Living-related liver transplantation for biliary atresia associated with polysplenia syndrome

This report describes a 1-yr-old boy with biliary atresia (BA) and polysplenia syndrome (PS) who underwent successful living-related liver transplantation (LTx). At the time of initial hepatic portoenterostomy, he was noticed to have a preduodenal portal vein (PV), non-rotation of the intestine, and polysplenia. Because he did not achieve good bile excretion, he underwent a living-related LTx (using a left lateral segment from his mother) at the age of 14 months. Evaluation of the vascular anatomy was made by angiography, magnetic resonance imaging (MRI), computerized tomography (CT), and Doppler ultrasound. The PV was stenotic from the confluence of the superior mesenteric vein (SMV) and splenic vein (SpV) to the hepatic hilum. The retrohepatic inferior vena cava (IVC) was deficient cranially to the renal vein and was connected to the azygous vein. The supra-hepatic IVC was detected below the diaphragm and was connected to three hepatic veins. The common hepatic artery (HA) originated from the superior mesenteric artery. At LTx, the PV was dissected to the level of confluence of the SMV and the SpV, from which the venous graft was interposed using the donor's ovarian vein. Three hepatic veins were plastied into one orifice, which was anastomosed to the graft's hepatic vein under the diaphragm. The graft vascularity and function has been good for 1 yr after LTx. In the present case, sufficient pre-LTx evaluation of vascular anomalies seemed to help performance of the successful LTx.