Mori K, Dohi T, Yamamoto H, Kamada M
Department of Pediatrics, School of Medicine, University of Okayama, Japan.
Pediatr Radiol. 1990;21(1):66-8. doi: 10.1007/BF02010820.
A 9-year-old boy with a rare combination of multiple coronary artery fistulas and congenital shunt between the portal and hepatic veins (portal-systemic shunt) is presented. The most likely pathogenesis for the portal-systemic shunt in this case was persistence of the ductus venosus as a bypass tract of the portal vein. This shunt is considered as one cause of cardiomegaly and dilatation of the hepatic vein in this case, and careful follow-up is mandatory because this shunt could induce portal-systemic encephalopathy. There are a few reports of an anomalous portal venous connection to the hepatic vein as a result of abnormal portal embryogenesis. We experienced a unique case of congenital portal-systemic shunt associated with coronary artery fistulas. The present paper reports angiographic findings in this case and the etiological importance of the congenital portal-systemic shunt.
本文介绍了一名9岁男孩，其患有多种冠状动脉瘘与门静脉和肝静脉之间的先天性分流（门体分流）的罕见组合。该病例中门体分流最可能的发病机制是静脉导管持续存在作为门静脉的旁路通道。在该病例中，这种分流被认为是心脏肥大和肝静脉扩张的原因之一，并且由于这种分流可能诱发门体性脑病，因此必须进行仔细随访。有一些关于由于门静脉胚胎发育异常导致门静脉与肝静脉异常连接的报道。我们遇到了一例与冠状动脉瘘相关的先天性门体分流的独特病例。本文报道了该病例的血管造影结果以及先天性门体分流的病因学重要性。
