Familial Occurrence of Pancreaticobiliary Maljunction With Type VI Choledochal Cyst: A Case Report

Muta Yuki, Uemura Sadashige, Nouso Hiroshi, Kuyama Hisako, Yamamoto Mayumi, Yoshida Atsushi

doi:10.11164/jjsps.51.7_1186

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濃厚な家族歴を有し，稀な胆管拡張形態を呈した膵・胆管合流異常の1例
症例報告濃厚な家族歴を有し,稀な胆管拡張形態を呈した膵・胆管合流異常の1例
ショウレイホウコクノウコウナカゾクレキオユウシ,マレナタンカンカクチョウケイタイオテイシタスイ・タンカンゴウリュウイジョウノ 1レイ

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Abstract

Familial pancreaticobiliary maljunction (PBM) is rare, and its genetically inheritable character is unproved. An 8-year-old girl, whose mother and maternal aunt had a history of PBM, visited our hospital for repetitive abdominal pain. Ultrasonography showed a cystic lesion of 2.5 cm diameter adjacent to the gallbladder neck, resembling a type II congenital biliary dilatation based on Todani’s classification. MRCP suggested the existence of PBM. Laparoscopic cholecystography revealed dilatation of the cystic duct, a common channel of 8 mm length, and a visualized main pancreatic duct. With the final diagnosis of type VI choledochal cyst, the laparoscopically assisted excision of the extrahepatic bile duct followed by Roux-en-Y hepaticojejunostomy was successfully performed. Inflammation was histopathologically demonstrated on the excised gallbladder and cystic duct. This is the first case report of a type VI choledochal cyst in Japan, and it showed the implication of a genetic predisposition in some types of PBM. We recommend the examination of PBM in those who have a strong family history of this anomaly.

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Journal of the Japanese Society of Pediatric Surgeons

Journal of the Japanese Society of Pediatric Surgeons 51 (7), 1186-1189, 2015

The Japanese Society of Pediatric Surgeons

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