Introduction: Diffuse portal thrombosis with progressive liver insufficiency is one of the indications of multivisceral transplantation. We present the case of a patient with Budd-Chiari syndrome and portal, mesenteric and splenic veins thrombosis.

Case description: A 52 year old man with two month abdominal pain and distension led to Budd-Chiari syndrome diagnosis in November 2018, in other institution. A left hepatic vein angioplasty was done, with dalteparin treatment. After the second dose he showed abdominal rash. Warfarin treatment was used for 5 days without proper coagulation control. A new dalteparin dose induced an anaphylactic reaction with ICU admission. Several weeks later, with the diagnosis of splachnic veins thrombosis, a thrombectomy and portacaval  shunt was performed. Shunt thrombosed at second postoperative day.  JAK2-positive polycythemia vera was diagnosed and treated with hydroxyurea to keep a hematocrit <45%, and the patient was anticoagulated with fondaparinux. He had a 3 month postoperative stay due to complications. Ascitis developed in 2020, with repeated paracentesis, and a right inguinal hernia. Esophageal varices were treated with elastic bands ligation. The patient retired and moved to other country, and shortly after he was referred to our hospital. CT and MRI showed liver cirrosis with regenerative nodules, portal vein thrombosis and cavernous transformation, with a thrombosed portacaval shunt., and splenic and SMV thrombosis. Sodium heparin tolerance test was decided to rule out problems derived from donor heparinization, and done at the ICU, without adverse effects. He was listed for multivisceral transplantation on December 2020. Oral anticoagulation with acenocumarol was instituted. A multivisceral transplant was performed on July 2021. Due to anticoagulation  (PT 28%), portal hypertension and adhesions and intense fibrosis from previous surgery, massive transfusion was needed: 60 RBC units, 45 FFP units, 5 platelet pools, 8 g fibrinogen, 2000 U Beriplex. Aortic conduit to recipient aorta. A side to side caval suture was performed due to the Budd-Chiari syndrome. Gastric anastomosis with preservation of half the stomach in both recipient and graft. Right colon of the graft to transverse colon in the recipient. Ten days after the transplant he had a reoperation due to spontaneous punctiform perforation at mid-jejunum. Two months after the transplant the liver profile deteriorated due to biliary sludge, and a biliary stent was inserted. Discharged after three months and a half.

Conclusion: Multivisceral transplantation may be a good indication in patients with diffuse portomesenteric thrombosis with procoagulant disorders and challenging anticoagulation treatments.