Tsai Ching-Chung, Huang Po-Kai, Liu Hsien-Kuan, Su Yu-Tsun, Yang Ming-Chun, Yeh Ming-Lun
Department of Pediatrics Department of Pediatric Surgery, E-Da Hospital, I-Shou University, Kaohsiung, Taiwan (R.O.C.).
Medicine (Baltimore). 2017 Oct;96(42):e8306. doi: 10.1097/MD.0000000000008306.
Choledochal cysts are a congenital disorder of the common bile duct that can cause progressive biliary obstruction and biliary cirrhosis. They were classified by Todani into five types. Of these, type VI choledochal cysts are rarely reported in the literature.
A 22-month-old girl presented with intermittent epigastralgia for approximately 10 days and fever for three days. Fasting and total parenteral nutrition were administered after admission. However, sudden onset of severe epigastric pain occurred. An abdominal sonogram showed turbid ascites and peritonitis was impressed.
An emergent exploratory laparotomy was performed, and perforation of the posterior wall of types I and VI choledochal cysts was observed.
Intraoperative cholangiography revealed concomitant types I and VI choledochal cysts with stricture of the distal common bile duct. Definite surgery for resection of the choledochal cysts and gallbladder was performed with Roux-en-Y choledochojejunostomy.
The patient had no evidence of ascending cholangitis at three years after the operation.
Type VI choledochal cysts are rarely reported in the literature. To our knowledge, this is the first reported pediatric case of concomitant types I and VI choledochal cysts complicated with acute pancreatitis and spontaneous perforation.
胆总管囊肿是一种胆总管的先天性疾病，可导致进行性胆管梗阻和胆汁性肝硬化。它们由托达尼分为五种类型。其中，Ⅵ型胆总管囊肿在文献中报道较少。
一名22个月大的女孩出现间歇性上腹部疼痛约10天，发热3天。入院后给予禁食和全胃肠外营养。然而，突然出现严重的上腹部疼痛。腹部超声显示有浑浊腹水，提示腹膜炎。
进行了急诊剖腹探查术，观察到Ⅰ型和Ⅵ型胆总管囊肿后壁穿孔。
术中胆管造影显示合并Ⅰ型和Ⅵ型胆总管囊肿，胆总管远端狭窄。行胆总管囊肿和胆囊切除术，并进行Roux-en-Y胆总管空肠吻合术。
术后三年患者无胆管炎复发迹象。
Ⅵ型胆总管囊肿在文献中报道较少。据我们所知，这是首例报道的合并Ⅰ型和Ⅵ型胆总管囊肿并伴有急性胰腺炎和自发性穿孔的儿科病例。
