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Definition of Ectopic Ureter
An ectopic ureter is a ureter with an abnormally located ureteral orifice. Instead of draining into the bladder, the ureteral orifice is located in the urethra, vagina or in structures of the mesonephric duct (ductus deferens or seminal vesicles).
1:2000, more girls than boys.
See etiology of ureteral duplication.
Pathology of Ectopic Ureter
80% of ectopic ureters are associated with an ureteral duplication, especially in girls. In boys, the ectopic ureter may also drain a single renal system. In girls, the ectopic ureter drains into the urethra (35%), vulval vestibule (34%), vagina (25%) or uterus (5%). In boys, the ectopic ureter drains into the prostatic urethra (47%), seminal vesicles (33%), prostatic utricle (10%) or vas deferens (10%).
The further the distance of the ureteral opening from its normal position, the higher the likelihood of a renal malformation (renal dysplasia, renal hypoplasia) and dysfunction. If the ectopic ureter is associated with a duplex system, the ectopic ureter drains the upper pole of the kidney.
Signs and Symptoms of an Ectopic Ureter
Recurrent urinary tract infections, flank pain, fever. Additionally and depending on gender and location of the ureteral orifice:
- Girls: urinary incontinence (day and night, but sometimes also intermittent), vaginal discharge.
- Boys: LUTS, epididymitis.
Diagnosis of Ureteral Ectopia
Pelvic examination is indicated in girls with urinary incontinence, sometimes the orifice of the ureter can be identified in the vagina or vulval vestibule.
Cystoscopy and retrograde pyelography:
Cystoscopy and retrograde pyelography to search for ureteral orifices.
Ureteral ectopia may cause hydronephrosis. If a duplex kidney is present, urinary obstruction of the upper kidney portion may be visible. A dilated ureter may be detectable behind the bladder.
Intravenous urography is increasingly replaced by MR urography or in adults by CT. Urography often lacks to image in the upper part of the duplex kidney due to poor renal function. Hints for a non-contrasting upper portion is obtained from the small number of calyces shown and the greater distance of the renal system to the spine. Late images after 1–3 hours may however contrast the upper renal portion.
VCUG may detect reflux into the lower renal pole, if a duplex kidney is present.
Renal scintigraphy is indicated to determine the renal function on the side of the ectopic ureter. If a duplex system is present, the renal function of the upper and lower pole must be analyzed separately.
MRI Urography or CT:
MRI urography is the most accurate imaging tool and indicated for imaging in children, especially if unclear findings in previous investigations are present and an ectopic ureter is suspected. CT is an imaging alternative in adults which is more sensitive compared to intravenous urography.
Treatment of Ectopic Ureter
In principle, treatment is only necessary for patients with symptoms, with relevant vesicoureteral reflux or significant obstruction.
Ectopic ureter with a nonfunctioning kidney:
Standard treatment is heminephrectomy for a double system and nephrectomy for a single system; surgery is possible with a laparoscopic technique. In the case of reflux in the ectopic ureter, an additional distal ureterectomy is necessary. In the case of reflux in the lower part of a duplex kidney, ureteral reimplantation may be necessary.
Heminephrectomy is a complex operation with a small risk of organ loss due to bleeding or urinoma. Alternatively, although the partial function of the upper portion is poor, the ectopic ureter can be anastomosed (ureteroureterostomy or ureteropyelostomy) to the lower pole ureter. The procedure has fewer complications and the prognosis regarding long-term complications (UTI, hypertension) are equal (Kawal et al, 2019.
Ectopic ureter with sufficient renal function:
- Common sheat ureteroneocystostomy (UCN) for a duplex kidney with closely spaced ureteral orifices.
- Ureteropyelostomy or ureteroureterostomy for a duplex kidney and widely spaced ureteral orifices. If reflux into the ectopic ureter is present, resection of distal ectopic ureter is necessary.
- Ureteroneocystostomy (UCN), if the ureter drains a single renal system.
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