symptoms such as abdominal pain, fever, vomiting, or any signs of clinical deterioration, for prompt evaluation and management.

The patient was advised to follow up with the Surgical Gastroenterologist in Hyderabad at PACE Hospitals, after 5 days.

This case highlights successful management of advanced chronic liver disease with portal hypertension through liver transplantation. The postoperative course required intensive monitoring and multidisciplinary care for associated complications. With timely interventions, the patient showed good recovery and a favourable outcome.

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