Cheung K M, Lee C Y, Wong C T, Chan A K H
Department of Pediatrics, Caritas Medical Centre, Hong Kong, China.
J Paediatr Child Health. 2005 Jan-Feb;41(1-2):72-5. doi: 10.1111/j.1440-1754.2005.00542.x.
A 9-year-old girl presented with cyanosis and decreased exercise tolerance. Chest X-ray, lung function and echocardiogram were normal. Contrasted echocardiogram showed intrapulmonary right to left shunt and computerized tomography of the thorax showed dilated pulmonary vasculature. There was no arteriovenous malformation. Computerized tomography of the abdomen revealed absence of the intrahepatic portion of the portal vein. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained into the right atrium. We concluded that the patient had hepatopulmonary syndrome secondary to absence of the portal vein. This is the first report of hepatopulmonary syndrome in a female paediatric patient with a congenital absence of the portal vein. As all portal blood entered directly into systemic circulation, the condition was equivalent to congenital portosystemic shunt. Cases of congenital portosystemic shunt complicated by hepatopulmonary syndrome were also reviewed.
一名9岁女孩出现紫绀和运动耐力下降。胸部X光、肺功能和超声心动图均正常。对比增强超声心动图显示肺内右向左分流，胸部计算机断层扫描显示肺血管扩张。无动静脉畸形。腹部计算机断层扫描显示门静脉肝内部分缺如。肠系膜上静脉和脾静脉汇合成一个共同的主干，绕过肝脏并引流至右心房。我们得出结论，该患者患有门静脉缺如继发的肝肺综合征。这是首例先天性门静脉缺如的女性儿科患者发生肝肺综合征的报告。由于所有门静脉血直接进入体循环，该情况等同于先天性门体分流。还对先天性门体分流并发肝肺综合征的病例进行了综述。
