Familial occurrence of congenital biliary dilatation diagnosed at adult age

• Kogure Michio

  Department of Surgery, Umeda Hospital

• Sugiyama Masanori

  Department of Surgery, Kyorin University School of Medicine

• Satake Ryosuke

  Department of Surgery, Umeda Hospital

• Hosouchi Yasuo

  Department of Surgery, Saiseikai Maebashi Hospital

Bibliographic Information

Other Title

• 成人発症の先天性胆道拡張症の1家族例―本邦報告例の検討―

Abstract

Familial occurrence of congenital biliary dilatation (CBD) has been reported, but that in adult cases is rarely reported. Although CBD is sometimes associated with bile duct cancer, coexistence of cancer, dysplasia and hyperplasia of the bile duct has never been documented. We experienced a case of mother and daughter with CBD who were diagnosed at adult age. A 70-year-old woman presented with jaundice and underwent extrahepatic bile duct resection for Todani Type IV CBD and anomalous pancreatobiliary junction. Pathological examination revealed cancer, dysplasia and hyperplasia in the dilated bile duct. Her daughter had undergone surgery for Todani Type I CBD without biliary neoplasia at the age of 37 years. In 32 reported cases of CBD from 16 families, only two cases from one family were diagnosed at adult age and none had biliary tract cancer. Because biliary tract cancer associated with CBD has poor prognosis, CBD should be treated before cancer develops. Screening for CBD is recommended to relatives with family history of CBD.<br>

Journal

• Tando

  Tando 25 (1), 120-126, 2011

  Japan Biliary Association

Keywords

• congenital bile duct dilatation
• occurrence within the family
• oncogenesis

Details

• CRID: 1390001204347761280
• NII Article ID: 130004545381
• NII Article ID: 10030362088
• NII Book ID: AN10062001
• DOI: 10.11210/tando.25.120
• ISSN: 18836879
• ISSN: 09140077
• Web Site: https://search.jamas.or.jp/link/ui/2011162816
• Text Lang: ja
• Data Source:
  • JaLC
  • CiNii Articles
• Abstract License Flag: Disallowed