Retrocaval ureter in a 6-year-old child presenting with isolated right flank pain: A case report from Syria

Abstract

Introduction: Retrocaval ureter, or circumcaval ureter, is a rare congenital anomaly where the ureter passes behind the inferior vena cava (IVC), occurring in 0.13 % of the population. While symptoms can occur at any age, they typically arise in the third or fourth decade of life, making the case of our six-year-old patient rare and distinctive.

Case presentation: A six-year-old male presented with isolated right flank pain and a medical history of hypothyroidism and epilepsy. Despite normal laboratory tests and no urinary symptoms, renal ultrasonography revealed severe right hydronephrosis. A retrograde ureteropyelogram showed a dilated, tortuous and J-shaped proximal ureter with a collapsed distal portion. CT imaging confirmed retrocaval ureter, and a double-J stent was initially placed. Surgical intervention involved transection of the ureter and repositioning it anterior to the IVC, followed by ureteroureterostomy. The patient had an uneventful postoperative recovery and was discharged after 24 h.

Discussion: Symptoms vary depending on the degree of ureteral obstruction and hydronephrosis, with flank pain being the most common symptom. Other symptoms include recurrent urinary tract infections (UTIs) and haematuria. While intravenous pyelogram was once the diagnostic standard, CT scanning is now preferred. Management for asymptomatic patients involves watchful waiting, while surgical options aim to restore the anatomical position of the ureter, often through open surgery or minimally invasive techniques.

Conclusion: Retrocaval ureter, though rare to present in children, should be considered in cases of unexplained, persistent right flank pain. Early diagnosis and intervention are vital to preserve kidney function and prevent complications.

Keywords: Case report; Circumcaval ureter; Inferior vane cava abnormality; Pediatric surgery; Retrocaval ureter; Urology.

Fig. 1

A retrograde ureteropyelogram revealed a tortuous, J-shaped proximal ureter (black arrow) with hydronephrosis (red arrow) and a collapsed portion of the distal ureter where it passes behind the IVC (blue arrow).

Fig. 2

Multi-Slice CT scan with a DJ stent inserted. It clearly demonstrates the ureter (red arrows) encircling the IVC.

Fig. 3

Intraoperative views. (a), the ureter (black arrow) passes behind the IVC (red arrow) with dilation of the proximal portion (blue arrow). (b), both proximal and distal ureteral segments after transection (blue arrows). (c), the ureter after anastomosis (black arrow).