Department of Transplant Surgery, Jichi Medical University, Tochigi, Japan.
Surgery. 2012 Mar;151(3):404-11. doi: 10.1016/j.surg.2011.07.035. Epub 2011 Oct 1.
Congenital extrahepatic portosystemic shunt (CEPS) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. It is still a matter of debate whether conservative or operative strategies should be used to treat symptomatic CEPS. The aim of this study was to evaluate the role of operative intervention in the management of CEPS.
Between June 2004 and August 2010, 6 consecutive patients with symptomatic CEPS were treated in our department. There were 3 male and 3 female patients, with a median age of 3.5 years (range, 1-8). Their demographic, clinical, and laboratory data were analyzed. All patients were scheduled to undergo shunt ligation or liver transplantation (LT).
Living donor LT was carried out in 4 patients, and shunt ligation in 2. After a median follow-up of 25 months, all the patients are alive currently with marked relief of symptoms.
Shunt ligation or LT for symptomatic CEPS is potentially curative.
先天性肝外门体静脉分流（CEPS）是一种罕见的静脉畸形，肠系膜静脉血液直接流入体循环。对于有症状的 CEPS，是采用保守治疗还是手术治疗策略，目前仍存在争议。本研究旨在评估手术干预在 CEPS 治疗中的作用。
2004 年 6 月至 2010 年 8 月，我院收治了 6 例有症状的 CEPS 患者。其中男 3 例，女 3 例，中位年龄 3.5 岁（范围，1-8 岁）。分析了他们的人口统计学、临床和实验室数据。所有患者均拟行分流结扎或肝移植（LT）。
4 例患者接受活体供体 LT，2 例患者行分流结扎。中位随访 25 个月后，所有患者均存活，症状明显缓解。
对于有症状的 CEPS，分流结扎或 LT 具有潜在的治愈作用。
