A Successful Liver Transplant for a Patient with Wilson’s Disease

Chief Complaints

A 24-year-old female patient with a Body Mass Index (BMI) of 20, a known case of Wilson’s disease, presented to the Liver Transplant Department at PACE Hospitals, Hitech City, Hyderabad, with advanced decompensated chronic liver disease.

Her condition was associated with complications including gastrointestinal bleeding and hepatic encephalopathy, indicating significant deterioration of liver function and the need for specialised liver transplant evaluation.

The patient was a known case of Wilson’s disease, a condition in which excess copper accumulates in the liver and other organs. Despite medical management, the disease had progressed to decompensated chronic liver disease.

On examination, the patient was conscious, coherent, and oriented, with stable hemodynamic status. General physical examination was performed, followed by cardiovascular, respiratory, abdominal, and neurological assessment. The abdomen was examined for distension, tenderness, fluid accumulation, and organ enlargement. Neurological status was also assessed carefully, including level of consciousness, behavior, speech, coordination, and limb movements.

Upon admission to PACE Hospitals, following a detailed clinical assessment, the Liver Transplantation team evaluated the patient with a known history of Wilson’s disease that had progressed to decompensated chronic liver disease. Her history of gastrointestinal bleeding and hepatic encephalopathy was also reviewed as part of the assessment of advanced liver dysfunction.

The diagnosis of decompensated chronic liver disease secondary to Wilson’s disease was established based on her known medical history, clinical presentation, and comprehensive liver transplant evaluation. Gastrointestinal bleeding and hepatic encephalopathy were identified as associated complications of advanced liver failure.

Routine and specialised investigations, including complete blood picture, serum electrolytes, liver function tests, renal function assessment, coagulation-related tests, D-dimer, urine examination, and 24-hour urinary copper assessment, were performed to evaluate the severity of liver dysfunction and the patient’s overall medical condition. A chest X-ray showed mild right-sided pleural effusion without any other significant cardiopulmonary abnormality, while the SARS-CoV-2 RT-PCR test was negative.

Based on the confirmed diagnosis, the patient was advised to undergo advanced Wilson’s Disease Treatment in Hyderabad, India, under the expert care of the Liver Transplantation Department.

After detailed consultation with Dr. CH Madhusudhan (Senior Consultant Surgical Gastroenterologist and Liver Transplant Surgeon), Dr. Govind Verma (senior consultant interventional gastroenterologist and hepatologist), Dr. Suresh Kumar (Consultant Surgical Gastroenterologist), a comprehensive evaluation was performed to determine the most appropriate therapeutic approach.

Considering the patient’s known history of Wilson’s disease, progression to decompensated chronic liver disease, and associated complications of gastrointestinal bleeding and hepatic encephalopathy, further assessment confirmed advanced liver dysfunction requiring definitive treatment. Laboratory investigations, urinary copper assessment, liver function tests, coagulation-related tests, chest imaging, and pre-anaesthesia evaluation were reviewed as part of the liver transplant work-up.

Based on the patient’s clinical condition, progressive liver failure, and limited likelihood of recovery with medical treatment alone, Living Donor Liver Transplantation was considered the most appropriate and potentially life-saving management strategy. The patient’s mother voluntarily came forward as the donor and underwent detailed medical assessment to confirm her suitability. A modified right-lobe liver graft was selected to provide adequate liver volume and support restoration of liver function.

The patient, donor, and family members were counselled regarding the diagnosis, urgency of transplantation, donor and recipient procedures, potential risks, expected recovery, lifelong immunosuppressive therapy, infection-prevention measures, and the importance of regular postoperative follow-up. 

Following the decision, the patient was scheduled for Living Donor Liver Transplantation in Hyderabad at PACE Hospitals, under the expert care of the liver transplant team.

The following major steps were carried out during the procedure:

• Patient Preparation and Anaesthesia: After completing the preoperative evaluation, donor assessment, transplant clearances, and consent process, the patient was taken up for surgery under general anaesthesia. Continuous monitoring was established to assess blood pressure, oxygenation, fluid balance, blood loss, and cardiovascular stability.

• Donor Surgery and Graft Preparation: The patient’s mother underwent living donor liver surgery after being confirmed medically suitable. A modified right-lobe liver graft was carefully removed while preserving adequate liver volume and function for the donor, and the graft was prepared for transplantation.

• Removal of the Diseased Liver: The recipient’s damaged liver was carefully separated from the surrounding tissues and blood vessels. It was removed while controlling blood loss and maintaining haemodynamic stability, and the explanted liver was sent for histopathological examination.

• Graft Implantation and Biliary Reconstruction: The donor liver graft was placed in the recipient’s abdomen and connected to the major blood vessels to restore blood supply and venous drainage. The donor bile duct was then reconstructed and connected to the recipient’s biliary system to allow proper bile flow.

• Hemostasis, Drain Placement and Closure: The surgical team assessed the graft perfusion, vascular connections, bile flow, and operative field for bleeding or leakage. Abdominal drains were placed, and the incision was closed in layers after confirming satisfactory haemostasis. The procedure was completed successfully without major intraoperative complications.
  

The patient had a satisfactory postoperative recovery and initially required supplemental oxygen, which was gradually discontinued as her condition improved. Intravenous fluids were given to maintain hydration, while fluid balance, urine output, kidney function, and electrolyte levels were monitored regularly. Increased abdominal drain output was managed with albumin infusions and close observation. Histopathological examination of the explanted liver was performed after transplantation to assess the underlying liver changes.

Liver function tests showed gradual improvement, and immunosuppressive doses were adjusted accordingly. She also received medications for infection prevention, pain relief, gastric protection, nausea control, nutritional support, and maintenance of fluid and electrolyte balance. After achieving stable graft function, adequate oral intake, and hemodynamic stability, she was discharged in a stable condition with appropriate follow-up advice.

Upon discharge, the patient was prescribed oral medications for prevention of liver graft rejection, prevention and treatment of infection, gastric protection, pain control, nutritional supplementation, and other supportive requirements.

The patient was advised to wear a mask in public places, maintain good hand hygiene, avoid crowded areas and contact with individuals who have infections, and consume a hygienically prepared, protein-rich diet with safe drinking water.

She was instructed to take all prescribed medications regularly, avoid missing immunosuppressive doses, maintain adequate hydration, keep the surgical wound clean and dry, avoid strenuous physical activity until medically cleared, and attend scheduled follow-up visits with regular blood investigations.

The patient was informed to contact the emergency ward at PACE Hospitals in case of any emergency or development of symptoms such as fever, severe abdominal pain, persistent vomiting, reduced urine output, or redness, swelling, or discharge from the surgical wound.

The patient was advised to return for a follow-up visit with the Surgical Gastroenterologist in Hyderabad at PACE Hospitals after 3 days.

This case highlights the successful management of Wilson’s disease with decompensated chronic liver disease through Living Donor Liver Transplantation. A modified right-lobe graft was transplanted successfully without major complications. Gradual improvement in liver function and a stable postoperative recovery demonstrated the effectiveness of timely transplantation and specialized multidisciplinary care.