Abstract

Congenital portosystemic shunts may result in the development of hepatopulmonary syndrome, typically presenting with progressive hypoxemia in later childhood. We describe a case of a 5-month-old male with heterotaxy with polysplenia presenting with new onset hypoxemia. Subsequent evaluation identified an extrahepatic portosystemic shunt arising from the confluence of the main portal and superior mesenteric veins draining into the left renal vein. To treat his hypoxemia and prevent future complications of shunting, the patient underwent a successful single-stage endovascular closure.

中文翻译：

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先天性肝外门体分流术的血管内闭合治疗婴儿肝肺综合征

摘要

先天性门体分流可能导致肝肺综合征的发生，通常在儿童后期表现为进行性低氧血症。我们描述了一例 5 个月大的雄性异位性多脾症，表现为新发低氧血症。随后的评估发现，主门静脉和肠系膜上静脉汇入左肾静脉，导致肝外门体分流。为了治疗低氧血症并预防未来分流并发症，患者接受了成功的单阶段血管内封堵术。