The classification based on intrahepatic portal system for congenital portosystemic shunts
The classification based on intrahepatic portal system for congenital portosystemic shunts
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DOI：
10.1016/j.jpedsurg.2015.01.009
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发表时间：
2015-04-01
影响因子：
2.4
通讯作者：
Kasahara, Mureo
中科院分区：
文献类型：
--
作者：
Kanazawa, Hiroyuki;Nosaka, Shunsuke;Kasahara, Mureo
背景/目的：肝移植被认为是治疗先天性门静脉缺如的根治性手术。放射介入技术的最新进展可以精确显示肝内门脉系统(IHPS)的结构。因此，需要从放射学的角度重新评估先天性门体分流(CPS)的治疗方法。本研究的目的是提出一种能够解释CPS病理生理特点的IHPS分型，并对CPS的治疗方法和管理起到补充作用。确诊时的中位年龄为6.8岁。结果：18例接受分流闭塞试验的患者按IHPS分级分为轻度7例、中度6例、重度5例。IHPS分级与分流时门静脉压力、病理组织学改变、术后门静脉血流及肝再生相关。分流闭合术后实验室指标明显改善，亚临床脑病发生率明显降低。2例重症患者治疗后出现脓毒症伴门静脉高压症，并因意外侧支血管发育而出现门脉血流盗血现象。分流试验中PVP较低时，分流一步关闭术后并发症的风险较高。结论：分流阻断试验可直观显示IHPS。IHPS分型反映了CPS的临床病理特征，对CPS的治疗方法和治疗方法有一定的指导意义。(C)2015 Elsevier Inc.保留所有权利。
Background/purpose: Liver transplantation was previously indicated as a curative operation for congenital absence of portal vein. Recent advances in radiological interventional techniques can precisely visualize the architecture of the intrahepatic portal system (IHPS). Therefore, the therapeutic approach for congenital portosystemic shunt (CPS) needs to be reevaluated from a viewpoint of radiological appearances. The aim of this study was to propose the IHPS classification which could explain the pathophysiological characteristics and play a complementary role of a therapeutic approach and management for CPS.Methods: Nineteen patients with CPS were retrospectively reviewed. The median age at diagnosis was 6.8 years old. Eighteen of these patients underwent angiography with a shunt occlusion test and were classified based of the severity of the hypoplasia of IHPS.Results: The eighteen cases who could undergo the shunt occlusion test were classified into mild (n=7), moderate (n=6) and severe types (n=5) according to the IHPS classification. The IHPS classification correlated with the portal venous pressure under shunt occlusion, the histopathological findings, postoperative portal venous flow and liver regeneration. Shunt closure resulted in dramatic improvement in the laboratory data and subclinical encephalopathy. Two patients with the severe type suffered from sepsis associated with portal hypertension after treatment, and from the portal flow steal phenomenon because of the development of unexpected collateral vessels. The patients with the severe type had a high risk of postoperative complications after shunt closure in one step, even if the PVP was relatively low during the shunt occlusion test.Conclusion: The IHPS could be visualized by the shunt occlusion test. The IHPS classification reflected the clinicopathological features of CPS, and was useful to determine the therapeutic approach and management for CPS. (C) 2015 Elsevier Inc. All rights reserved.