Boles E T, Wise W E, Birken G
Am J Surg. 1986 Jun;151(6):734-9. doi: 10.1016/0002-9610(86)90054-1.
A long-term evaluation of 43 children with extrahepatic portal hypertension indicates a high success rate and excellent subsequent health when a splenorenal or mesocaval shunt can be performed. On the other hand, makeshift shunts inevitably fail. Direct operations are considerably less successful than standard shunts. Esophagogastric resections with interpositions result in long-term freedom from bleeding in only about half the cases, and portoazygous disconnection procedures have been uniformly disappointing. Sclerotherapy in a relatively recent experience has been quite successful, but long-term results are presently unavailable. The condition carries a significant mortality rate. Complications from failed operations, division of the vena cava, and multiple transfusions are numerous. The general health of long-term survivors is excellent in those with successful operations, and is surprisingly good for patients whose operations have been unsuccessful and for those who have had no operations.
对43例肝外门静脉高压症患儿进行的长期评估表明，若能施行脾肾分流术或肠系膜上腔静脉分流术，则成功率较高，且患儿随后的健康状况良好。另一方面，临时分流术不可避免地会失败。直接手术的成功率远低于标准分流术。食管胃切除术加间置术仅在约一半的病例中能实现长期无出血，而门静脉奇静脉断流术一直令人失望。近期经验显示硬化疗法颇为成功，但目前尚无长期结果。该疾病的死亡率较高。手术失败、腔静脉破裂及多次输血引发的并发症众多。手术成功的长期存活者总体健康状况良好，而手术不成功的患者以及未接受手术的患者的健康状况也出奇地好。
