Total internal biliary diversion during liver transplantation for type 1 progressive familial intrahepatic...

Area of Science:

• Hepatology
• Pediatric Gastroenterology
• Surgical Innovation

Background:

• Progressive familial intrahepatic cholestasis type 1 (PFIC type 1) often presents with severe cholestatic jaundice, pruritus, and growth retardation.
• Liver transplantation (LT) is a potential treatment for PFIC type 1, but complications like postoperative diarrhea and recurrent graft steatosis can occur.
• Current management strategies for PFIC type 1 require effective methods to mitigate post-LT complications.

Observation:

• A 26-month-old female with PFIC type 1, confirmed ATP8B1 defects, and elevated bilirubin underwent LT with a segment 2, 3 graft from her father.
• A transintestinal biliary diversion (TIBD) using a 35-cm jejunum conduit was performed, connecting the graft hepatic duct to the mid-transverse colon.
• Post-diversion, stools immediately became pigmented, and follow-up demonstrated resolution of jaundice and pruritus with improved stool consistency.

Findings:

• Radioisotope hepato-biliary scintigraphy confirmed unobstructed bile drainage into the colon with decreasing transit times (t½: 34, 27, and 19 minutes).
• Contrast studies at 62 and 140 days post-LT showed no colo-jejuno-hepatic reflux and a patent jejuno-colonic anastomosis.
• Graft biopsies revealed stable, low-grade macrovesicular steatosis (<20%) at multiple follow-up points (138 days and 9 months).

Implications:

• Transintestinal biliary diversion (TIBD) during LT appears to be a clinically effective, stoma-free approach for biliary diversion in PFIC type 1.
• This technique may prevent recurrent graft steatosis, a significant complication following LT for PFIC type 1.
• TIBD represents a promising surgical innovation for improving outcomes in pediatric liver transplantation for cholestatic liver diseases.