Rocha-Santos Vinicius, Waisberg Daniel Reis, Pinheiro Rafael Soares, Nacif Lucas Souto, Arantes Rubens Macedo, Ducatti Liliana, Martino Rodrigo Bronze, Haddad Luciana Bertocco, Galvao Flavio Henrique, Andraus Wellington, Carneiro-D'Alburquerque Luiz Augusto
Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil.
World J Hepatol. 2021 Jan 27;13(1):151-161. doi: 10.4254/wjh.v13.i1.151.
Budd-Chiari syndrome (BCS) is a challenging indication for liver transplantation (LT) due to a combination of massive liver, increased bleeding, retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava (IVC). Occasionally, it may be totally thrombosed, increasing the complexity of the procedure, as it should also be resected. The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC; thus, it may be necessary to reconstruct it.
A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction. It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed, up to almost the right atrium. A right-lobe graft was retrieved from his sister, with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein. Owing to massive subcutaneous collaterals in the abdominal wall, venovenous bypass was implemented before incising the skin. The right atrium was reached a transdiaphragramatic approach. Hepatectomy was performed with the retrohepatic vena cava. It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor. The patient remains well on outpatient clinic follow-up 25 mo after the procedure, under an anticoagulation protocol with warfarin.
Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.
布加综合征（BCS）是肝移植（LT）的一项具有挑战性的适应证，原因在于肝脏肿大、出血增加、腹膜后纤维化，且常伴有下腔静脉（IVC）狭窄。偶尔，下腔静脉可能完全血栓形成，这增加了手术的复杂性，因为还需要切除血栓。当进行活体肝移植时，挑战更大，因为移植物不包含肝后段下腔静脉；因此，可能需要对其进行重建。
一名35岁男性患者，因布加综合征导致肝硬化，且肝细胞癌超出米兰标准，接受了活体肝移植并进行下腔静脉重建。由于肝后段下腔静脉完全血栓形成，直至几乎右心房，因此有必要切除下腔静脉。从他的妹妹获取了右叶移植物，进行流出道重建，包括右肝静脉以及第V和第VIII段分支至肝中静脉。由于腹壁大量皮下侧支循环，在切开皮肤前实施了静脉-静脉转流。通过经膈肌途径到达右心房。进行肝切除时连同肝后腔静脉一并切除。用取自一名已故供体的肝下腔静脉移植物进行重建。术后25个月门诊随访时，患者情况良好，正在接受华法林抗凝治疗。
对于布加综合征合并下腔静脉血栓形成的患者，采用细致的手术技术和量身定制的策略进行活体肝移植是可行的。
