Portohepatic fusion mimics biliary aplasia.

IF 0.6 Q3 MEDICINE, GENERAL & INTERNAL
Yousef El-Gohary, Christopher R Moir, Abdalla Zarroug
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引用次数: 0

Abstract

We report a rare case of biliary aplasia in an infant who presented with persistent jaundice and elevated direct bilirubin despite having cholic stools. Imaging confirmed the absence of extrahepatic bile ducts, consistent with biliary atresia. Intraoperatively, there was a complete absence of an extrahepatic biliary tree consistent with biliary hypoplasia. We identified a cryptogenic hepatic plate directly fused to the right portal vein on further dissection. Despite the atypical anatomy, a modified Kasai portoenterostomy was successfully performed. The patient showed progressive clinical improvement with complete resolution of jaundice and normalisation of liver function tests over the following months. This case underscores a rare anatomical variant that can still permit a favourable outcome with a timely Kasai procedure. To our knowledge, this is one of the few documented cases of hepatic plate-portal vein fusion in biliary atresia.

肝门融合模拟胆道发育不全。
我们报告一个罕见的病例胆道发育不全的婴儿谁提出了持续黄疸和直接胆红素升高,尽管有胆汁性便。影像学证实肝外胆管缺失,符合胆道闭锁。术中完全没有肝外胆道树,符合胆道发育不全。在进一步的解剖中,我们发现了一个隐源性肝板直接融合到右门静脉。尽管解剖不典型,改良的Kasai门肠造口术成功实施。在接下来的几个月中,患者表现出渐进式的临床改善,黄疸完全消退,肝功能检查恢复正常。该病例强调了一种罕见的解剖变异,通过及时的Kasai手术仍然可以获得良好的结果。据我们所知,这是胆道闭锁中肝板-门静脉融合的少数病例之一。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
BMJ Case Reports
BMJ Case Reports Medicine-Medicine (all)
CiteScore
1.40
自引率
0.00%
发文量
1588
期刊介绍: BMJ Case Reports is an important educational resource offering a high volume of cases in all disciplines so that healthcare professionals, researchers and others can easily find clinically important information on common and rare conditions. All articles are peer reviewed and copy edited before publication. BMJ Case Reports is not an edition or supplement of the BMJ.
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