Department of Respiratory Medicine, Sydney Children's Hospital, level 0 South West Wing, High St, Randwick, NSW, 2031, Australia.
Discipline of Paediatrics, School of Women's and Children's Health, Medicine, University of New South Wales, Sydney, NSW, Australia.
BMC Pediatr. 2019 Mar 11;19(1):78. doi: 10.1186/s12887-019-1443-5.
Cholecystoduodenostomy is a surgical procedure that bypasses the extrahepatic biliary tree and connects the gallbladder directly to the duodenum. This case describes the successful use of this procedure in a novel situation.
A premature (34 weeks gestation) female infant with cystic fibrosis required a laparotomy on day 1 of life due to an intrauterine small bowel perforation. Resection of small bowel and ileostomy formation resulted in short gut syndrome, with 82 cm residual small bowel and intact ileocaecal valve. Post-ileostomy reversal at 2 months old, she developed conjugated hyperbilirubinaemia. Despite conservative management including increased enteral feeding, ursodeoxycholic acid, cholecystostomy drain insertion and flushes, her cholestatic jaundice persisted. A liver biopsy revealed an "obstructive/cholestatic" picture with fibrosis. To avoid further shortening her gut with an hepatoportoenterostomy, cholecystoduodenostomy was performed at 3 months of age with subsequent post-operative improvement and eventual normalisation of her clinical jaundice and liver biochemistry.
This is the first reported case of a cholecystoduodenostomy being used successfully to treat an infant with persistent conjugated hyperbilirubinemia, cystic fibrosis and short gut syndrome. Cholecystoduodenostomy is a treatment option that with further study, may be considered for obstruction of the common bile duct in patients with short gut and/or where a shorter operating time with minimal intervention is preferred.
胆肠吻合术是一种绕过肝外胆道系统并将胆囊直接与十二指肠连接的手术。本案例描述了在一种新情况下成功使用该手术的情况。
一名早产（34 周妊娠）女性婴儿，因宫内小肠穿孔，在出生后第 1 天行剖腹手术。小肠切除和回肠造口术导致短肠综合征，残留小肠 82cm，回盲瓣完整。2 个月大时行回肠造口术逆转后，她出现结合胆红素血症。尽管进行了保守治疗，包括增加肠内喂养、熊去氧胆酸、胆囊造口引流管插入和冲洗，但她的胆汁淤积性黄疸仍持续存在。肝活检显示“梗阻性/胆汁淤积性”表现伴有纤维化。为避免因肝门肠吻合术进一步缩短肠道，在 3 个月大时进行了胆肠吻合术，术后情况改善，最终临床黄疸和肝功能生化指标恢复正常。
这是首例成功使用胆肠吻合术治疗持续性结合胆红素血症、囊性纤维化和短肠综合征婴儿的病例。胆肠吻合术是一种治疗选择，随着进一步研究，对于短肠和/或需要更短手术时间和最小干预的患者的胆总管阻塞，可能会被考虑。
