Secondary sclerosing cholangitis after long-term treatment in an Intensive care unit: clinical presentation, endoscopic findings, treatment, and follow-up

Background and Study Aims: We present ten patients who developed secondary sclerosing cholangitis following long-term treatment in an intensive care unit (ICU) between 1999 and 2004.
Patients and Methods: Ten consecutive patients who had no evidence suggestive of pre-existing hepatobiliary disease were admitted to an ICU because of trauma (n = 5), intracerebral hemorrhage (n = 3), or nonabdominal postsurgical complications (n = 2). All the patients had required treatment with long-term ventilation, catecholamines, total parenteral nutrition, and several antimicrobial agents.
Results: Cholestasis was first noted within 11 days after the initial insult. Endoscopic retrograde cholangiopancreatography (ERCP), performed after a median follow-up of 69 days, revealed multifocal stricturing and beading of the intrahepatic bile ducts, and attenuation of the peripheral branches. In all the patients, the bile ducts were partially filled by black-pigmented thrombotic material. All the patients underwent endotherapy, which comprised sphincterotomy and removal of the occluding material, in an attempt to improve biliary drainage; the treatment had to be repeated in seven of the ten patients. After a median follow-up period of 21 months, despite transient clinical improvement following endotherapy, complete recovery has not been achieved in any of the patients and so far one patient has had to undergo orthotopic liver transplantation as a result of end-stage liver disease.
Conclusions: The development of secondary sclerosing cholangitis in patients who have received long-term treatment in an ICU is a rare event of unknown pathophysiology, but patients demonstrate characteristic findings on ERCP. It is not known whether endotherapy can delay the progress of the condition in the long term.

References

• 1 Amor A, Chapoutot C, Michel J. et al . Secondary sclerosing cholangitis.  Presse Med. 1995;  24 948-952
• 2 Nakanuma Y, Tsuneyama K, Harada K. Pathology and pathogenesis of intrahepatic bile duct loss.  J Hepatobiliary Pancreat Surg. 2001;  8 303-315
• 3 Engler S, Elsing C, Flechtenmacher C. et al . Progressive sclerosing cholangitis after septic shock: a new variant of vanishing bile duct disorders.  Gut. 2003;  52 688-693
• 4 Lazaridis K N, Wiesner R H, Porayko M K. et al .Primary sclerosing cholangitis. In: Schiff ER, Sorrell MF, Maddrey WC (eds) Schiff’s diseases of the Liver. 8th edn. Philadelphia; Lippincott-Raven 1999: 649-669
• 5 Li-Yeng C, Goldberg H I. Sclerosing cholangitis: broad spectrum of radiographic features.  Gastrointest Radiol. 1984;  9 39-47
• 6 Abou-Rebyeh H, Veltzke-Schlieker W, Radke C. et al . Complete bile duct sequestration after liver transplantation, caused by ischemic-type biliary lesions.  Endoscopy. 2003;  35 616-620
• 7 Scheppach W, Druge G, Wittenberg G. et al . Sclerosing cholangitis and liver cirrhosis after extrabiliary infections: report on three cases.  Crit Care Med. 2001;  29 438-441
• 8 Mohi-ud-din R, Lewis J H. Drug- and chemical-induced cholestasis.  Clin Liver Dis. 2004;  8 95-132
• 9 Tanaka Y, Koshiyama H, Nakao K. et al . Rapid progress of acute suppurative cholangitis to secondary sclerosing cholangitis sequentially followed-up by endoscopic retrograde cholangiography.  Endoscopy. 2001;  33 633-635
• 10 Richardet J P, Lons T, Sibony M. et al . Secondary sclerosing cholangitis and chemo-embolization with lipiodol.  Gastroenterol Clin Biol. 1994;  18 168-171
• 11 Moore F A. The role of the gastrointestinal tract in postinjury multiple organ failure.  Am J Surg. 1999;  178 449-453
• 12 Quigley E, Marsh M, Shaffer J, Markin R S. Hepatobiliary complications of total parenteral nutrition.  Gastroenterology. 1993;  104 286-301
• 13 Velayudham L S, Farrell G C. Drug-induced cholestasis.  Expert Opin Drug Saf. 2003;  2 287-304
• 14 Deviere J. Therapeutic endoscopy and primary sclerosing cholangitis.  Endoscopy. 1996;  28 576-577
• 15 Stiehl A, Rudolph G, Sauer P. et al . Efficacy of ursodeoxycholic acid treatment and endoscopic dilation of major duct stenosis in primary sclerosing cholangitis: an 8-year prospective study.  J Hepatol. 1997;  26 560-566