S3291 Biliary Cast Syndrome and Secondary Sclerosing Cholangitis Following Trauma and Amputation in a Young Patient With Metabolic Syndrome

Introduction: Biliary cast syndrome (BCS) is a rare condition characterized by intraductal casts causing biliary obstruction and cholestasis. While first described in liver transplant recipients, it is increasingly recognized in critically ill, non-transplant patients. Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is a progressive biliary injury commonly triggered by ischemia, bile stasis, and infection, with drug-induced liver injury potentially exacerbating the course. We present a rare case of BCS and SSC-CIP in a young, non-transplant patient following trauma and amputation. Case Description/Methods: A 39-year-old man with metabolic syndrome (body mass index 47), chronic kidney disease, and non-ischemic cardiomyopathy sustained a traumatic leg fracture complicated by compartment syndrome and popliteal artery injury. He underwent vascular bypass, serial debridements, and right above-knee amputation. His intensive care unit course was complicated by acute respiratory distress syndrome, ventilator-associated pneumonia, and surgical site infections with VRE and XDR Acinetobacter, requiring prolonged treatment with hepatotoxic agents (linezolid, daptomycin). Five months later, he developed jaundice and pruritus. magnetic resonance cholangiopancreatography (MRCP) showed intrahepatic ductal dilation and wall thickening. Liver biopsy demonstrated cholestasis, bile infarcts, ductular reaction, and macrovesicular steatosis (35–40%) without fibrosis. Autoimmune and viral workup was negative. Endoscopic retrograde cholangiopancreatography (ERCP) revealed long, fibrous biliary casts consistent with BCS, which were extracted with balloon sweep. Biliary stenting was performed, and a subsequent ERCP addressed hemobilia from an ulcerated ampulla. Discussion: This case highlights multifactorial SSC-CIP triggered by ischemia, bile stasis, and infection, with hepatotoxic antibiotics possibly aggravating bile duct injury. Histology ruled out congestive hepatopathy and confirmed SSC-CIP. Though ERCP provided temporary improvement, persistent MRSA bacteremia and poor functional status precluded liver transplant, the only definitive therapy for SSC-CIP. Clinicians should maintain a high suspicion for SSC-CIP in intensive care unit survivors with cholestasis. Early recognition may improve outcomes, but the prognosis remains poor without transplant.

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Publication Details

Journal
The American Journal of Gastroenterology
Published
2025-10-01
DOI
https://doi.org/10.14309/01.ajg.0001140624.26360.64
Primary Topic
Medical Imaging and Pathology Studies
Type
article
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article

S3291 Biliary Cast Syndrome and Secondary Sclerosing Cholangitis Following Trauma and Amputation in a Young Patient With Metabolic Syndrome

Carl Manzo, Timothy J. Barreiro, Enayat Shahidifar, Arman Hemmat et al.
The American Journal of Gastroenterology
Medical Imaging and Pathology Studies
article

S3291 Biliary Cast Syndrome and Secondary Sclerosing Cholangitis Following Trauma and Amputation in a Young Patient With Metabolic Syndrome

Carl Manzo, Timothy J. Barreiro, Enayat Shahidifar, Arman Hemmat, Marlena Lesniowska
article en

Abstract

Introduction: Biliary cast syndrome (BCS) is a rare condition characterized by intraductal casts causing biliary obstruction and cholestasis. While first described in liver transplant recipients, it is increasingly recognized in critically ill, non-transplant patients. Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is a progressive biliary injury commonly triggered by ischemia, bile stasis, and infection, with drug-induced liver injury potentially exacerbating the course. We present a rare case of BCS and SSC-CIP in a young, non-transplant patient following trauma and amputation. Case Description/Methods: A 39-year-old man with metabolic syndrome (body mass index 47), chronic kidney disease, and non-ischemic cardiomyopathy sustained a traumatic leg fracture complicated by compartment syndrome and popliteal artery injury. He underwent vascular bypass, serial debridements, and right above-knee amputation. His intensive care unit course was complicated by acute respiratory distress syndrome, ventilator-associated pneumonia, and surgical site infections with VRE and XDR Acinetobacter, requiring prolonged treatment with hepatotoxic agents (linezolid, daptomycin). Five months later, he developed jaundice and pruritus. magnetic resonance cholangiopancreatography (MRCP) showed intrahepatic ductal dilation and wall thickening. Liver biopsy demonstrated cholestasis, bile infarcts, ductular reaction, and macrovesicular steatosis (35–40%) without fibrosis. Autoimmune and viral workup was negative. Endoscopic retrograde cholangiopancreatography (ERCP) revealed long, fibrous biliary casts consistent with BCS, which were extracted with balloon sweep. Biliary stenting was performed, and a subsequent ERCP addressed hemobilia from an ulcerated ampulla. Discussion: This case highlights multifactorial SSC-CIP triggered by ischemia, bile stasis, and infection, with hepatotoxic antibiotics possibly aggravating bile duct injury. Histology ruled out congestive hepatopathy and confirmed SSC-CIP. Though ERCP provided temporary improvement, persistent MRSA bacteremia and poor functional status precluded liver transplant, the only definitive therapy for SSC-CIP. Clinicians should maintain a high suspicion for SSC-CIP in intensive care unit survivors with cholestasis. Early recognition may improve outcomes, but the prognosis remains poor without transplant.

The American Journal of GastroenterologyVol. 120(10S2)
Openalex Percentile: Top 56%
Medical Imaging and Pathology Studies
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