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A patient with bilateral single-system ectopic ureters who achieved urinary continence after ureteral reimplantation
Kristin Ebert, MD; Rama Jayanthi, MD
Nationwide Children's Hospital

The patient has a history of VATER and was born at 36 weeks gestation. Her prenatal ultrasound was significant for unilateral right hydronephrosis and concern for an absent bladder. A postnatal ultrasound on day-of-life three demonstrated moderate right hydronephrosis and a left pelvic kidney with dilated calyces. A VCUG showed a urogenital sinus with two hemivaginae and a refluxing ectopic right ureter draining into the right hemivagina. A MAG3 renogram at one month of age showed a poorly functioning left kidney, right hydroureteronephrosis, and poor drainage from the right system. An exam under anesthesia at age two confirmed a gaping ectopic ureteral orifice in the right hemivagina and an extremely small bladder with an estimated capacity of 10mL. The left ureteral orifice was unable to be identified but was presumed to drain into the left hemivagina given the defuctionalized appearance of the bladder. At age 26 months, she underwent an open bilateral ureteral reimplantation. Both ureters were found to enter the ipsilateral hemivagina. There was not enough bladder muscle to perform antirefluxing anastomoses, so refluxing anastomoses were performed. At most recent follow up at age seven, the patient reports normal voiding patterns and no daytime incontinence. Even though this child had bilateral single system ureters ectopic to bilateral hemivaginas, she developed normal urinary control after reimplantation, which usually does not occur with typical bilateral single system ectopic ureters. This suggests that continence is dependent solely on bladder neck development, not ureteral location, in children with bilateral ectopic ureters.


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