A 15-year-old, female was born with anorectal malformations with a vaginal fistula. There was an atresia of the anal sphincter with a fistula in the vestibule of the vagina and agenesis of the coccyx. At two years old she underwent a colostomy and one year later abdominoperineal proctoplasty with Romualdi’s technique was performed. At 11 years old investigations showed pyelonephritis of a single left kidney. At 14 years old the GFR began to decrease and CKD stage 2 was developed. CKD progressed in six months and the patient began renal replacement therapy by HD. When she came for kidney transplantation, the GFR (creatinine clearance) was 1,48 ml/min. Laboratory results showed that the patient had anemia (hemoglobin level of 73 g/L, RBC-2.6 million, Ht-23%). Proteinuria level – 1.65 g/L. We found an infection in the urine. The patient had a positive urine culture with 100,000 CFU/ml of Escherichia Coli which was treated by antibiotic. We evaluated the patient's mother as a live donor (41 years old). The donor was AB0 compatible with two HLA mismatches (HLA-A and HLA-B) and a negative crossmatch test.

The patient underwent a kidney transplant. During the surgery, we revealed duplicated IVC and transposed iliac arteries. The renal graft vein was anastomosed end-to-side to the right IVC and the artery by end-to-side to the right iliac artery. Graft function was immediately observed during surgery. The kidney function in the one-year posttransplant period was good. There were no signs of urinary tract infections or other complications. On CT-angiography we found the IVC duplication with the two connections between the right and left IVCs with the formation of an “anomalous circle”, high-positioned BAA at the level of L2 vertebral body, and transposition of right and left iliac arteries. Also, we revealed the right kidney aplasia and the absence of blood circulation in the left native kidney. Graft function, vessels, and ureterovesical anastomoses were in good condition.

Anorectal malformations with a vaginal or urethral fistula may be associated with IVC, abdominal aorta, and iliac arteries anomalies with renal aplasia. Early diagnosis of such congenital anomalies could prevent the development of pyelonephritis of a single kidney with end-stage renal failure. In our case, a delayed diagnosis of pyelonephritis resulted in the progression to ESRD, necessitating a kidney transplant where we found all these anomalies. It confirms the asymptomatic course of these anomalies, diagnosed only during radiological imaging or surgical intervention. Such patients should undergo complete investigations before making surgical decisions. Diagnosis of this pathology in the preoperative period, especially in transplant patients, will help to avoid intraoperative difficulties because such anomalies can be presented as IVC transposition or aplasia.