Morgan G, Superina R
Department of Surgery, University of Toronto, Ontario, Canada.
J Pediatr Surg. 1994 Sep;29(9):1239-41. doi: 10.1016/0022-3468(94)90812-5.
Congenital absence of the portal vein (CAPV) is an unusual condition that often is associated with other anomalies. This is the first report of reduced-size liver transplant in a patient with CAPV. Because the presence of this rare congenital portasystemic shunt, there was no portal-systemic pressure differential, and thus an absence of collateral vessels in the pretransplant state. As a result, surgery was complicated by severe mesenteric edema caused by an increase in portal pressure when the allograft was implanted. The morbidity associated with CAPV usually results from associated conditions, but if transplantation is necessary, careful management of mesenteric congestion is crucial to success. The authors' experience and a review of the literature indicate that the CAPV can be classified into one of two groups of portasystemic anomalies.
先天性门静脉缺如（CAPV）是一种罕见的病症，常与其他异常情况相关联。本文首次报道了1例患有CAPV的患者接受减体积肝移植的情况。由于存在这种罕见的先天性门体分流，术前不存在门体压力差，因此也没有侧支血管。结果，植入同种异体移植物时，门静脉压力升高导致严重的肠系膜水肿，使手术变得复杂。与CAPV相关的发病率通常由相关病症引起，但如果有必要进行移植，仔细处理肠系膜充血对于成功至关重要。作者的经验以及文献回顾表明，CAPV可分为两种门体异常类型之一。
