Liver transplantation with reconstruction of infrarenal vena cava and iliac veins

A 31-year-old lawyer presented with bouts of severe encephalopathy. He was diagnosed with a1-antitrypsin deficiency type MZ. In addition, his mobility was poor due to markedly edematous legs. At birth, he underwent exchange transfusion for rhesus (Rh)-antagonism, which caused umbilical vein sepsis and subsequent portal vein thrombosis. He underwent a splenorenal shunt operation for repeated variceal bleeds, which soon failed due to thrombosis of the shunt. The upper gastrointestinal bleeds were treated conservatively until the age of 9 years, when he received a Marion-Clatworthy shunt1, 2 (Fig. 1). While he no longer experienced upper gastrointestinal bleeds, he progressively suffered from swelling of both legs and encephalopathy, which interfered significantly with mobility and his profession as a lawyer. Portal vein thrombosis (1), thrombosed spleno-renal shunt (2), and mesentericocaval shunt (Marion-Clatworthy shunt) (3). It was decided to offer the patient a liver transplantation and to reconstruct the distal vena cava and iliac veins with the vessels from the donor (Figs. 2 and 3). The recipient hepatectomy was performed, including retrohepatic vena cava, followed by the implantation of the donor liver. The portal vein was reconstructed to the vena cava of the recipient, belonging to the Marion-Clatworthy shunt, while the vena cava of the graft was anastomosed to the suprarenal vena cava of the recipient. The left renal artery was identified during the dissection of the stump of vena cava of the recipient. After recirculation and completion of the arterial and biliary anastomoses, the distal vena cava was reconstructed. Then, the stumps of the internal iliac veins were dissected. After the anastomoses between the vena cava graft, the infrarenal vena cava, the left iliac veins of the recipient were completed, the graft was opened, after which the right iliac vein reconstruction followed to complete the venous reconstruction. Liver and vena cava graft (hatched) with reconstruction of portal vein to vena cava belonging to the Clatworthy shunt (1), vena cava of the liver graft to suprarenal vena cava of the recipient (2), vena cava graft to infrarenal vena cava and iliac veins of the recipient (3). Situation after the reconstruction of portal vein (1) and vena cava (2,3) during the operation. Right renal artery (4), crossing the segment of the vena cava of the recipient, is visible. The patient recovered successfully and is currently (10 years later) in good condition. In addition to the disappearance of the encephalopathy, allowing a normal diet, the patient was mostly thrilled by the disappearance of the leg edema and regaining his normal mobility.