Angeborene Anomalien und Malformationen der Gallenwege
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- 作者：Prof. Dr. J. Rädle
- 刊名：Der Gastroenterologe
- 出版年：2016
- 出版时间：July 2016
- 年：2016
- 卷：11
- 期：4
- 页码：256-267
- 全文大小：1,635 KB
- 刊物主题：Gastroenterology; General Practice / Family Medicine; Internal Medicine; Hepatology; Infectious Diseases; Oncology;
- 出版者：Springer Berlin Heidelberg
- ISSN：1861-969X
- 卷排序：11
文摘
Congenital anomalies and malformations may affect the intra- and/or extrahepatic bile duct system and present with different symptoms and impact depending on the specific type. Mere anomalies of the bile duct system are common and are generally asymptomatic but may cause substantial problems during hepatobiliary surgery. Severe malformations such as complete or partial biliary atresia are rare and present with prolonged and progressive icterus in the neonatal period requiring surgical reconstruction of the bile duct system or even liver transplantation. Fibrocystic diseases affecting the liver are caused by ductal plate malformations and are characterized by dilation of the intrahepatic bile duct system and focal liver fibrosis. Autosomal recessive and dominant polycystic kidney disease, progressive destructive cholangitis, congenital hepatic fibrosis, Caroli disease and biliary (von Meyenburg complex) as well as mesenchymal hamartomas belong to this heterogeneous group of disorders. Choledochal cysts are usually diagnosed in childhood; however, in about 20 % of patients they are detected in adulthood. According to Todani’s classification they are graduated into 5 categories. Choledochal cysts are common in Asia and show a high prevalence in Japan. An anomalous long common orifice of the extrahepatic bile and pancreatic duct is considered an important pathoanatomical substrate. Adults mainly suffer from symptoms due to cholelithiasis, pancreatitis, biliary cirrhosis and carry a high risk for developing cholangiocarcinoma. Therefore, early and complete excision of the choledochal cysts and long-term surveillance are warranted.KeywordsHereditary diseases of the biliary systemCholedochal cystsTodani’s classificationCaroli diseaseCholangiocarcinoma