Children's Hospital at Westmead, Sydney, Australia.
Nagano Children's Hospital, Nagano, Japan.
Pediatr Surg Int. 2021 Jan;37(1):101-107. doi: 10.1007/s00383-020-04773-2. Epub 2020 Nov 17.
Many biliary atresia (BA) patients will eventually develop liver failure even after a successful Kasai portoenterostomy. A common complication of long-term BA survivors with their native liver is problematic portal hypertension. The aim of this study was to defend the view that portosystemic shunts can delay or negate the need for transplantation in these children.
A retrospective single center review of the efficacy of portosystemic shunts in BA patients after a successful Kasai portoenterostomy was conducted.
From 1991 to 2017, 11 patients received portosystemic shunts. Median age of Kasai operation was 48 (36-61) days. Shunts were performed at the median age of 6.2 (4.1-6.8) years. Three of these eleven patients required subsequent liver transplantation. OS at 5 and 10 years were 90.9% and 81.8%, respectively. TFS at 5 and 10 years were 90.9% and 72.7%, respectively. Long-term complications included mild encephalopathy in 2 patients, hypersplenism in 3, and cholestasis in 1.
Portosystemic shunt for the treatment of portal hypertension in carefully selected BA patients is an effective option in delaying or negating the need for liver transplantation.
许多先天性胆道闭锁（BA）患者即使在成功进行了 Kasai 胆肠吻合术后最终也会发展为肝功能衰竭。长期生存的 BA 患者的一个常见并发症是棘手的门静脉高压。本研究旨在证明门体分流术可以延迟或否定这些儿童进行肝移植的必要性。
回顾性分析了 1991 年至 2017 年间成功进行 Kasai 胆肠吻合术后 BA 患者行门体分流术的疗效。
11 例患者接受了门体分流术。Kasai 手术的中位年龄为 48（36-61）天。分流术在中位年龄 6.2（4.1-6.8）岁时进行。这 11 例患者中有 3 例需要后续进行肝移植。5 年和 10 年的 OS 分别为 90.9%和 81.8%。5 年和 10 年的 TFS 分别为 90.9%和 72.7%。长期并发症包括 2 例患者轻度脑病，3 例患者脾功能亢进，1 例患者胆汁淤积。
在仔细选择的 BA 患者中，门体分流术治疗门静脉高压是一种有效的选择，可以延迟或否定肝移植的必要性。
