Difficult Management of a Patient with Complicated Bilateral Duplication of Renal Collecting Systems and Associated Pathology

A 31-year-old female with a 19-year history of chronic urinary tract infections, intermittent hematuria, and associated flank pain was referred to our service for evaluation and treatment. Increasing frequency and severity of the infections prompted the following diagnostic studies to elucidate the etiology of the patient’s condition: abdominal and pelvic computed tomography scans, intravenous pyelography, voiding cystourethrography, kidney ureter and bladder x-rays, flexible cystoscopy, bilateral renal ultrasonographies, cystourethroscopy with sounding, bilateral retrograde pyelographies with fluoroscopy, and ureteroscopy. Our studies revealed an uncommon, bilateral duplication of the urinary collecting systems. Upper and lower pole collecting systems, each with their own ureter, were discovered bilaterally. Chronic changes of the upper pole collecting system of the left kidney were observed, including hydroureter, caliectasis, and renal calculi. Furthermore, a large ureterocele, measuring 4 cm in length x 2 cm maximum transverse diameter, was observed in the distal portion of the left upper pole ureter. This ureter inserted into the proximal portion of the external sphincter muscle of the female urethra. These findings are consistent with an obstructive process that resulted in chronic damage to the left upper pole collecting system, and presents a difficult clinical scenario to manage. The duplicated collecting system on the right showed normal function despite its anatomical variation. Based on this finding, we believe that the location where the duplicated ureter inserts into the bladder is more predictive of chronic renal insult and associated pathology versus the presence of the variant alone causing problems.