[Upper abdominal pain with recurrent cholangitis and pyelonephritis].
D Maxien1, M F Reiser, S Wirth
1Institut für Klinische Radiologie, Klinikum der Ludwig-Maximilians-Universität München, Campus Grosshadern, Marchioninistr. 15, 81377, München, Deutschland. daniel.maxien@med.uni-muenchen.de
Der Radiologe
|February 23, 2010
Summary
Caroli
Area of Science:
- Hepatology
- Radiology
- Medical Genetics
Background:
- Caroli's disease is a rare congenital disorder characterized by cystic dilatation of the intrahepatic bile ducts.
- It often presents with recurrent cholangitis, abdominal pain, and elevated liver enzymes.
- Associated renal cystic disease is common.
Observation:
- A 38-year-old male presented with right upper quadrant pain, elevated C-reactive protein, and creatinine.
- Duplex sonography revealed intrahepatic cystic structures and renal cysts.
- MRI demonstrated dilated bile ducts around portal vein branches, forming the "central dot sign".
Findings:
- The constellation of imaging findings, including the "central dot sign," intrahepatic biliary dilatation, and cystic renal disease, confirmed the diagnosis of Caroli's disease.
- Recurrent cholangitis was a significant clinical feature.
- The patient's presentation was consistent with advanced liver involvement.
Implications:
- Early diagnosis of Caroli's disease is crucial for timely intervention and management.
- Liver transplantation is a definitive treatment option for advanced cases, effectively managing symptoms and preventing cholangiocarcinoma.
- This case highlights the importance of integrating imaging findings with clinical presentation for accurate diagnosis of rare congenital biliary disorders.
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