Proximal ureteral atresia, a rare congenital anomaly-incidental finding: a case report

Abstract

Ureteral atresia is a rare disease usually associated with a non-functioning dysplastic kidney. The condition may be unilateral or bilateral; focal, short or long and may involve any part of the ureter. Association with other urinary anomalies is rare. We report the case of a 10-month-old boy with prenatal diagnosis of multicystic right kidney. This suspicion was confirmed after birth by ultrasound and static scintigraphy; a right vesicoureteral reflux (VUR) was recorded at cystography. The boy presented a regular renal function but was hospitalized twice for suspected pyelonephritis between the 8th and the 10th month of life and were recorded occasional mild changes in blood pressure. Antibiotic prophylaxis was administered until surgery. When he was 10-month underwent retroperitoneoscopy to perform a nephroureterectomy finding a complete atresia of the upper third of the ureter with the blind end at the level of the uretero-pelvic-junction. The programmed surgery was performed. By a revision of literature, only few cases of imperforate distal ureter have been described. This condition is associated with a kidney dysplasia. The atresia of the ureter with no signs of infection in the dysplastic kidney may be unknown up to adulthood or throughout one's life. Prognosis usually depends on the severity of the obstruction.

Keywords: Dysplastic kidney; retroperitoneoscopy; ureteral atresia; ureteropelvic junction.

Figure 1

The technetium-labeled-dimercapto-succinic-acid (DMSA) scintigraphy. The left kidney visualized in different projections: orthotopic in position, of normal size and morphology without significant pathological defects in uptaking the radionuclide. No uptake in right kidney.

Figure 2

Voiding cystourethrogram showing a vesicoureteral reflux on the right side of grades II–III, just until the atresic point.

Figure 3

Intraoperative view. (A) The right kidney, up on the left, with dysplastic morphology, and the blind end of the ureter, down on the right; (B) dissection of the ureter and its mobilization. Traction of the cul-de-sac blind end versus the kidney.