Choi G S, Park J B, Jung G O, Chun J M, Kim J M, Moon J I, Kwon C H D, Kim S J, Joh J W, Lee S-K
Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Transplant Proc. 2010 Apr;42(3):839-42. doi: 10.1016/j.transproceed.2010.02.045.
Budd-Chiari syndrome (BCS), which is characterized by hepatic venous outflow obstruction due to occlusion of the major hepatic vein and/or the inferior vena cava (IVC), is rare. Traditionally, a caval resection is advocated for these patients; however, such a maneuver renders living donor liver transplantation (LDLT) impossible. We encountered BCS in 4/377 LDLT patients during a 5-year period (January 2003 to December 2007). This report examine the various surgical modifications in these 4 patients, who underwent to LDLT for BCS. Resection of right hepatic vein (RHV) with an adjacent fibrotic part of the IVC with direct anastomosis of the graft RHV to the IVC was performed in 2 patients. One patient underwent retrohepatic IVC excision and reconstruction with a cryopreserved autologous IVC graft. The fourth patient, with a preexisting mesoatrial shunt for BCS, underwent conversion of this to a RHV atrial shunt. Graft and patient survivals were 100%. There were few complications in either donors or recipients. LDLT for BCS can be performed safely with adequate venous drainage techniques and with anticoagulant therapy and good follow-up for early diagnosis and treatment of recurrence leading to excellent long-term results.
布加综合征(BCS)以主要肝静脉和/或下腔静脉(IVC)闭塞导致肝静脉流出道梗阻为特征,较为罕见。传统上,这类患者主张行腔静脉切除术;然而,这种操作使得活体肝移植(LDLT)无法进行。在5年期间(2003年1月至2007年12月),我们在377例LDLT患者中有4例遇到BCS。本报告探讨了这4例因BCS接受LDLT患者的各种手术改良方法。2例患者行右肝静脉(RHV)及IVC相邻纤维化部分切除,将移植肝RHV直接吻合至IVC。1例患者接受了肝后下腔静脉切除并用冷冻保存的自体下腔静脉移植物进行重建。第4例患者因BCS先前已存在肠系膜心房分流,将其转换为RHV心房分流。移植物和患者生存率均为100%。供体和受体的并发症均很少。采用适当的静脉引流技术、抗凝治疗以及对复发进行早期诊断和治疗的良好随访,BCS患者的LDLT可以安全进行,并能取得优异的长期效果。