Living Donor Liver Transplantation Using a Graft from a Donor with Situs Inversus Totalis (2009)

In Asia, various cultural, social, and historic factors have severely limited the availability of deceased donor organs. Therefore, living donor liver transplantation (LDLT) has emerged as an important therapeutic option for patients with end-stage liver disease. According to data reported to the Korean Network for Organ Sharing liver transplantation registry, LDLT accounted for more than 80% of the liver transplants performed in Korea during 2007. As the number of cases has increased, transplant surgeons have introduced technical innovations over the past 2 decades, and most technical obstacles have been overcome. Situs inversus is a rare congenital anomaly that was once regarded as a contraindication to liver transplantation because of the technical difficulties associated with the unique vascular anatomy and concern about achieving accurate graft positioning.1 However, several reports have presented uneventful outcomes after liver transplantation performed in adult recipients with situs inversus,2-5 and 4 cases of deceased donor organs with situs inversus used for liver transplantation have been reported in the English literature.6-9 Here, we present a case of LDLT in an adult using a graft from a situs inversus donor with a successful result. A 53-year-old male was referred to our hospital in April 2007 for further evaluation for an LDLT. He was diagnosed with alcoholic liver cirrhosis in 2004 by ultrasonography, and multiple episodes of hepatic encephalopathy had occurred since that time. Physical examination revealed jaundice and a flapping tremor, and he had a Model for End-Stage Liver Disease score of 22. The prospective donor was a 31-year-old male son. The liver function testing of the donor was within normal limits, and the hepatitis viral markers (hepatitis B surface antigen and hepatitis C virus antibody) were negative. The contrast-enhanced computed tomography scan of the abdomen showed a complete situs inversus with a mirror-image location of the abdominal organs with respect to the situs solitus and a reversed right hepatic artery (anatomically a left hepatic artery) that originated from the superior mesenteric artery and a reversed left hepatic artery arising from the left gastric artery (Fig. 1). The volumetric calculations of the donor's whole liver and reversed right hemiliver, calculated by computed tomography imaging, were 1290 and 807 cm3, respectively, and the graft to recipient weight ratio was 1.15%. Contrast-enhanced abdominal computed tomography scans of the donor: complete situs inversus with a mirror-image location of the abdominal organs with respect to the situs solitus. (A) Axial view. (B) Coronal view. (C) A reversed right hepatic artery (arrow head), anatomically a left hepatic artery, originates from the superior mesenteric artery, and the left gastric artery gives off the reversed left hepatic artery (arrow). The LDLT was performed in June 2007. Situs inversus was confirmed on donor laparotomy, and a right hepatectomy was performed with standard procedures. The liver graft was a reversed right lobe excluding the middle hepatic vein, and there were no prominent veins draining reversed segments 5 and 8. During the surgery for the recipient, following full mobilization of the liver from the adjacent organs, ligaments, and inferior vena cava, the Glissonian pedicles were cut intrahepatically, as high as possible, with a high hilar dissection technique as reported previously.10 The right hepatic vein of the recipient was dissected as much as possible intrahepatically so that it was long enough to perform a tension-free hepatic vein anastomosis. Implantation of the graft was performed with a modified piggyback technique. When the graft was implanted into the right upper quadrant, after a 180° rotation of the graft along the axis of the vena cava groove, with adjustment for the hepatic vein location, the bile duct lay in the most dorsal portion of the hepatic hilum. The reversed right hepatic vein of the donor was anastomosed directly to the right hepatic vein of the recipient without vascular modifications, and this was followed by an end-to-end anastomosis of the donor portal vein to the confluence of the right anterior branch and the right posterior branch of the recipient's portal vein. The reversed right hepatic artery of the donor was anastomosed to the recipient's right hepatic artery with a microvascular technique. Routine protocol intraoperative Doppler examinations prior to biliary reconstruction showed good flow in all the vessels. Biliary reconstruction was performed by duct-to-duct anastomosis with the right bile duct of the donor and the confluence of the right anterior and posterior ducts of the recipient. Although the anastomosed portal vein was situated just above the bile duct, we could perform biliary reconstruction without limitation of exposure by gently elevating the portal vein with a vein retractor (Fig. 2). The patient had excellent graft function postoperatively and was in satisfactory condition 1 year after liver transplantation (Fig. 3). Vascular and biliary reconstructions. (A) Long hepatic vein cuff of the recipient (arrow head) and reversed right hepatic artery of the donor (arrow) anastomosed to the right hepatic artery of the recipient. (B) Duct opening of the graft. (C) Duct-to-duct reconstruction. Contrast-enhanced abdominal computed tomography scans of the recipient (1 year after the living donor liver transplantation), showing patent vascular structures of the graft and normal intrahepatic duct. A 4-cm low-attenuated fluid collection can be observed at the medial side of reversed segment 6 of the liver. (A) Hepatic vein. (B) Portal vein. LDLT, living donor liver transplantation. To date, only 4 cases of liver transplantation with deceased donors with situs inversus have been reported; modifications of transplant techniques were applied to overcome the problems associated with the vascular anatomy and graft positioning. Herrera et al.8 performed liver transplantation using the standard piggyback technique and implanted the entire liver graft without rotation. However, such graft positioning leaves the graft in an unfavorable abdominal cavity, and this can result in venous pedicle torsion or graft displacement. Pomposelli et al.9 reported a novel approach using retroversus implantation of the liver graft; they reconstructed the biliary patency using a Roux en-Y choledochojejunostomy because of the technical difficulties in performing direct duct-to-duct anastomosis. When the graft was rotated by 180° along the axis of the vena cava groove, hilar vascular and biliary structures were flipped vertically, and the hilar plate itself was located more ventrally. This positioning might cause technical difficulties because of a greater length requirement for the portal venous or bile duct conduit. We could overcome this anatomical challenge by using a high hilar dissection technique. After total occlusion of the hepatoduodenal ligament, we could get as high as the third level of pedicles through blunt dissection of the high hilar plate. This approach makes it technically possible to anastomose more directly to the ventrally located vascular and biliary structures without tension or angulation. Initially, we contemplated the use of an interposition graft, vein patch, or diaphragmatic plication and colon mobilization for the graft to obtain optimal outflow and position because we thought that the direct hepatic venous reconstruction might cause positional misalignment. However, after adjustments for the hepatic vein location, the long recipient hepatic vein cuff that was obtained facilitated the hepatic vein anastomosis without vascular modifications. In addition, the right upper quadrant fossa was sufficient to position the graft without rotation or kicking of the vascular structures. In conclusion, after careful preoperative surveillance of the vascular and biliary anatomy, LDLT using a graft from a situs inversus donor can be performed successfully by high hilar dissection and with a long hepatic vein cuff.