Successful Paediatric Liver Transplantation for Biliary Atresia Following Kasai Procedure
PACE Hospitals
PACE Hospitals’ expert Liver Transplant team successfully performed Paediatric Liver Transplantation (PLT) on a 7-month-old female infant diagnosed with congenital extrahepatic biliary atresia Type I with secondary biliary cirrhosis following a previous Kasai procedure. 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 7-month-old female infant presented to the Liver Transplant Department at PACE Hospitals, Hitech City, Hyderabad, with jaundice for the past 6 months. The jaundice showed a gradual progression with increasing bilirubin levels.
Past Medical History
The child was a known case of Type I extrahepatic biliary atresia, identified early in life with jaundice, clay-coloured stools, and hepatomegaly. The infant had undergone a Kasai procedure approximately 4 months before presentation.
During the previous Kasai procedure, gross hepatomegaly with hard liver consistency, neovascularisation, and chronic cholecystitis were noted. Despite the earlier surgery, the child developed progressive liver damage and secondary biliary cirrhosis.
On Examination
On examination, the child was conscious, coherent, and alert, with evident icterus indicating yellowish discolouration of the eyes. She appeared clinically unwell in the background of long-standing jaundice and progressive liver dysfunction. Abdominal and systemic examinations were performed to assess liver enlargement, clinical severity, overall condition, and suitability for paediatric liver transplantation.
Diagnosis
Upon admission to PACE Hospitals, the liver transplant team conducted a comprehensive evaluation based on the infant’s long-standing jaundice, progressive rise in bilirubin levels, and previous history of Type I extrahepatic biliary atresia.
A detailed medical history, physical examination, blood tests, liver function assessment, liver biopsy, and other required investigations were performed to evaluate the severity of liver damage. The child had previously undergone Kasai hepatoportoenterostomy approximately four months earlier. During the earlier surgery, gross hepatomegaly with hard liver consistency, neovascularisation, and chronic cholecystitis were noted.
Based on the clinical history and investigation findings, the infant was diagnosed with congenital Type I biliary atresia with secondary biliary cirrhosis. The Paediatric End-Stage Liver Disease (PELD) score was 30, indicating advanced liver disease requiring urgent specialist care.
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), along with cross-consultation with Dr. Govind Verma (Senior Consultant Interventional Gastroenterologist and Hepatologist), and Dr. Suresh Kumar S (Consultant Surgical Gastroenterologist), the infant’s condition was reviewed to decide the most appropriate treatment plan.
The child had congenital Type I extrahepatic biliary atresia with secondary biliary cirrhosis and a PELD score of 30. Despite undergoing Kasai hepatoportoenterostomy earlier in life, she continued to have long-standing jaundice and progressive liver dysfunction.
Considering the severity of liver damage and the risk of further complications, it was determined that paediatric liver transplantation was the appropriate treatment to save the child’s life and restore liver function.
The infant was maintained on required supportive care while transplant planning was completed. After necessary investigations, medical fitness assessment, pre-anaesthesia evaluation, and required clearances, paediatric liver transplantation using a left lateral segment graft was planned.
The family was counselled regarding the child’s condition, need for transplantation, 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 Paediatric Liver Transplantation (PLT) 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 investigations, pre-anaesthesia assessment, medical clearances, and supportive optimisation, the infant was taken up for surgery under general anaesthesia with continuous monitoring.
- Graft Preparation: A left lateral segment liver graft was prepared for transplantation, as this graft size is commonly suitable for paediatric liver transplant recipients.
- 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 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. Biliary reconstruction was performed to allow 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 for close postoperative monitoring.
Postoperative Care
After surgery, the infant was closely monitored for transplanted liver function, blood flow to the graft, breathing, infection, abdominal symptoms, and overall recovery. Postoperative liver Doppler findings were satisfactory, indicating good graft blood flow.
As the infant had advanced liver disease, previous Kasai surgery, secondary biliary cirrhosis, and complex paediatric transplant anatomy, close monitoring was especially important. In such high-risk paediatric liver transplant cases, postoperative abdominal fluid collection, bile drainage-related concerns, infection, adhesions, or bowel-related complications may occur despite a successfully completed transplant procedure.
On the fifth day after transplantation, ultrasound showed fluid collection in the left lumbar and right subdiaphragmatic areas. Percutaneous transhepatic biliary drainage (PTBD) was performed to drain the collection and relieve pressure.
On the sixth postoperative day, the infant developed tachycardia, abdominal distension, and guarding, which raised suspicion of a serious abdominal complication. The liver transplant team promptly planned emergency exploratory laparotomy. Jejunal perforation was identified and successfully treated with primary closure. The infant continued to receive intensive monitoring, medicines to protect the transplanted liver, infection prevention, supportive care, and parental counselling. After achieving hemodynamic stability, she was discharged with medications and follow-up advice.
Discharge Medications
At discharge, the infant was prescribed medicines to protect the transplanted liver and prevent rejection, medicines to prevent infection, gastric protection, nutritional support, vomiting control, pain relief, fever control, and other supportive medicines as required.
Advice on Discharge
The parents were advised to give all prescribed medicines on time and not stop anti-rejection medicines without medical advice. They were instructed to maintain proper hygiene, protect the infant from infections, follow feeding and wound-care instructions, and attend regular follow-up visits.
Emergency Care
The parents were advised to contact the emergency ward at PACE Hospitals in case of fever, repeated vomiting, poor feeding, breathing difficulty, worsening jaundice, unusual drowsiness, reduced urine output, bleeding, abdominal distension, wound or drain-related concerns, or any sudden deterioration in the infant’s condition.
Review and Follow-Up Notes
The parents were advised to attend regular follow-up visits with the Surgical Gastroenterologist in Hyderabad at PACE Hospitals to monitor transplanted liver function, graft recovery, wound healing, infection risk, medicine adjustment, and overall growth and recovery.
Conclusion
This case highlights the successful management of congenital Type I biliary atresia with secondary biliary cirrhosis in a 7-month-old female infant through Paediatric Liver Transplantation using a left lateral segment graft.
Although the transplant surgery was completed without reported intraoperative complications, the infant developed postoperative abdominal fluid collection and a rare jejunal perforation, which were managed through percutaneous transhepatic biliary drainage insertion and emergency exploratory laparotomy with primary closure. After intensive monitoring, supportive care, medications, and counselling, the infant achieved hemodynamic stability and was discharged with follow-up advice.
Importance of Early Recognition of Complications After Paediatric Liver Transplantation
Recovery after paediatric liver transplantation needs close monitoring, especially in infants with advanced liver disease. Complications such as fluid collection, bile drainage issues, infection, abdominal distension, or bowel-related problems may occur after surgery. In this case, timely imaging helped detect abdominal fluid collection, and later symptoms such as tachycardia, abdominal distension, and guarding helped identify jejunal perforation. Prompt drainage, emergency surgery, intensive monitoring, and follow-up with a Liver transplant doctor/specialist supported safe recovery.
Frequently Asked Questions (FAQs)
Why does biliary atresia cause jaundice in babies?
Biliary atresia causes jaundice because the bile ducts are blocked, damaged, or absent, so bile cannot flow properly from the liver to the intestine. When bile flow is blocked, bilirubin builds up in the blood and causes yellow discoloration of the eyes and skin. This is why babies with biliary atresia often develop persistent jaundice and pale or clay-coloured stools.
What is secondary biliary cirrhosis?
Secondary biliary cirrhosis is liver scarring that develops when bile flow remains blocked for a long time. In biliary atresia, bile gets trapped inside the liver and gradually damages liver cells. Over time, this can lead to fibrosis, cirrhosis, poor liver function, and the need for liver transplantation.
Can liver damage continue even after Kasai surgery?
Yes, liver damage can continue even after Kasai surgery in some children. The Kasai procedure helps improve bile drainage, but it may not fully stop disease progression in every child. If jaundice, poor bile flow, infection, or liver scarring continues, the child may eventually require liver transplantation.
When does a child with biliary atresia need liver transplantation?
A child with biliary atresia may need liver transplantation when the liver becomes severely damaged or when the Kasai procedure does not provide enough bile drainage. Transplant may be advised if the child has progressive jaundice, cirrhosis, poor growth, repeated infections, fluid buildup, bleeding problems, or worsening liver function.
What does fluid collection after a liver transplant mean?
Fluid collection after a liver transplant means fluid has accumulated inside the abdomen after surgery. This may happen due to bile leakage, infection, bleeding, lymphatic fluid, or postoperative inflammation. Doctors usually evaluate it with ultrasound or other imaging and decide whether observation, drainage, antibiotics, or further treatment is needed.
Why was PTBD insertion done after liver transplantation?
PTBD insertion was done to drain the collected fluid and relieve pressure after the transplant. In some cases, bile drainage-related issues or suspected bile leak can lead to fluid collection inside the abdomen. PTBD helps drain bile or fluid safely, supports healing, and helps doctors manage the complication without delay.
What is jejunal perforation after liver transplant?
Jejunal perforation means there is a hole or tear in the jejunum, which is the middle part of the small intestine. After liver transplantation, this is a rare but serious complication that can cause abdominal distension, increased heart rate, guarding, infection, or sudden clinical worsening. It usually needs urgent surgical evaluation and repair, as was done in this case.
What precautions should parents follow after paediatric liver transplant?
Parents should give all prescribed medicines exactly on time, especially anti-rejection medicines, and should not stop them without medical advice. They should maintain good hygiene, protect the child from infections, follow feeding and wound-care instructions, and attend all scheduled follow-up visits. Regular monitoring helps check liver function, medicine dose, growth, and early signs of complications.
What warning signs require emergency care after paediatric liver transplant?
Parents should seek emergency care if the child develops fever, repeated vomiting, poor feeding, breathing difficulty, worsening jaundice, unusual sleepiness, reduced urine output, bleeding, abdominal distension, wound redness, wound discharge, or any sudden deterioration. These symptoms may indicate infection, rejection, bile drainage problems, abdominal complications, or other serious issues that need urgent attention.
Can infants recover well after liver transplant complications are treated on time?
Yes, infants can recover well when complications after liver transplantation are identified early and treated promptly. Close ICU monitoring, timely imaging, drainage procedures, emergency surgery when needed, infection control, and regular follow-up can help protect the transplanted liver and support recovery. In this case, early recognition and timely treatment of complications helped the infant achieve hemodynamic stability before discharge.
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