How to do liver transplantation in situs inversus t otalis : a simple technique

Situ inversus totalis (SIT), a defect in the global situs orientation resulting in a complete mirror image of the normal arrangement of internal organs, occurs in one out of 4000–20 000 newborns.1Due to its rarity and complexity, SIT presents a unique technical challenge. The main problems for orthotopic liver transplantation in SIT are: (i) having to place the greater right lobe of the graft over the right-sided stomach; and (ii) the presence of a large empty space in the left upper quadrant predisposing to lateral displacement of the graft leading to kinking of the hepatic veins. Raynor et al. reported the first successful liver transplant in a recipient with SIT in 1988.2 Though different successful approaches have been proposed, agreement on a standard technique has not been achieved. They are: (i) plication of the left diaphragm together with stabilization of the graft with an inflated gastric balloon of the Sengstaken–Blakemore tube inserted percutaneously;3 (ii) segmental living donor liver transplantation (i.e. reduced size);4 (iii) varying degrees (15–90°) of lateral rotation (clockwise in SIT recipients or counterclockwise in donor SIT) of the graft with standard piggy-back, end-to-side or side-to-side cavo-caval anastomosis;5 and (iv) 180° flip (retroversus or backward facing) of the graft.6 Here we describe simple technical aspects of a successful orthotopic cadaveric donor liver transplant in a patient with SIT, which we consider optimal for such cases.A 44-year-old man with cryptogenic cirrhosis, previously diagnosed with complete SIT (Child-Pugh score B; Model for End-Stage Liver Disease, 24), was submitted to liver transplantation in 2010. The liver was procured in the standard fashion with the University of Wisconsin solution. The native liver weighed 825 g and the donor liver 1295 g. Back-table preparation of the donor liver involved an arterial reconstruction (an accessory left hepatic artery was anastomosed to the gastroduodenal artery) and closing of the suprahepatic vena cava with a running suture.While placing the graft in the cavity, it was rotated 90° clockwise. This allowed perfect alignment of the recipient and donor hila. The donor infrahepatic vena cava was anastomosed end-to-side to the recipient inferior vena cava. The artery and portal vein were anastomosed end-to-end. A choledococholedocostomy was established over a number 8 T-tube. The triangular and falciform ligaments were fixed to the abdominal wall. Figure 1 shows the schematic representation of the final graft placement.Figure 1Schematic representation: final placement of the graft in the abdominal cavity. T-L, termino-lateral; IVC, inferior vena cava.The patient was discharged on the 24th postoperative day. At 3 months, due to a narrow native biliary tree, the choledococholedocal anastomosis was converted to a Roux-en-Y choledochojejunostomy. Magnetic resonance imaging 3 months after the transplant showed adequate graft placement and venous drainage of the graft. Currently, the patient is doing fine 3 years after orthotopic cadaveric donor liver transplantation, has normal graft function and is asymptomatic.SIT per se is not a contraindication for liver transplantation. Preoperative evaluation of the native vascular and biliary anatomy is essential. In hindsight, a Roux-en-Y bilio-enteric anastomosis at the outset would have been beneficial and should always be considered while planning the surgery.This technique in our opinion is most suited for this anatomical variation. It has several advantages: there is no risk of kinking of the venous outflow; the hepatic hilum is aligned and allows a choledococholedocostomy (if the recipient anatomy permits); the hepatic veins drain medially and there is no stagnant pool of blood in the outflow; and there is no need for a reduced size graft, as the larger graft in this case clearly demonstrates. And finally, the same technique can also be used when the donor presents SIT.