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[Primary sclerosing cholangitis].
S Grüne1, B Müllhaupt, D Moradpour
1Departement für Innere Medizin, Universitätsspital Zürich.
Deutsche Medizinische Wochenschrift (1946)
|July 12, 1991
Summary
A 40-year-old patient experienced recurrent cholestatic jaundice, ultimately diagnosed with cholangiocarcinoma after initial sclerosing cholangitis treatment failed. Liver transplantation was required but ultimately unsuccessful, highlighting the aggressive nature of this biliary tract cancer.
Area of Science:
- Hepatology
- Gastroenterology
- Oncology
Background:
- A 40-year-old presented with a 9-year history of unexplained recurrent epigastric colic and transient cholestatic jaundice.
- Progressive, painless jaundice with elevated liver enzymes (alkaline phosphatase 900 U/l, direct bilirubin 305 mumol/l) prompted hospitalization.
Observation:
- Sonography revealed hepatomegaly (17 cm), dilated intrahepatic bile ducts, and a 1 cm echodense area at the common hepatic duct bifurcation.
- Endoscopic retrograde cholangiopancreatography suggested sclerosing cholangitis, confirmed by liver biopsy.
Findings:
- Initial treatment with ursodeoxycholic acid for presumed sclerosing cholangitis led to temporary improvement.
- The patient developed severe cholangiosepsis and acute renal failure, necessitating a liver transplant.
- Histological examination of the explant revealed cholangiocarcinoma at the common hepatic duct bifurcation.
Implications:
- This case underscores the importance of considering cholangiocarcinoma in patients with prolonged unexplained cholestatic jaundice, even after initial diagnosis of benign biliary disease.
- Despite liver transplantation, the patient experienced graft rejection and mortality, emphasizing the challenges in managing advanced biliary tract cancers.
- The diagnostic pathway highlights the limitations of initial investigations and the need for thorough evaluation in complex hepatobiliary presentations.