A Challenging Case of Paediatric Liver Transplant and Seizures
PACE Hospitals
PACE Hospitals’ expert Liver Transplant team successfully performed a Living Donor Liver Transplantation (LDLT) on a 9-month-old female infant diagnosed with progressive liver dysfunction secondary to Type 1 extrahepatic biliary atresia following Kasai surgery. The aim of the procedure was to restore liver function, manage paediatric end-stage liver disease, prevent further complications, and improve the child’s long-term survival and quality of life.
Chief Complaints
A 9-month-old female infant presented to the Liver Transplant Department at PACE Hospitals, Hitech City, Hyderabad, with jaundice for the past one month, accompanied by recurrent episodes of intermittent fever.
Past Medical History
The child was a known case of Type 1 extrahepatic biliary atresia, diagnosed during early infancy following jaundice, clay-coloured stools, and an enlarged liver. She underwent a Kasai procedure (hepatoportoenterostomy) with duodenojejunostomy approximately eight months before presentation. Previous liver evaluation showed lobular cholestasis, bile ductular proliferation, and focal fibrosis, indicating progressive liver injury despite the earlier Kasai procedure.
On Examination
On examination, the child was conscious and alert, with evident icterus indicating yellowish discoloration of the eyes. She appeared clinically unwell in the background of persistent jaundice and intermittent fever. Abdominal and systemic examinations were performed to assess the severity of liver dysfunction and her suitability for liver transplantation.
Diagnosis
Upon admission to PACE Hospitals, the liver transplant team conducted a comprehensive evaluation based on the infant’s persistent jaundice and intermittent fever. She was a known case of Type 1 extrahepatic biliary atresia and had previously undergone Kasai hepatoportoenterostomy with duodenojejunostomy.
A detailed medical history, physical examination, liver function assessment, liver biopsy, and other required investigations were performed to evaluate the severity of liver damage. Previous findings included lobular cholestasis, bile ductular proliferation, and focal fibrosis, indicating progressive liver injury.
Based on the clinical history and investigation findings, the infant was diagnosed with progressive liver dysfunction secondary to biliary atresia following Kasai surgery. Persistent liver disease after the Kasai procedure may eventually require liver transplantation when adequate bile drainage is not achieved or liver damage continues to progress.
Based on the confirmed diagnosis, the infant was advised to undergo Paediatric End-Stage Liver Disease Treatment in Hyderabad, India, under the care of the Liver Transplant Department.
Medical Decision Making (MDM)
After detailed evaluation by Dr. CH Madhusudhan (Senior Consultant Surgical Gastroenterologist and Liver Transplant Surgeon), Dr. Govind Verma (Senior Consultant Interventional Gastroenterologist and Hepatologist), and Dr. Suresh Kumar S (Consultant Surgical Gastroenterologist), the infant’s condition was reviewed to determine the most appropriate treatment plan. Despite undergoing Kasai surgery during early infancy, she continued to experience jaundice and intermittent fever, with investigations showing progressive liver damage.
Considering the worsening liver dysfunction and risk of further complications, it was determined that Living Donor Liver Transplantation was the most appropriate definitive treatment to restore liver function and improve long-term outcomes.
The infant was maintained on the required supportive care while a suitable living donor was evaluated. Her mother voluntarily came forward to donate a portion of her liver.
After completing the donor and recipient investigations, medical fitness assessment, pre-anaesthesia evaluation, and required clearances, Living Donor Liver Transplantation using a left lateral segment graft was planned.
The family was counselled regarding the infant’s condition, the need for transplantation, the surgical procedure, possible risks and complications, postoperative intensive care, anti-rejection medicines, infection prevention, and the importance of regular long-term follow-up.
Surgical Procedure
Following the decision, the infant was scheduled to undergo Living Donor Liver Transplantation (LDLT) in Hyderabad at PACE Hospitals under the care of the liver transplant team.
The procedure involved the following steps:
- Preoperative Preparation and Anaesthesia: After completing the required donor and recipient investigations, pre-anaesthesia assessment, and medical clearances, the infant was taken up for surgery under general anaesthesia with continuous monitoring.
- Donor Graft Preparation: The infant’s mother was evaluated and accepted as the living liver donor. A left lateral segment of her liver was prepared for transplantation.
- Removal of the Diseased Liver: The infant’s diseased liver was carefully mobilised and removed after controlling the major blood vessels and bile duct structures.
- Graft Implantation: The left lateral segment graft donated by the mother was positioned in the recipient’s abdomen.
- Vascular and Biliary Reconstruction: The graft blood vessels were connected to the recipient’s blood vessels to restore blood flow. The bile duct was reconstructed to allow proper bile drainage from the transplanted liver.
- Graft Assessment and Closure: After confirming satisfactory graft perfusion and controlling bleeding, the abdomen was closed in layers. The procedure was completed without reported intraoperative complications, and the infant was shifted to the Surgical Intensive Care Unit for close postoperative monitoring.
Postoperative Care
After surgery, the infant was closely monitored in the Surgical Intensive Care Unit (SICU) for graft function, breathing, and overall recovery. She required temporary ventilator support, later developed seizures that were medically managed, and received high-flow oxygen support.
A suspected bile leak with abdominal fluid collection was managed using antibiotics and ultrasound-guided drainage. She also developed respiratory distress requiring temporary intubation and bronchoscopy. Her condition gradually improved, and she was discharged after achieving clinical stability.
Discharge Medications
At discharge, the infant was prescribed medicines to protect the transplanted liver and prevent rejection, prevent infection, provide gastric protection, control seizures, reduce fever, relieve pain, prevent vomiting, and support nutrition and recovery.
Advice on Discharge
The parents were advised to give all prescribed medicines on time, maintain proper hygiene, protect the infant from infections, and follow feeding and wound-care instructions. Regular follow-up was advised to monitor liver function, adjust anti-rejection medicines, and assess the child’s recovery.
Emergency Care
The parents were advised to contact the emergency ward at PACE Hospitals in case of any emergency or development of symptoms, such as fever, repeated vomiting, poor feeding, breathing difficulty, worsening jaundice, unusual drowsiness, seizures, reduced urine output, bleeding, wound or drain-related concerns, or any sudden deterioration.
Review and Follow-Up Notes
The parents were advised to attend regular follow-up visits with the Surgical Gastroenterologist in Hyderabad at PACE Hospitals for monitoring transplanted liver function, adjusting anti-rejection medicines, assessing recovery, and identifying early signs of infection, rejection, bile leakage, or respiratory complications.
Conclusion
This case highlights the successful management of progressive liver dysfunction following Kasai surgery for Type 1 extrahepatic biliary atresia through Living Donor Liver Transplantation using a left lateral segment graft donated by the infant’s mother. Despite postoperative seizures, a suspected bile leak, and respiratory complications, the infant improved with coordinated specialist care and was discharged in stable condition with medications and follow-up advice.
Managing Postoperative Complications After Paediatric Liver Transplantation
Recovery after paediatric liver transplantation requires close monitoring because infants may develop breathing difficulties, seizures, infections, bile leakage, or other postoperative complications. Early recognition and prompt treatment are important for protecting the transplanted liver and supporting the child’s recovery. Postoperative care may include ventilator or oxygen support, liver Doppler monitoring, infection control, drainage of abdominal fluid collections, neurological assessment, and adjustment of anti-rejection medicines. Regular follow-up with a liver transplant doctor/specialist helps monitor graft function, identify complications early, and support the child’s long-term growth and recovery.
Frequently Asked Questions (FAQs)
What is extrahepatic biliary atresia in infants?
Extrahepatic biliary atresia is a condition present from early infancy in which the bile ducts outside the liver become blocked, damaged, or do not develop normally. As a result, bile cannot flow from the liver into the intestine. The trapped bile gradually damages the liver and may lead to scarring, cirrhosis, and liver failure if not treated promptly.
What do clay-coloured stools indicate in a baby with jaundice?
Clay-coloured, pale, grey, or white stools may indicate that bile is not reaching the intestine. Bile normally gives stools their brown colour. In a baby with jaundice, persistently pale stools can be an important warning sign of biliary atresia or another condition blocking bile flow and need to be evaluated promptly.
What is the Kasai procedure, and why is it performed?
The Kasai procedure is an operation performed for biliary atresia. During the procedure, the damaged bile ducts are removed, and a section of the intestine is connected directly to the liver to create a new pathway for bile drainage. It can slow liver damage when performed early, but some children may still develop progressive liver disease and later require transplantation.
How is graft blood flow monitored after liver transplantation?
Blood flow through the transplanted liver is commonly monitored using a liver Doppler ultrasound. This painless and non-invasive test checks blood flow through the hepatic artery, portal vein, and hepatic veins. It helps doctors identify possible narrowing, blockage, or clot formation in the graft vessels at an early stage.
Why do infants require ventilator support after liver transplant surgery?
Many infants require temporary ventilator support immediately after liver transplantation because the operation is lengthy and is performed under general anaesthesia. Ventilation supports breathing while the child recovers from anaesthesia and allows the medical team to closely monitor oxygen levels, graft function, fluid balance, and overall stability. The breathing tube is removed once the infant can breathe safely without assistance.
How are seizures managed after liver transplant surgery?
A seizure after liver transplantation requires immediate medical assessment. Doctors first stabilise the child’s breathing and oxygen levels and check for causes such as low oxygen, abnormal blood sugar or electrolytes, infection, bleeding, or side effects of anti-rejection medicines. Medicines may be given to stop and prevent further seizures, with paediatric and neurological monitoring continued as required.
What precautions should parents follow after a paediatric liver transplant?
Parents should give all prescribed medicines at the correct time and never alter anti-rejection medicines without medical advice. Good hand hygiene, safe food preparation, proper wound care, and avoiding close contact with unwell people are important. Follow-up visits and blood tests must not be missed, and any changes in feeding, activity, temperature, urine, stool, or wound condition.
How does biliary atresia gradually damage the liver?
When the bile ducts are blocked, bile becomes trapped inside the liver. This causes inflammation and ongoing damage to liver cells and bile ducts. Over time, scar tissue develops and may progress to cirrhosis, portal hypertension, poor growth, and liver failure. Early treatment can improve bile drainage, but liver disease may continue to progress in some children.
What signs may indicate graft rejection in an infant?
Possible signs of liver graft rejection may include fever, poor feeding, unusual irritability or tiredness, vomiting, abdominal discomfort or swelling, and worsening jaundice. However, rejection may sometimes cause no clear symptoms and may first be detected through abnormal liver function tests.
What may cause fever after paediatric liver transplantation?
Fever after liver transplantation may be caused by bacterial, viral, or fungal infection, inflammation around the surgical site, respiratory infection, bile leakage, or occasionally graft rejection. Because anti-rejection medicines weaken the immune response, even a mild fever should be reported promptly. The transplant team may perform blood tests, cultures, scans, and other investigations to identify the cause and provide appropriate treatment.
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