Kohli V, Pande G K, Dev V, Reddy K S, Kaul U, Nundy S
Department of Gastrointestinal Surgery, All India Institute of Medical Sciences, New Delhi.
Lancet. 1993 Sep 18;342(8873):718-22. doi: 10.1016/0140-6736(93)91712-u.
Hepatic venous outflow obstruction (HVOO) is a rare cause of portal hypertension and conservative treatment is usually ineffective. A large series of patients gave us an opportunity to devise a management protocol for this disorder. Between 1978 and 1992, we prospectively studied 75 patients with HVOO. The obstruction was in the hepatic vein in 24, in the inferior vena cava (IVC) in 44, and in both in 7. For hepatic vein obstruction proximal splenorenal shunts were done in 7 (2 died postoperatively); 4 shunts blocked and only 1 patient became completely symptom free. In 2 patients with partial obstruction we performed balloon dilatation of the right hepatic veins but within 6 months the obstruction recurred. In the next 6 patients we constructed a side-to-side portocaval shunt; 2 died of encephalopathy after discharge and 4 are alive and well. For IVC obstruction, after surgical procedures had yielded poor results in 14 patients, we changed to balloon angioplasty which was successful in 28 of the 30 other patients; restenosis occurred in 4. Of the 7 patients with a combined block, 3 have had balloon angioplasty followed by a side-to-side portocaval shunt; 1 died, 2 are well, and the remainder have not completed treatment. Of our 75 patients, 22 have died (5 in hospital and 17 after discharge), 7 have not completed treatment, and 2 have been lost to follow-up. However, 44 are symptom free. We did not encounter any case of hepatocellular carcinoma. We suggest that patients with HVOO should be actively managed with a side-to-side portocaval shunt for hepatic vein obstruction, balloon angioplasty for inferior vena caval obstruction, and perhaps both procedures for those with combined obstructions.
肝静脉流出道梗阻（HVOO）是门静脉高压的一种罕见病因，保守治疗通常无效。一大组患者为我们提供了为这种疾病制定管理方案的机会。1978年至1992年期间，我们对75例HVOO患者进行了前瞻性研究。梗阻位于肝静脉的有24例，位于下腔静脉（IVC）的有44例，两者均有的有7例。对于肝静脉梗阻，7例患者进行了近端脾肾分流术（2例术后死亡）；4例分流术堵塞，只有1例患者症状完全消失。2例部分梗阻患者，我们对右肝静脉进行了球囊扩张，但6个月内梗阻复发。接下来的6例患者，我们构建了侧侧门腔分流术；2例出院后死于脑病，4例存活且情况良好。对于IVC梗阻，14例患者手术治疗效果不佳后，我们改用球囊血管成形术，30例其他患者中有28例成功；4例发生再狭窄。7例合并梗阻的患者中，3例进行了球囊血管成形术，随后进行了侧侧门腔分流术；1例死亡，2例情况良好，其余患者尚未完成治疗。我们的75例患者中，22例死亡（5例在医院死亡，17例出院后死亡），7例未完成治疗，2例失访。然而，44例患者无症状。我们未遇到任何肝细胞癌病例。我们建议，对于HVOO患者，肝静脉梗阻应积极采用侧侧门腔分流术治疗，下腔静脉梗阻采用球囊血管成形术治疗，合并梗阻的患者可能两种手术都需要。
