Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia
Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia
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DOI：
10.1007/s00247-009-1508-y
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发表时间：
2010-07-01
影响因子：
2.3
通讯作者：
Chan, Fandics P.
中科院分区：
文献类型：
--
作者：
Newman, Beverley;Feinstein, Jeffrey A.;Chan, Fandics P.
多脾异位与许多心血管异常有关，包括偶尔发生先天性肝外门体分流（CEPS）。忽视这种异常可能会导致不适当和无效的治疗。强调 CEPS 与多脾异位结合的重要性和相关解剖结构。对三名患有紫绀和肺动脉高压但没有心脏病病因的幼儿进行回顾。已知（1）或发现（2）它们与多脾异位。所有三个病例中均不存在具有奇点或半奇点延续的肝内下腔静脉（IVC）。尽管肝功能正常，但他们被发现存在较大的门体分流、脾肾位置，以及弥漫性外周肺动脉扩张，提示患有 CEPS（阿伯内西畸形）并伴有肝肺或更准确地说是门肺综合征。所有 CEPS 均位于脾脏同侧。在这些病例中，门静脉通畅，可以进行经皮分流术闭合并消除紫绀。CEPS 与多脾异位相关，并且可能因肺动静脉 (AV) 分流而出现症状。门静脉和肝静脉通畅对于确定 CEPS 封堵的可行性至关重要。
Heterotaxy with polysplenia is associated with many cardiovascular anomalies including the occasional occurrence of congenital extrahepatic portosystemic shunts (CEPS). Missing this anomaly can lead to inappropriate and ineffective therapy.To emphasize the importance and associated anatomy of CEPS in conjunction with heterotaxy with polysplenia.Review of three young children who presented with cyanosis and pulmonary hypertension without a cardiac etiology. They were known (1) or discovered (2) to have heterotaxy with polysplenia.There was absence of the intrahepatic inferior vena cava (IVC) with azygos or hemiazygos continuation in all three cases. In spite of normal liver function, they were discovered to have large portosystemic shunts, splenorenal in location, along with diffuse peripheral pulmonary arterial dilatation suggestive of CEPS (Abernethy malformation) with hepatopulmonary or, more accurately, portopulmonary syndrome. All CEPS were ipsilateral to the spleens. Patency of the portal veins in these cases allowed for percutaneous shunt closure with resolution of cyanosis.CEPS is associated with heterotaxy with polysplenia and can be symptomatic because of pulmonary arteriovenous (AV) shunting. Portal and hepatic vein patency are critical for determining feasibility of CEPS closure.