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Total Internal Biliary Diversion for Post-Liver Transplant PFIC-1-Related Allograft Injury
Naresh Shanmugam1, Jagadeesh Menon1, Mukul Vij1
1The Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, Bharath Institute of Higher Education & Research, Chennai, India.
Insights
Progressive familial intrahepatic cholestasis type 1 (PFIC-1) complications after liver transplantation (LT), including steatohepatitis and diarrhea, can be effectively managed. Total internal biliary diversion offers a safe solution for these challenging post-transplant conditions.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Transplantation Surgery
Background:
- Progressive familial intrahepatic cholestasis type 1 (PFIC-1) often leads to complications like steatohepatitis and refractory diarrhea in children post-liver transplantation (LT).
- Standard medical management, including bile acid binders, is frequently insufficient, potentially leading to allograft dysfunction and loss.
Observation:
- A 7-year-old boy with PFIC-1 experienced worsening liver function, steatohepatitis, and intractable diarrhea 5 years after LT.
- Conventional therapies failed to resolve these progressive post-transplant complications.
Findings:
- A total internal biliary diversion was performed to interrupt the enterohepatic circulation, resolving the patient's refractory diarrhea and normalizing transaminases.
- Follow-up liver biopsy after one year demonstrated complete resolution of steatohepatitis, with sustained clinical improvement and weight gain at 18 months.
Implications:
- Total internal biliary diversion is a safe and effective therapeutic option for managing severe post-LT complications, specifically allograft injury secondary to PFIC-1 pathology.
- This surgical approach offers a promising strategy to improve outcomes and prevent graft loss in pediatric liver transplant recipients with PFIC-1.
Abstract:
Steatohepatitis and diarrhea are well-known complications in children undergoing liver transplantation (LT) for progressive familial intrahepatic cholestasis (PFIC) type 1. Despite medical management with bile acid binders, the condition is progressive and can be associated with allograft loss. We report the case of a seven-year-old boy who underwent LT at the age of 2 years for PFIC type 1. Over the next five years, he developed refractory diarrhea, emaciation, worsening liver function, and steatohepatitis. Aiming to interrupt the enterohepatic circulation, at the age of 7 years, he underwent a total internal biliary diversion. The patient's postprocedure period was uneventful. His diarrhea settled and the transaminases normalized his follow-up liver biopsy after a year showed a complete resolution of steatohepatitis. At 18 months' follow-up, he has gained weight and remains asymptomatic. In this report, we show post-LT complications especially allograft injury related to the pathology of PFIC-1 can be safely and effectively managed by performing a total internal biliary diversion.
