小儿布—加综合症的诊断和治疗

小儿布—加综合症(BCS)临床报道较少,1988～1991年我院手术治疗8例。经消化道钡餐检查者6例,其中4例显示明显食道静脉曲张。血管造影显示:8例均有不同程度下腔静脉狭窄,其中2例伴有 MOVC;4例显示中度或重度肝静脉狭窄;3例为肝静脉阻塞。指出经右心房手指破膜术和球囊导管破膜术是简单、安全和有效的治疗方法。随访1/2～3年均恢复顺利,仅1例于肠腔侧侧吻合术后并发肝性脑病,经治疗而愈。肝大(除1例外)及食管静脉曲张患者,均于手术后2～3个月临床症状完全消失。

DIAGNOSIS AND TREATMENT OF BUDD-CHIARI SYN-DROME IN CHILDREN

Budd-chiari syndrome(BCS)in children was rare described.Eight cases of BCS in children was treated surgically in our hospital from 1988 to 1991.Upper gastroentestinal radiography by Barium meal revealed that 4 cases had remarkable esophageal varices in the 6 cases with this examination method.The results of angiography indicate that all 8 cases had stenosis inferior vena cava in different digree,2 cases of them was associated.with membranous obstruction of the inferior vena cava(MOVC),and that 4 cases had mod- erate or severe stenosis of hepatic vena,and 3 cases had obstruction of hepatic vena.It is suggested that transcardiac membranotomy with finger or membranotomy with balloon catheter by means of percutaneous transluminal angiography(PTA)is a simple,safe and effective surgical technique.The results of following-up through 3～36 months indi- cate that only one case had post-operative encephalopathy after performed side-to-side meso-caval shunt,and this case have been cured by conservative therapy.All hepatomegaly except one and all esophageal varices signs in all cases disappeared complete- ly within 2～3 months after operation.