Bilateral Single Ectopic Ureters Draining Into a Grossly Dilated Vagina in an Adolescent Female
A 16-year-old female presented with a complaint of involuntary leakage of urine since birth. The patient reported being able to void normally apart from the involuntary leak. The previous leak of a few drops had increased in amount over the past 2 months, thus prompting medical attention. She could manage with 2 to 3 pads during the day, with 10 to 12 daytime voids in amounts ranging from approximately 90 to 240 mL. Her nighttime incontinence was severe; she consistently woke up wetting her bed every night.\nOn examination, the external urethral meatus was normal as was the external genitalia, and the vaginal introitus was moist. Renal parameters and the results of urine analysis were normal. Ultrasonography revealed right kidney hydroureteronephrosis (HDUN) changes and an atrophic left kidney. The intravenous urogram showed left kidney calcification with nonvisualisation of the same. The right kidney was hydronephrotic with a dilated ureter and dilated vagina wrongly interpreted as a normal urinary bladder (). A diethylenetriaminepentaacetic acid renal scan showed a nonfunctioning left kidney, delayed excretion phase of the right kidney, and a glomerular filtration rate of 65.71 mL/min. On cystovaginoscopy, the external urethral meatus and urethra appeared normal, with a small urinary bladder with approximately 20-mL capacity and an absent trigone. Urine could be seen coming out through the vaginal introitus. The vagina was highly capacious but ureteric orifices could not be identified. Computed tomography revealed calcification in the left kidney and left ureter, a nonexcreting left kidney, HDUN changes on the right side with delayed excretion, and a small urinary bladder separate from the capacious vagina (). The accumulating contrast was well delineated.\nOn exploration, the urinary bladder was represented by a tubular structure engulfed by the capacious vagina. The right ureter was moderately dilated, and the left ureter was minimally dilated and calcified. Both ureters terminated on the anterolateral wall of the vagina (). Ileocystoplasty with right ureteral reimplantation was done. The bowel contributed 90% of the reconstructed bladder wall, because the native bladder was atrophic. Left-sided nephroureterectomy was done. The histopathological examination revealed tuberculosis, for which antitubercular therapy was given. There was no evidence of tuberculosis in the excised urinary bladder specimen. The postoperative period was uneventful. The patient voided well with straining and is fully continent. Clean intermittent self-catheterization fourth hourly was advised for the initial few months, which the patient was later weaned from because she maintained good voiding.