Congenital Anomalies of The Ureter Bladder, and Urethra

Congenital anomalies of the ureter, bladder, and urethra occur in about 5% of all births. They're among the most common birth defects. Some of these abnormalities are obvious at birth; others aren't recognized until they produce symptoms. Obstructions and malformations that necessitate surgery have a good prognosis.

Causes

Signs and Symptoms

Diagnostic tests

In duplicated ureter, diagnostic tests may include excretory urography, cystoscopy, voiding cystourerthrography, and retrograde pyelography.

In retrocaval ureter, excretory or retrograde urography demonstrates superior ureteral enlargement
with spiral appearance.

In ectopic orifice of the ureter, diagnostic tests include excretory urography, urethroscopy, vaginoscopy, voiding cystourethrography, and retrograde urethrography.

In stricture or stenosis of the ureter, diagnostic tests include ultrasonography, excretory and retrograde urography, renography, and voiding cystourethrography.

In ureterocele, voiding cystourethrography, excretory urography, cystography, and cystoscopy may help to diagnose the disorder. Excretory urography and cystography may show a thin, translucent mass. Cystoscopy is used to assess the location of ureteral orifices and the changes on the submucosal tunnel.

In exstrophy of the bladder, diagnosis requires a radionuclide scan and renal ultrasonography. Buccal smears and karyotyping may be necessary.

In congenital bladder diverticulum, excretory urography shows a diverticulum obstructing the ureteral entry to the bladder. Voiding cystourethrography shows vesicoureteral reflux in the ureter, and cystoscopy confirms the diverticulum.

In hypospadias, buccal smears and karyotyping may be necessary when sexual identification is questionable.

Treatment

In duplicated ureter, surgery may be necessary for obstruction, reflux, or severe renal damage.

In retrocaval ureter, treatment involves surgical resection and anastomosis of the ureter with the renal pelvis or reimplantation into the bladder.

In ectopic orifice of the ureter, treatment consists of resection and ureteral reimplantation into the bladder for incontinence.

In stricture or stenosis of the ureter, treatment consists of surgical repair of the stricture. Nephrectomy may be necessary for severe renal damage, based on radionuclide studies.

In ureterocele, treatment involves surgical excision or resection of the ureterocele and reimplantation of the ureter.

In exstrophy of the bladder in infants, treatment includes surgical closure of the defect and bladder and urethral reconstruction to allow pubic bone fusion and normal continence and renal function. Additional measures may include bladder reconstruction with the use of an artificial urinary sphincter and reconstruction of a functional penis in males. Alternative treatment includes protective dressing and diapering. Urinary diversion eventually is necessary for most patients.

In congenital bladder diverticulum, surgery may be necessary to correct reflux.

If hypospadias is mild, it requires no treatment. If the anomaly is severe, surgical repair usually is necessary before the child reaches school age.

In epispadias, surgical repair in several stages is always necessary.