Single-system ectopic ureters: A review of 12 cases

Single-System

Ectopic Ureters: A Review of 12 Cases By Saeed Ahmed

and Andrew

Barker

North Adelaide, Australia l Eight boys and 4 girls with single-system ectopic ureters were seen during a 15.year period. The abnormality was bilateral in 4 patients and the majority had major associated urological and other abnormalities. Eight ectopic ureters were reimplanted successfully into the bladder and 4 nonfunctioning kidneys were removed, as were 2 “blind” ectopic ureters. One patient died during the neonatal period and the others have been followed from 1 to 15 years. Renal function was noted to be normal in 10 patients and 1 has mild renal failure. Bladder function was normal in 8 patients. Early surgery should be undertaken in patients with single-system ectopic ureters. Stabilization of the upper urinary tract can be anticipated and satisfactory bladder function can also be expected in the majority of cases. Copyright Q 1992 by W.B. Saunders Company INDEX WORDS: reimplantation.

Ectopic

ureters,

single

system;

ureteric

A

SINGLE-SYSTEM ectopic ureter may be defined as a ureter that opens caudal to the bladder trigone in a nonduplicated system.’ Singlesystem ectopic ureters are usually associated with multiple congenital abnormalities and the corresponding renal unit is frequently abnormal.‘.’ We reviewed our experience with 12 cases of single-system ectopic ureters, encountered more commonly in males than in females. Early surgery was undertaken in the majority of cases, conserving renal parenchyma whenever possible. In the follow-up, 10 patients had normal renal function and 8 patients had normal bladder function. One patient had mild renal failure and one died in the neonatal period. MATERIALS

AND METHODS

Thirty-seven patients with ectopic ureters were seen during a 15-year period, including 12 cases of single-system ectopic ureters (Table 1). Five cases of single-system ectopic ureters were diagnosed during urological investigations of patients with congenital esophageal and anorectal abnormalities. One case was diagnosed after antenatal recognition of bilateral ureterohydronephrosis and one after clinical recognition of bilateral renal masses. Other presentations included failure to thrive, urinary infection, urinary incontinence, abdominal pain, and hypertension. In addition, there was also a past history of epididymoorchitis in one patient. In Table 2, the sites of insertion of single ectopic ureters are shown together with the major associated congenital abnormalities. Urethral ectopic ureters were the most common. In 4 patients, single ectopic ureter was an isolated abnormality, but in the others there were major urological or other system abnormalities. Nine kidneys were hydronephrotic, 5 showed severe dysplasia, and 2 were absent. On micturating cystourethrogram (MCU), reflux into the ectopic ureter and/or kidney was noted in 6 patients (8 systems). Single-system ectopic ureter was suspected on intravenous pyelogram (IVP) in one patient and on urinary tract ulttaJournalofPediarric

Surgery,

Vol27, No 4 (April),1992: pp

491-496

sound (US) in another. In 2 patients, ectopic ureteric insertion was recognized at cystouretheroscopy, in one at examination of the introitus under anesthesia, but one case was diagnosed only after the bladder had been opened and no intravesical ureteric orifice found. Surgical management aimed at preservation of functioning renal parenchyma, which was further assessed by performing a renal nuclide scan (RNS) using “)“Tc DTPA. All but one patient undergoing ureteric reimplantation had surgery in the first 2 months of life (Table 3). Eight ectopic ureters and one refluxing contralateral vesical ureter were reimplanted in 6 patients. The transverse advancement technique was used in the majority.’ Four nonfunctioning renal units were removed, as were 2 “blind” single-system ectopic ureters. These patients were older (aged 1 to 11 years; mean, 5 years), the delayed surgery usually being a reflection of delayed diagnosis. Additional surgical procedures included fulguration of posterior urethral valves, bladder tubularization, and eventually permanent left uteterostomy in case 8. Case 3 also had a bladder tubularization procedure. RESULTS

Case 2, who has been reported previously, died in the neonatal period.4 Case 10 was lost to follow-up at the age of 1 year, but the others have been followed from 2 to 15 years with a mean of 6 years (Table 3). Postoperative investigations included MCU, IVP, US, and RNS as considered necessary. All ureteric reimplantations were technically successful with dramatic improvement in the ureterohydronephrosis (Figs 1 and 2). Renal function was noted to be normal on biochemical criteria and also on RNS in 10 patients. In case 4, who has been reported previously, there was mild renal failure.’ Eight chiIdren, including one male (case 1) and one female (case 4) with bilateral single-system ectopic ureters, have normal bladder function. One female (case 3), who has also been reported previously, was initially managed by clean intermittent catheterization, but is now in diapers.6 One male (case 8) with multiple abnormalities has a permanent urinary diversion. DISCUSSION An ectopic ureter inserts at a site distal to the bladder trigone, is predominant in females, and is From the Department of Paediatric Surgery Adelaide Children> Hospital, North Adelaide, Australia. Date accepted: September II, 1990. Address reprint requests to Dr Saeed Ahmed, FRCS, FRAU, Department of Surgery (MBC-40), King Faisal Specialist Hospital and Research Centre, PO Box 3354, Riyadh 11211, Kingdom of Saudi Arabia. Copyright o 1992 by W.B. Saunders Company 0022..3468/92/2704-0019$03.00/0 491

AHMED AND BARKER

492

Table 1. &topic

cases, but there was no relationship between the degree of renal abnormality and the associated abnormalities. Therefore, it seems unlikely that the spectrum of abnormalities seen in single-system ectopic ureter cases is due to a critical field defect. 1 The diagnosis of single-system ectopic ureter may be incidental, in that it may be made during investigation of patients with other major problems or after antenatal recognition. The clinical syn@oms depend on the sex of the child, the position of the ectopic opening, and whether the abnormality is unilateral or bilateral. In both sexes, the patient may present with unilateral or bilateral renal masses, urinary infection, septicemia, and failure to thrive. In unilateral male cases, although there is usually no incontinence, the patient may have some urgency and frequency.’ Symptoms and signs of a genital infection may become evident, although such presentations may be delayed into adulthood.” In unilateral female cases, the patient may present with urinary incontinence despite normal voiding. The patient may also have a vaginal discharge or a vaginal cyst may be noted. Again, the presentation may be delayed into adulthood.” Bilateral single ectopic ureters are likely to present with urinary incontinence in both sexes or in a manner discussed for unilateral cases. The severity of the clinical problem is dependent on the site of ectopia, in that bladder neck and proximal urethral cases will have reasonable bladders, but more distal ectopia may be associated with very poor bladder development.2.7’12.‘3 The diagnosis of single-system ectopic ureter may be difficult, particularly in the female. A high index of

Umters, 1974-1988, Adelaide Children’s Hospital Ectopic Ureters Double System

Single System

Male

8

3

Female

4

22

12

25

Total

more commonly seen in association with complete ureteric duplication.? When an ectopic ureter is part of a nonduplicated system, it is best described as a single-system ectopic ureter, which is more common in males.’ However, vaginal single-system ectopic ureters are being reported with increasing frequency, particularly from Japan.8’9 Both the double-system and single-system ectopic ureters have the same embryological basis in that, compared with the normal, the ureteric bud arises from the mesonephric duct in a more cranial position. As a result, the ureter has to migrate for a longer time and for a greater distance and fails to reach its normal location on the bladder trigone.’ In both sexes, the ectopic ureter may insert along an ectopic pathway in the urinary or the genital tracts.’ It is generally considered that the more distal or remote the ectopic opening, the more abnormal the kidney. In these 12 examples of “urinary” ectopia, 7 kidneys had salvageable renal function, 3 kidneys were dysplastic, and 1 was absent. In the 4 examples of genital ectopia, 1 kidney was salvageable, 2 were dysplastic, and 1 was absent. The ureter is also said to be abnormal in patients with single-system ectopia, but all the reimplanted ureters in this series functioned satisfactorily.’ Major abnormalities of other systems were noted in half of the Table 2 Spectrum of Abnormalities Ectopic Sl?X

Abnormalitv

Ureter Kidnev

1

Male

Bilateral, urethral

Bilateral hydronephrosis

2

Male

Bilateral, urethral

Right hydronephrosis,

in Cases of Single-System

Ectopic Ureters

Other

Urological

Kidnev

Association

Other System Abnormalities

Nil Urethra-anal fistula

Nil EA. TEF

left multicystic, bilateral reflux 3

Female

Bilateral, urethral

Left hydronephrosis,

Urachal cyst

right agenesis,

Imperforate anus, caudal regression, congenital heart disease

bilateral reflux 4

Female

Bilateral, bladder neck

Bilateral hydronephrosis

5

Male

Left, urethral

Hydronephrosis,

Nil Absent

Congenital heart disease

Nil

Nil

reflux 6

Male

Right, urethral

Small, dysplastic

Normal

Crossed renal ectopia

Nil

7

Male

Left, urethral

Hydronephrosis.

Normal

Pelvic right kidney

Nil

very poor function 8

Male

Right, seminal vesicle

Agenesis. reflux

Normal

Posterial urethral valves

9

Male

Left, seminal vesicle

Dysplasia, reflux

Normal

Nil

10

Male

Left, vas deferens

Dysplasia, reflux

Normal

11

Female

Left, urethral

Hydronephrosis

Reflux,

Horseshoe kidney Nil

EA. TEF Nil

EA. TEF, imperforate anus lmperforate anus

dysplasia 12

Female

Right, vaginal

Smell, dysplastic

Abbreviations: EA. esophageal atresia; TEF, tracheoesophageal

Normal etresia.

Nil

Nil

SINGLE-SYSTEM

ECTOPIC URETERS

493

Table 3. Urological Management

CW3

NO. 1

Sex Male

Abnormality Bilateral, urethral

Ectopic Ureter Kidney

Other Kidney

Bilateral hydronephrosis

and Results

PrimaryUrologicalManagement, Age and Procedure 2 mo, bilateral ureteric reim-

Renal Function

Bladder Function

Normal

Normal

Normal

In diapers

Mild failure

Normal

Normal

Normal

Normal

Normal

plantation 2

Male

Bilateral, urethral

Right hydronephrosis,

Died before surgery

left multicystic, bilateral reflux 3

Female

Bilateral, urethral

Left hydronephrosis,

3 yr, left ureteric reimplanta-

right agenesis, bilateral

tion, right ureterectomy

reflux 4

Female

Bilateral, bladder neck

Bilateral hydronephrosis

3 d, bilateral ureteric reimplantation

5

Male

Left, urethral

Hydronephrosis,

Absent

2 wk, left ureteric reimplanta-

reflux 6

Male

Right, urethral

Small, dysplastic

tion Normal

11 yr, right nephroureterectomy

7

Male

Left, urethral

Hydronephrosis,

Normal

6 yr, left nephroureterectomy

Normal

Normal

very poor function 0

Male

Right, seminal vesicle

Agenesis, reflux

Normal

4 yr, right ureterectomy

Normal

Permanent left

9

Male

Left, seminal vesicle

Dysplasia, reflux

Normal

1 yr. left nephroureterectomy

Normal

Normal

10

Male

Left, seminal vesicle

Dysplasia, reflux

Normal

6 wk, left ureteric

Normal,

No follow-up

ureterostomy

reimplantation 11

Female

Left, urethral

Hydronephrosis

Reflux,

6 wk. bilateral ureteric

dysplasia 12

Female

Right, vaginal

Small, dysplastic

Normal

aged 1 yr Normal

Normal

Normal

Normal

reimplantation 2 yr, right nephroureterectomy

clinical suspicion is necessary. When there is reflux into the ectopic system, the abnormality will be recognized on MCU and when there is adequate renal function, it may also be recognized on IVP. Urinary tract US is particularly sensitive in identifying ureterohydronephrosis and it may be possible to trace the dilated ureter distally beyond the bladder. However, cystouretheroscopy may be necessary in both sexes when abnormalities of the trigone and bladder neck may be noted and the ectopic orifice identified.‘s13 In females, careful examination of the introitus and vaginoscopy is usually necessary.“‘3 Oral administration of phenazopyridine hydrochloride (Pyridium) or intravenous administration of indigocarmine or methylene blue may also be helpful and flush vaginograms may be carried out.13 Hysterosalpingography may, on rare occasions, show the ectopic ureter, but is generally applicable only to adult cases.14 The surgical management of single-system ectopic ureters must be tailored to the individual case. When there is adequate function in the affected kidney, the ureter should be reimplanted into the bladder, for which purpose we recommend the transverse advancement technique.3 The ureter may need to be shortened and tailored before reimplantation. Nonfunctioning kidneys should be removed, as should the “blind” ureters. In both situations, the ureter should be

mobilized as far inferiorly as possible, where it is ligated and cut. We do not consider it necessary to dissect the ureter flush with the organ entered.’ This conservative approach, also used by others, avoids a difficult and dangerous dissection and has not caused any complications.9 In patients with bilateral single ectopic ureters, when a major sphincter weakness is present, various major reconstructions may be necessary, but eventually the patient may require a urinary diversion.13 The two examples of bladder tubularization in this series were not successful. Our experience indicates that one can anticipate a satisfactory surgical result in patients with unilateral ectopia, although the majority require nephroureterectomy. The good results, both from the renal and bladder point of view, are explained by the fact that a good contralateral kidney ensures satisfactory bladder development. However, in case 5 with a urethral ectopic ureter, there was contralateral renal agenesis, but the result was nevertheless excellent. Bilateral single ectopic ureter is usually associated with a poor prognosis, although our results and recent reports in the literature are encouraging.“-” One of the bilateral cases died in the neonatal period and one is managed in diapers. A satisfactory result was achieved in one male and one female patient with bilateral urethral and bladder neck ectopia. Early surgical reconstruction is recommended to achieve these results.

AHMED AND BARKER

Fig 1. Case 1. (A) Early film on preoperative IVP shows advanced hydronephrosis with marked calyceal dlletation abd prominent Dunbar’s rings. (B) Delayed film shows bilateral uretbrohydronaphrosis with marked tortuousity of the ureters. (C) IVP 3 y(tars after operation showad marked improvement with good renal parenchyma, residual calyceal dilatation, and undilated straight ureters.

SINGLE-SYSTEM

ECTOPIC URETERS

Fig 2. Case 5. (A) IVP showed marked left ureterohydronephrosis. The right kidney was absent. (B) MCU showed reflux into the left ureter and pelvicalyceal system. The ureter inserted into the posterior urethra and was dilated and tortuous. (C) IVP 2 years after operation showed marked improvement with good function in the solitary left kidnev and satisfactorv drainage.

AHMED AND BARKER

496

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A: Bilateral single ectopic ureter.

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