逆行门静脉造影术应用于八例门静脉海绵样变患儿诊治分析

目的: 探讨逆行门静脉造影术在儿童门静脉海绵样变中的临床应用价值。方法: 回顾性分析2018年1月至2019年9月浙江大学医学院附属儿童医院收治的8例门静脉海绵样变患儿的临床资料。8例患儿均通过逆行门静脉造影术明确门静脉系统相应血管形态及大小，若逆行门静脉造影结果显示门静脉左支通畅且直径大于3 mm，分流术时对Rex隐窝解剖探查并证实后，则实施Rex分流术；若逆行门静脉造影结果显示门静脉左支直径小于3 mm，分流术时对Rex隐窝解剖探查并证实，而解剖出的左肾静脉直径大于5 mm，则选择远端脾肾静脉分流术（Warren手术）。患儿出院后1、3、6个月按计划进行门诊随访，之后每隔6个月随访一次。结果: 8例患儿均成功行逆行门静脉造影，明确了门静脉主干及其左右分支、左肾静脉等重要血管的解剖位置及大小。其中4例患儿门静脉左、右支均发育良好，且左右门静脉有明显汇合，但与门静脉主干不相通，门静脉左支测量直径大于3 mm，分流术时解剖探查结果与此相符，遂行Rex分流术。另4例患儿中，3例患儿门静脉左支直径小于3 mm、右支显影不清，1例患儿门静脉左支发育差，近乎闭塞，但这4例患儿左肾静脉显影良好，血流通畅且直径大于5 mm，遂行Warren手术。术后7例患儿恢复良好，另1例患儿于术后1年再次出现消化道出血，经保守治疗后病情平稳。结论: 术前逆行门静脉造影可以评估门静脉海绵样变患儿的门静脉系统血管情况，为患儿制订恰当的手术方案提供重要依据。

Preoperative retrograde portography for children with cavernous transformation of the portal vein: clinical application in 8 cases

Objective: To assess the clinical application of preoperative retrograde portal venography for children with cavernous transformation of the portal vein (CTPV). Methods: The clinical data of 8 cases of CTPV admitted in the Children's Hospital of Zhejiang University from January 2018 to September 2019 were retrospectively analyzed. Preoperative retrograde portography was performed to determine the corresponding vascular morphology and size of portal vein system. If the retrograde portography showed that the left branch of the shadow portal vein was unobstructed and its diameter was greater than 3 mm, Rex shunt would be performed after anatomic exploration of Rex recess; if retrograde portography showed that the diameter of left portal vein was less than 3 mm, but the diameter of left renal vein dissected during shunt operation was greater than 5 mm, Warren operation was selected. The patients were followed up for 1, 3 and 6 months after discharge, and then were followed up every 6 months. Results: Retrograde portal venography was successfully performed in 8 child patients.The anatomical position and size of main portal vein and its left and right branches, left renal vein and other important vessels were determined. Among them, there was the well-developed left and right branches of portal vein in 4 child patients, in which the left and right branches of portal vein converged together, but did not communicate with the main portal vein. In addition, the left branch diameter of the portal vein was greater than 3 mm, and the anatomical exploration results during shunt were consistent with it, so Rex shunt was performed. In the other 4 cases, the left branch diameter of the portal vein was small (less than 3 mm) in 3 cases, and the right branch was not clearly developed. Moreover, the left branch of the portal vein was poorly developed and almost occluded in 1 case. However, the left renal vein in these 4 child patients was well developed, the blood flow was unobstructed and the diameter was greater than 5 mm, so Warren operation was performed. Seven patients recovered well after the operation, and the other one had digestive tract rudimentary one year after operation, and the condition was stable after conservative treatment. Conclusion: The preoperative retrograde portal venography can be used to evaluate the portal vein system in children with CTPV, which provides important clinical basis for making appropriate treatment plan before surgery.