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| 篇名 |
Monolobar Caroli’s Disease:Report of Two Cases within a Family |
| 作者 |
Min-Chieh Shieh, Ching-Chung Chiang, Chi-Wen Tu,Chao-Ming Shih, Chi-Szu Liao, Chen-Hsien Su |
| 卷期/出版年月 |
40卷3期 (2007/6) |
| 頁次 |
136-141 |
| 摘要 |
Caroli’s disease is a congenital disorder characterized by segmental communicating
saccular dilatation of larger intrahepatic bile ducts. This malformation may occur in
association with autosomal dominant or recessive polycystic kidney disease of
varying severity. This study reports the successful management of two cases with
monolobar Caroli’s disease within a family by partial hepatic resection. Case one
was a 74 year-old female, who had a known diagnosis of autosomal dominant
polycystic kidney disease, and clinically manifested repeated episodes of bacterial
cholangitis and septicemia. Abdominal computed tomography and magnetic
resonance cholangiopancreatography confirmed the presence of saccular dilatation
of intrahepatic bile ducts confined to the left lobe, hepatolithiasis and
choledocholithiasis. Case two was a 43 year-old male, who was the son of case one,
had autosomal dominant polycystic kidney disease, and clinically manifested acute
bacterial cholangitis. Abdominal computed tomography demonstrated the presence
of dilated left intrahepatic bile ducts, hepatolithiasis and choledocholithiasis. Both
patients underwent cholecystectomy, choledocholithotomy and left lateral
segmentectomy and the post-operative recovery was uneventful in both cases. There
was no recurrence of cholangitis during a 14-month and a four-month follow-up,
respectively. The diagnosis of Caroli’s disease was established by imaging studies.
Magnetic resonance cholangiopancreatography is preferred and it could provide a
noninvasive, safe and accurate diagnosis of Caroli’s disease. Partial hepatic resection
may be curative in patients with Caroli’s disease confined to a single lobe or segment
of the liver. |
| 關鍵詞 |
Caroli’s disease, autosomal dominant polycystic kidney disease, hepatic resection |
| 分類 |
Case Report |
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