Odell J A, Rode H, Millar A J, Hoffman H D
Department of Pediatric Surgery, Red Cross Children's Hospital, Rondebosch, Republic of South Africa.
J Thorac Cardiovasc Surg. 1995 Oct;110(4 Pt 1):916-23. doi: 10.1016/s0022-5223(05)80158-8.
Membranous obstruction of the inferior vena cava at the level of the diaphragm is a rare cause of Budd-Chiari syndrome in children. Medical therapy usually fails. Surgical intervention aims at reestablishing patency of the inferior vena cava and hepatic venous outflow to the right atrium. We report on the management of this condition in 19 children of whom 7 were treated surgically. Indications for operation were persistent ascites, deteriorating liver function, and hepatic and inferior vena caval obstruction without significant collateral circulation. Three pathologic types were identified by ultrasonography and cavography and were confirmed at operation. These were type I (4 cases), with a thin membrane occluding the inferior vena cava at the level of the diaphragm; type II (12 cases), with segmental fibrotic obstruction of the inferior vena cava with variable involvement of hepatic veins; and type III (3 cases), with complete absence or nonvisualization of the inferior vena cava. All procedures were done with an extended midline sternotomy incision, cardiopulmonary bypass, core cooling to 16 degrees to 20 degrees C, and periods of circulatory arrest. Type I lesions necessitated membranectomy; type II lesions necessitated transcaval resection of the occluded confluence of the inferior vena cava and the hepatic vein with repair of the defect with an autogenous pericardial patch. One type II lesion, in addition, called for use of a 14 cm polytetrafluoroethylene tube graft to restore inferior vena caval flow. After the operation, marked clinical improvement was observed with an immediate reduction in liver and spleen size and resolution of ascites. Repeat cavography 10 to 30 days after the operation revealed complete patency in four cases and residual stenosis, which required transiliac balloon angioplasty to normalize the inferior vena cava/right atrial pressure gradient, in 3 cases. Thus eventual relief of hepatic venous outflow obstruction and inferior vena caval flow was restored in all cases. We advocate transcardiac membranectomy and pericardial patch grafting for symptomatic and deteriorating membranous obstruction of the inferior vena cava in children.
膈水平下腔静脉膜性梗阻是儿童布加综合征的罕见病因。内科治疗通常无效。手术干预旨在重建下腔静脉通畅及肝静脉向右心房的流出道。我们报告了19例该疾病患儿的治疗情况，其中7例接受了手术治疗。手术指征为持续腹水、肝功能恶化以及肝和下腔静脉梗阻且无明显侧支循环。通过超声检查和腔静脉造影确定了三种病理类型，并在手术中得到证实。这些类型分别为：I型（4例），在膈水平有一薄隔膜阻塞下腔静脉；II型（12例），下腔静脉节段性纤维化梗阻，肝静脉受累程度不一；III型（3例），下腔静脉完全缺如或不显影。所有手术均采用胸骨正中切口延长、体外循环、将体温降至16℃至20℃以及循环阻断。I型病变需行隔膜切除术；II型病变需经腔静脉切除下腔静脉与肝静脉闭塞的汇合处，并用自体心包补片修复缺损。另外，1例II型病变需要使用14厘米的聚四氟乙烯人工血管移植来恢复下腔静脉血流。术后观察到明显的临床改善，肝脏和脾脏大小立即缩小，腹水消退。术后10至30天重复腔静脉造影显示，4例完全通畅，3例有残余狭窄，需要经髂球囊血管成形术使下腔静脉/右心房压力梯度恢复正常。因此，所有病例最终均缓解了肝静脉流出道梗阻，恢复了下腔静脉血流。我们主张对有症状且病情恶化的儿童下腔静脉膜性梗阻采用经心膜切除术和心包补片移植术。
