Department of Radiology, Stanford University School of Medicine, Lucile Packard Children's Hospital, 725 Welch Road, Stanford, CA 94305, USA.
Pediatr Radiol. 2010 Jul;40(7):1222-30. doi: 10.1007/s00247-009-1508-y. Epub 2010 Jan 13.
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.
多脾并内脏异位是多种心血管异常的相关因素，包括先天性肝外门体分流（CEPS）的偶发情况。如果漏诊这种异常，可能导致治疗不当和无效。
强调 CEPS 在多脾并内脏异位症中的重要性和相关解剖结构。
回顾了 3 名表现为发绀和肺动脉高压且无心脏病因的幼儿。他们（1）已知或（2）被发现患有多脾并内脏异位症。
所有 3 例均存在肝内下腔静脉（IVC）缺失，伴奇静脉或半奇静脉延续。尽管肝功能正常，但他们被发现存在大量门体分流，位于脾肾之间，同时伴有弥漫性外周肺动脉扩张，提示存在 CEPS（Abernethy 畸形），伴有肝肺或更准确地说是门肺综合征。所有的 CEPS 均与脾脏同侧。这些病例的门静脉通畅性允许经皮分流关闭，从而解决发绀问题。
CEPS 与多脾并内脏异位症相关，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性对于确定 CEPS 关闭的可行性至关重要。
