Reversal of surgical biliary diversion with ileal bile acid transport inhibitors: A new chapter in progressive familiar intrahepatic cholestasis type 1 management?

Progressive Familial Intrahepatic Cholestasis type 1 (PFIC1) is a multisystem disorder. Although liver transplant (LT) resolves the hepatic disease, post-LT complications may occur, including severe enteropathy and graft steatosis caused by impaired bile acids handling by the native intestine. Surgical biliary diversion (SBD) is commonly used to alleviate these complications but may result in long-term morbidity, such as fat-soluble vitamin deficiency and essential fatty acids malabsorption. We report a PFIC1 patient who developed post-LT protein-losing enteropathy, initially managed with SBD. While effective, SBD led to severe fat malabsorption requiring intravenous lipid supplementation. After initiation of ileal bile acid transport inhibitor (IBATi) therapy, SBD was surgically reversed without recurrence of enteropathy. This case highlights IBATi as a potential alternative to SBD and as a strategy to reverse prior surgical interventions and improve quality of life.