Ziv Ben-Ari1, David Levingston2, Ella Weitzman3, Yael Haviv-Yadid2, Oranit Cohen-Ezra1, Peretz Weiss3, Eli Zuckerman4, Yael Inbar5, Michal Amitai5, Uri Rimon5, Maya Goder6, Josef Haik6. 1. Liver Disease Center, Sheba Medical Center, Ramat Gan and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel. 2. Intensive Care Unit, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel. 3. Liver Disease Center, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel. 4. Liver Unit, Carmel Medical Center, Haifa, Israel. 5. Department of Radiology, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel. 6. Department of Plastic Surgery, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
Abstract
BACKGROUND AND AIMS: Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is a relatively new previously unrecognized entity which may lead to severe biliary disease with rapid progression to cirrhosis. We present for the first time a case series of patients with rapidly progressive SSC-CIP requiring aggressive intensive care treatment following major burn injury. RESULTS: SSC-CIP was diagnosed in 4 consecutive patients hospitalized due to major burn injuries at our Intensive Care Unit (ICU). SSC-CIP was diagnosed when ERCP (n = 1) or MRCP (n = 3) demonstrated irregular intrahepatic bile ducts with multiple strictures and dilatations and, when a liver biopsy (n = 3) demonstrated severe cholestasis and bile duct damage. All patients were males; none of whom had pre-existing liver disease. Ages: 18-56 y. All patients suffered from severe (grade 2-3) burn injuries with total burn surface area ranging from 35 to 95%. Mean length of ICU hospitalization was 129.2 ± 53.0 days. All patients required mechanical ventilation (with a mean PEEP of 8.4 ± 2.1 cm H2O) and the administration of catecholamines for hemodynamic stabilization. All patients demonstrated severe cholestasis. Blood cultures and cultures from drained liver abscesses grew hospital acquired multiple resistant bacteria. Liver cirrhosis developed within 12 months. One patient underwent orthotopic liver transplantation. Two patients (50%) died. In conclusion, SSC-CIP following major burn injury is a rapidly progressive disease with a poor outcome. Liver cirrhosis developed rapidly. Awareness of this grave complication is needed for prompt diagnosis and considerations of a liver transplantation.
BACKGROUND AND AIMS: Secondary sclerosing cholangitis in critically illpatients (SSC-CIP) is a relatively new previously unrecognized entity which may lead to severe biliary disease with rapid progression to cirrhosis. We present for the first time a case series of patients with rapidly progressive SSC-CIP requiring aggressive intensive care treatment following major burn injury. RESULTS:SSC-CIP was diagnosed in 4 consecutive patients hospitalized due to major burn injuries at our Intensive Care Unit (ICU). SSC-CIP was diagnosed when ERCP (n = 1) or MRCP (n = 3) demonstrated irregular intrahepatic bile ducts with multiple strictures and dilatations and, when a liver biopsy (n = 3) demonstrated severe cholestasis and bile duct damage. All patients were males; none of whom had pre-existing liver disease. Ages: 18-56 y. All patients suffered from severe (grade 2-3) burn injuries with total burn surface area ranging from 35 to 95%. Mean length of ICU hospitalization was 129.2 ± 53.0 days. All patients required mechanical ventilation (with a mean PEEP of 8.4 ± 2.1 cm H2O) and the administration of catecholamines for hemodynamic stabilization. All patients demonstrated severe cholestasis. Blood cultures and cultures from drained liver abscesses grew hospital acquired multiple resistant bacteria. Liver cirrhosis developed within 12 months. One patient underwent orthotopic liver transplantation. Two patients (50%) died. In conclusion, SSC-CIP following major burn injury is a rapidly progressive disease with a poor outcome. Liver cirrhosis developed rapidly. Awareness of this grave complication is needed for prompt diagnosis and considerations of a liver transplantation.
Authors: Caroline Klindt; Björn-Erik Jensen; Timo Brandenburger; Torsten Feldt; Alexander Killer; Lars Schimmöller; Gerald Antoch; Tina Senff; Sandra Hauka; Jörg Timm; Bahne Hendrik Bahners; Maximilian Seidl; Irene Esposito; Tom Luedde; Johannes G Bode; Verena Keitel Journal: Clin Case Rep Date: 2021-05-24