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INTRODUCTION

Situs inversus (SI) is a rare congenital defect resulting in a complete mirror image of visceral organs (Fig. 81-1), affecting 1 out of 4000 to 20,000 newborns. Performing liver transplantation (LT) in those patients poses several challenges for both the donor and recipient. The usual anatomical configuration is know as “situs solitus,” while instances with unclear layouts are known as “situs ambiguus.

FIGURE 81-1

Recipient anatomy with complete situs inversus. Note the mirror-image orientation of the viscera.

SITUS INVERSUS RECIPIENT

Common anomalies are:

  • Right-sided suprahepatic vena cava

  • Rudimentary proper hepatic artery

  • Accessory artery off the supraceliac aorta providing the main arterial supply (Fig. 81-2)

FIGURE 81-2

The preoperative view of the patient with situs inversus. The suprahepatic vena cava is located on the right side of the vertebral column. The proper hepatic artery is rudimentary. Arterial supply to the liver is mainly provided by the accessory artery off the supraceliac aorta.

Anatomic challenges:

  • Having to place the greater right lobe of the liver over the stomach

  • Remaining empty space in the left upper quadrant predisposing to displacement of graft and kinking of vessels

Technique:

  • Varying lateral rotation of the graft (15 to 90 degrees) (Fig. 81-3)

  • Plication of left hemidiaphragm to reduce empty space

  • Retroversus rotation of the graft (Fig. 81-4)

  • Segmental living donor transplantation

  • Usually for pediatric population

FIGURE 81-3

The liver graft from the donor with normal anatomy was transplanted to the recipient with situs inversus. The graft was rotated 90 degrees clockwise. The donor infrahepatic vena cava was anastomosed to the recipient inferior vena cava in an end-to-side fashion. The arterial and biliary anastomosis was accomplished in end-to-end fashion.

FIGURE 81-4

The liver graft from the donor with normal anatomy was transplanted to the recipient with situs inversus anatomy. The graft was rotated 180 degrees along its axis. A reversed cavaplasty was made, and end-to-side anastomosis was performed between the donor IVC and recipient IVC. Hepatic artery, portal vein, and biliary anastomoses were accomplished in an end-to-end, tension-free fashion.

Split left liver transplantation (Fig. 81-5):

  • Donor hepatic artery is passed behind the pancreas and anastomosed to the

  • infrarenal aorta

  • Left hepatic vein is anastomosed to the inferior vena cava (IVC)

  • Roux-en-Y hepaticojejunostomy is performed

FIGURE 81-5

A split left lobe liver graft from the donor with normal anatomy is transplanted to the patient with situs inversus. The donor hepatic artery is passed behind the pancreas ...

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