EXPERIMENTAL AND CLINICAL TRANSPLANTATION

Few cases of kidney transplant have been reported in patients with situs inversus totalis. We present a 35-year old female patient with situs inversus totalis and chronic kidney disease of unknown origin who received a third graft after failure of 2 previous transplants. The first living related transplant was performed on the right side and failed after 3 years. A second related transplant was then performed on the left side, but this thrombosed graft was removed in the first week. The patient then underwent a third related transplant on the left side. Retransplant in patients with situs inversus totalis is no different than in patients with normal anatomy, provided that attention is given to the altered vascular anatomy.

Key words : Kidney transplantation, Renal failure, Vascular anatomy

Situs inversus totalis (SIT) is a rare congenital condition where thoracic and abdominal viscera and the vasculature are a total mirror image of the normal anatomy, which occurs in 1 in 20 000 people.¹ The heart, great vessels, spleen, and stomach are on the right, and the liver lies on the left, a complete reversal of the normal anatomy. Situs inversus totalis is known to be associated with anomalies in other organs, including renal hypoplasia and dysplasia.^2,3 Retransplant in patients with SIT has not been reported; here, we present a patient who underwent a successful third living donor transplant.

A 35-year-old female with renal failure of unknown etiology was found to have SIT during her transplant work-up. All her thoracic and abdominal organs and blood vessels were reversed as in a mirror image (Figure 1). In patients with SIT, the only point of concern for kidney transplant and the vascular anastomoses is that the iliac vessels on the right are deeper than on the left. With regard to the patient’s previous transplant procedures, her first donor kidney was taken from a female sibling and placed on the right side; the graft failed after 3 years and remained in situ. In the patient’s second transplant, the donor kidney was taken from her spouse and placed on the left side but was removed in the first week because of thrombosis. She then underwent work-up for a third graft to be placed on the left side because the failed first graft was still present on the right side.

The panel reactive antibody and cross-match were negative; the third donor was a female first cousin with a 5 antigen HLA mismatch. The team decided to give her 6 mg/kg of anti-thymocyte globulin and steroids as induction and tacrolimus, mycophenolate, and steroids for maintenance. Both donor kidneys had 2 arteries of equal size, and the left kidney was recovered for end-to-side implantation on the left external iliac vessels. The vessels were exposed with sharp dis-section, and the required length of both vessels was prepared for implantation. The left renal vein was anastomosed first, and then the 2 arteries were connected separately for a total rewarming time of 52 minutes. The graft showed global perfusion with brisk urine output, and good perfusion was noted on Doppler ultrasonography. The patient’s mercap-toacetyltriglycine scan showed prompt global uptake and excretion (Figures 2 and 3). Serum creatinine dec-reased steadily and was normal on day 4, and she was discharged from the hospital on day 6. The patient’s serum creatinine level 13 years later was 89 mmol/L.

Patients with SIT develop chronic kidney disease similar to patients without SIT, although a distur-bance of transforming growth factor β and Smad protein signaling is common in nephrotic syndrome and SIT.⁴ Associations have been drawn between Kartagener syndrome and renal amyloidosis and SIT, along with chronic sinusitis and bronchiectasis,^5,6 but our patient had no other associated illnesses. Siblings with SIT who develop chronic kidney disease are hypothesized to have a genetic predisposition.⁷

In our practice, if possible, all first transplants are placed in the right iliac fossa, irrespective of the laterality of the donor kidney. This is by choice and is because the right external iliac vein is more superficial and horizontal than the left, which makes the venous anastomosis easier, as right-handed surgeons have a natural forearm positioning advan-tage on the right. In SIT, vessels on the right are deeper because they mirror the vessels of the left side; in our case, we would have performed the first transplant on the left side. It is unclear why the center that performed the first transplant placed the graft on the right, whether there was awareness of the anatomical differences in SIT or not. The second transplant was done on the left side because the failed first graft had not been removed. This second graft lost its blood supply and was removed a few days after transplant and provided space for a third transplant. The third transplant was conducted on the left side, and, being right handed, the implan-tation on the left took longer (52 minutes) but achieved immediate function. Anatomy of SIT can only be detrimental if it complicates the vascular anastomoses and thereby graft perfusion. In our case, global graft perfusion became apparent immediately after declamping with brisk urine production. Kinking of the renal vein was reported in one case of SIT that required reoperation.⁷

Situs inversus totalis is a rare condition, and not many cases of kidney transplant in SIT have been reported in the literature. To our knowledge, this is the first report of a case of retransplant in SIT.

Kidney transplant in SIT is no different from cases with normal anatomy, but surgeons should be aware of the patient’s vascular anatomy to ensure a good outcome. Retransplant in patients with SIT is no different from retransplant in patients with normal anatomy, provided that attention is paid to the altered vascular anatomy.

Volume: 23 Issue: 6 June 2025

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