Alvarez F, Bernard O, Brunelle F, Hadchouel P, Odièvre M, Alagille D
J Pediatr. 1983 Nov;103(5):696-702. doi: 10.1016/s0022-3476(83)80460-0.
We examined 108 children with obstruction of the portal vein. Symptoms included splenomegaly and gastrointestinal tract hemorrhage. Obstruction was secondary to portal vein injury in 44 children and was combined with congenital malformations in 17 others. Ultrasonography provided the correct diagnosis in 36 of the 37 children in whom it was performed. Angiography, performed in 101 children, showed that the obstruction extended to the superior mesenteric vein in 14 children and to the entire portal venous system in seven; intrahepatic branches were involved in half the cases. Natural splenorenal shunts were visible in 19 children but were not clearly associated with a lower risk of gastrointestinal tract bleeding; in five of 30 children, cavography displayed abnormalities of the inferior vena cava. Spontaneous gastrointestinal tract hemorrhage occurred in 78 children. Fiberoptic endoscopy showed esophageal varices in 79 of the 81 children studied. The presence of tense varices and congestion of esophageal mucosa clearly augmented the risk of bleeding. These results suggest a simple method of investigation based on ultrasonography for diagnosis and on endoscopy for prognosis. Angiography should be limited to children with a history of gastrointestinal tract bleeding for whom a surgical portosystemic shunt is being considered.
我们检查了108例门静脉梗阻患儿。症状包括脾肿大和胃肠道出血。44例患儿的梗阻继发于门静脉损伤，另有17例合并先天性畸形。37例行超声检查的患儿中，36例获得了正确诊断。101例行血管造影的患儿显示，14例患儿的梗阻延伸至肠系膜上静脉，7例延伸至整个门静脉系统；半数病例肝内分支受累。19例患儿可见自然脾肾分流，但与胃肠道出血风险降低无明显关联；30例患儿中有5例腔静脉造影显示下腔静脉异常。78例患儿发生自发性胃肠道出血。在81例接受研究的患儿中，79例经纤维内镜检查发现食管静脉曲张。紧张性静脉曲张和食管黏膜充血明显增加了出血风险。这些结果提示了一种基于超声检查进行诊断、基于内镜检查进行预后评估的简单检查方法。血管造影应仅限于有胃肠道出血病史且考虑行手术门体分流术的患儿。
