Maeda N, Horie Y, Koda M, Suou T, Andachi H, Nakamura K, Kawasaki H
Second Department of Internal Medicine, Tottori University Faculty of Medicine, Yonago, Japan.
Hepatogastroenterology. 1997 Sep-Oct;44(17):1317-20.
Extrahepatic portal obstruction is one of the causes of portal hypertension, in which well-developed hepatopetal pathways are commonly recognized. Herein an extremely rare case of extrahepatic portal obstruction without hepatopetal pathway, probably caused by arterioportal fistula, is reported. The patient was a normally matured 16-year-old girl admitted for further evaluation of jaundice, presenting with the clinical manifestations of the portal hypertension associated with hypersplenism and portosystemic venous shunt. Celiac angiography clearly demonstrated an intrahepatic arterial aneurysm fed by the right hepatic artery shunting to the superior mesenteric vein, and portography disclosed complete obstruction of the portal trunk with conspicuous hepatofugal pathway but no hepatopetal collateral veins. The exact mechanism of this phenomenon is not known and whether the extrahepatic portal obstruction was primary or secondary is still obscure. However, this is the first case report in the world literature describing extrahepatic portal obstruction with absence of hepatopetal pathway.
肝外门静脉阻塞是门静脉高压的病因之一，其中肝内向肝血流途径通常较为常见。本文报道了一例极为罕见的无肝内向肝血流途径的肝外门静脉阻塞病例，可能由动静脉瘘引起。患者为一名发育正常的16岁女孩，因黄疸进一步评估入院，表现出门静脉高压伴脾功能亢进和门体静脉分流的临床表现。腹腔血管造影清晰显示由右肝动脉供血的肝内动脉瘤分流至肠系膜上静脉，门静脉造影显示门静脉主干完全阻塞，有明显的离肝血流途径但无向肝侧支静脉。这种现象的确切机制尚不清楚，肝外门静脉阻塞是原发性还是继发性仍不明确。然而，这是世界文献中首例描述无肝内向肝血流途径的肝外门静脉阻塞的病例报告。
