Graft positioning at liver transplantation in situs inversus

Abstract

Situs inversus (SI) totalis is a rare congenital anomaly. In the past it was considered an absolute contraindication to liver transplantation (LT) because of associated malformations, and difficulty achieving accurate graft positioning. We describe successful outcome following LT in a 41-year-old with alcoholrelated chronic liver disease and complete SI using a novel technique (Fig. 1). Recipient hepatectomy was uncomplicated. A donor whole liver was implanted using a piggyback technique. Reduced space in the right upper quadrant from the stomach and spleen resulted in 40% clockwise graft rotation. To attenuate this effect, the left diaphragm was plicated and a Sengstaken-Blakemore tube was inserted percutaneously into the left upper quadrant (LUQ). The gastric balloon was inflated with 400 ml of normal saline to support the left lobe (Fig. 2). The donor remnant falciform ligament was fixed to the recipient diaphragmatic surface to effect long-term optimal positioning. Abdominal CT scan on day 7 demonstrated the gastric balloon elevating and supporting the graft (Figure 3). On day 12, 200 ml of normal saline was aspirated with gradual balloon deflation over the next 3 days, and removal of the Sengstaken-Blakemore tube on day 15. Serial Doppler ultrasonography demonstrated normal hepatic venous flow. An abdominal CT scan at 3 months demonstrated no change in graft position (Figure 4A). The hepatic veins, portal vein and hepatic artery appeared patent (Figure 4B). The patient remains well 17 months following LT with normal graft function. Abdominal SI is described in association with the polysplenia syndrome with inferior vena caval absence, preduodenal portal vein, midgut malrotation, aberrant hepatic arterial anatomy, and portal vein hypoplasia. Anatomic anomalies result in a more complex recipient hepatectomy. Consideration has to be given to correct donor graft positioning, and additional vascular reconstruction. However, LT has been performed successfully using modified surgical techniques. Most cases are in the paediatric population in whom graft displacement and hepatic venous pedicle torsion are less due to a smaller abdominal cavity when even split and reduced grafts are typically large-for-size, and greater use of caval replacement techniques. Cadaveric segmental and living related left lateral segment grafts have been successfully placed with suitable orientation for hilar vessel anastomoses. However, in adults following recipient hepatectomy, a large empty space exists in the LUQ predisposing to lateral displacement with supero-lateral graft rotation and torsion of the hepatic venous pedicle particularly with a piggyback technique. Split, reduced, and right lobe living related LT have been performed successfully. Technical modifications with an intact recipient cava include piggyback implantation over the right suprahepatic vein with orthotopic graft position, and graft rotation by 90°. In our patient, only partial volume reduction of the left hepatic fossa was achieved by diaphragmatic plication. Due to its large size, filling the defect with omentum and mobilized hepatic flexure would have been inadequate. The use of a Sengstaken-Blakemore tube represents an innovative and simple technique to provide effective graft support, and reduce the risk of outflow obstruction. Subsequent peri-hepatic adhesion formation and migration of bowel loops into the LUQ would be expected to provide longterm graft fixation.