Orthotopic, but reversed implantation of the liver allograft in situs inversus totalis-a simple new approach to a difficult problem

Abstract

Situs inversus totalis is a rare congenital anomaly in which the heart and abdominal organs are oriented in a mirror image of normal. It provides a unique challenge as there is no established technique for liver transplantation in these patients. Employing two major alterations from our standard technique, a liver was transplanted in the left subphrenic space of a patient with situs inversus totalis. First, the liver was flipped 180 degrees from right to left (facing backward). Second, a reversed cavaplasty (anterior, not posterior, donor suprahepatic caval incision) was performed. Otherwise, it was standard, with end-to-end anastomoses of the portal vein, hepatic artery and bile duct. Three years after the entirely uneventful transplant, the recipient continues to enjoy the benefits of a normally functioning liver. The described technique prevented torsion, kinking and tension on the anastomosed structures by allowing the liver to sit naturally in an anatomical position in the left hepatic fossa. As it required no special measurements or maneuvers, the technique was easy to execute and required no donor liver size restrictions. This novel technique, with a reversed cavaplasty and a 180 degrees right-to-left flip of the liver into a left-sided hepatic fossa, may be ideal for situs inversus totalis.

Figure 1. The left-sided native liver in situs inversus totalis.

Note the stomach crossing from the right subphrenic space.

Figure 2

(A) The liver has been removed. Note an intact vena cava with an open right-sided ‘left and middle hepatic vein trunk’ equivalent and a wide-open left subphrenic space (the left hepatic fossa). The portal bypass cannula is visible in the portal vein (PV). (B) The cavaplasty with an anterior incision in the upper cuff of the donor vena cava. The back wall is being anastomosed from the inside. (C) The completed, tension-free, reversed cavaplasty.

Figure 2

(A) The liver has been removed. Note an intact vena cava with an open right-sided ‘left and middle hepatic vein trunk’ equivalent and a wide-open left subphrenic space (the left hepatic fossa). The portal bypass cannula is visible in the portal vein (PV). (B) The cavaplasty with an anterior incision in the upper cuff of the donor vena cava. The back wall is being anastomosed from the inside. (C) The completed, tension-free, reversed cavaplasty.

Figure 3. The portal vein (PV) anastomosis (in progress).

Note the lack of tension on the vein. The recipient hepatic artery (HA) trunk is also visible.

Figure 4. The completed hepatic artery (HA), portal vein (PV) and bile duct (BD) anastomoses.

Note that all three structures sit naturally, without crossing. Above is the ligated lower inferior vena cava (IVC).

Figure 5. A diagram of the orthotopic, but reversed, liver transplant.