ORIGINAL ARTICLE
Year : 2007 | Volume : 12 | Issue : 4 | Page : 202-205
Single system ectopic ureter in females: A single center study
AN Gangopadhyaya, Vijay D Upadhyaya, Anand Pandey, DK Gupta, SC Gopal, SP
Sharma, Vijayendra Kumar
Department of Pediatric Surgery, IMS, BHU, Varanasi, India
Correspondence Address:
Vijay D Upadhyaya
Department of Pediatric Surgery, IMS, BHU, Varanasi - 221 005, UP
India
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/0971-9261.40835
Abstract
The purpose of this study was to inquire into the clinical features and methods for the diagnosis and
management of single-system ectopic ureters associated with renal dysplasia. Materials and
Methods: A total of 13 female patients were studied. Main stay of diagnosis was ultrasonography of
KUB region and intravenous urography and renal scan was used to confirm the diagnosis.
Histopathological evaluation was done in all cases for documentation of renal dysplasia. Result: In
eight cases ectopic ureter with dysplastic kidney was seen on left side and in five it was on right side.
All the patients were treated with nephroureterectomy of the affected side because of poor functioning
of ipsilateral dysplastic kidney. Conclusion: Continuous urinary incontinence in females with a
normal voiding pattern should prompt an evaluation for ureteric ectopia and when initial evaluation
yields diagnosis of solitary kidney the clinician should be aware of the possibility of a hypoplastic
and/or dysplastic on one side and normally functioning kidney on opposite side. Nephroureterectomy
is the treatment of choice for unilateral single system ectopic ureter with renal dysplasia of affected
side.
Keywords: Dysplastic kidney, females, nephroureterectomy, single system ectopic ureter
How to cite this article:
Gangopadhyaya A N, Upadhyaya VD, Pandey A, Gupta D K, Gopal S C, Sharma S P, Kumar V. Single system
ectopic ureter in females: A single center study. J Indian Assoc Pediatr Surg 2007;12:202-5
How to cite this URL:
Gangopadhyaya A N, Upadhyaya VD, Pandey A, Gupta D K, Gopal S C, Sharma S P, Kumar V. Single system
ectopic ureter in females: A single center study. J Indian Assoc Pediatr Surg [serial online] 2007 [cited 2015 Apr
12];12:202-5. Available from: http://www.jiaps.com/text.asp?2007/12/4/202/40835
Introduction
An ectopic ureter is defined as an ectopic ureteric orifice outside the posterolateral extremity of the
bladder trigone. [1] An ectopic ureter that inserts either into the urethra distal to the sphincter or into the
vagina in a girl typically presents with continuous wetting despite an otherwise normal micturition
pattern. In most cases, the ectopic ureter arises from the upper moiety of a duplex kidney and
detecting such an ectopic system is relatively straightforward. However, in a small proportion of
patients the ectopic ureter may arise from a small, dysplastic and poorly functioning kidney. In
general, 80% of ectopic ureters arise from the upper pole of a completely duplicated system. Ectopic
ureters draining single systems are rare [2],[3] occurring only in 20% cases. [4] Successful removal of the
dysplastic kidney with its draining ureter results in immediate and complete cure of the incontinence
problem. However, the small nonfunctioning kidney, which can often be ectopic, may defy detection
even with repeated examinations using conventional imaging modalities and cystourethrovaginoscopy
under anaesthesia, thus leading to a significant delay in the correct diagnosis and appropriate
treatment. Though this moiety is more common in male, but contrary to the literature we have
encountered all 13 cases in female patients during this period. We retrospectively analyzed the
clinical features and methods for the diagnosis and management of single-system ectopic ureters
associated with renal dysplasia.
Materials and Methods
The study was carried out at the university hospital BHU, Varanasi. A total of 13 females of single
system ectopic ureter associated with dysplastic kidney were admitted from 1995 to 2005. In a
retrospective review, the case notes, including presentations, findings on examination, imaging
studies, operative details, pathology findings and postoperative outcomes, were studied. All patients
were subjected to intravenous urography (IVU), ultrasound and renal scan. Prior to exploration,
cystourethrovaginoscopy was done in all patients after filling the bladder with methylene blue dye
either via per urethral route or by suprapubic approach under general anesthesia and exploration was
performed in all 13 patients [Table - 1] and renal dysplasia was further confirmed by pathologic
examination.
Results
All the patients were girls, with a median age of 2.5 years (range four months to three years). Eight
cases occurred on the left and five on the right side. The patients had developed and grown normally,
presented with wetting as well as normal voiding. They had the same volume of dribbling during the
daytime as well as in night, without any relation to position. Two patients had history of abdominal
pain. None of them presented with features of urinary tract infection. Methylene blue test was positive
in 10 cases.
All the patients were examined by ultrasonography, intravenous urography and renal scan [Table - 1].
Intravenous urography showed very poor function of affected kidney in nine cases where as in four
cases it was non conclusive however hypertrophy of contralateral kidney was observed in 30.7% of
cases only. Renal scan was done in all cases for confirmation as well as for decision making. In renal
scan, affected kidney was visualized in 12 cases (differential function was ranging form 1-7% in
affected kidney) and in one case affected kidney was not visualized. Perineal examination including
vaginocystoscopy revealed ectopic opening in vagina in five cases, in four cases the ectopic opening
was present in vestibule whereas in one case, the opening was present in urethra and was not
visualized in three cases. However methylene blue test showed clear fluid in perineum which
indirectly confirms that ectopic opening is outside the sphincter.
All patients underwent nephroureterectomy of the affected side. In five cases the affected kidney was
present at its normal position and in eight cases it was present in pelvis. The size of affected kidney in
all cases was very small ranging from 3 × 2 × 1 cm to 4.2 × 2.6 × 1.5 cm [Table - 1]. During operation
we were not able to identify renal vein and artery separately and mass ligature was done in nine
cases where as in four cases both of them were ligated individually.
Dribbling and wetting disappeared right after operation. All patients recovered well and were
discharged on the fifth to seventh postoperative day. At follow-up after one to seven years, they had
developed normally. The pathology examination reported renal dysplasia.
Discussion
Urinary incontinence is a common symptom in children; under most circumstances it is caused by
functional disturbances of the detrusor-sphincter complex, the treatment outcome of which is often
unpredictable. Wetting from organic causes, e.g. infrasphincteric ureteric ectopia, is much less
common but is particularly important because of the potential for immediate cure by surgical
intervention. A typical history of continuous or intermittent dribbling of urine despite an otherwise
normal pattern of voiding, often starting in early childhood, should prompt a clinical suspicion of an
ectopic ureter. The diagnosis can be further affirmed by a simple wet-pad test for dribbling of clear
urine after the introduction of methylene blue into the bladder.
As per the literature single-system ectopic ureter are more common in male. [5],[6] We are presenting a
series of 13 cases in females and contrary to literature we have not encountered any male patient
during this period. Such ectopic ureters are frequently found in association with VACTERL
syndrome [2],[7] though we observed this association in only 15.45% of cases. A small, dysplastic,
poorly-functioning single kidney associated with an ectopic ureter may be difficult to localize, leading
to delay in diagnosis or even to misdiagnosis as a solitary kidney. In the present series most of
affected kidneys were localized by renal scan which also help in decision making of the management.
The etiology of renal dysplasia is not yet quite clear. The presumed embryologic mechanism is that
the ureteric bud arises more craniad than usual from the mesonephric duct, [8] so that it is taken into
the bladder wall at a lower level during the seventh week of gestation or does not join the bladder wall
at all. Maizels and Stephens postulated that abnormal growth of the spine precludes normal renal
ascent and may even be responsible for renal agenesis and hypoplasia. [9] The ostium localizes along
a defined pathway from the bladder neck to the seminal vesicles in males where as it opens into the
mesonephric duct remnants in females. This abnormal location of the ureteral bud probably leads to
abnormal interaction with nephrogenic blastema and renal dysplasia. [5],[10],[11],[12] Therefore, an ectopic
ureter is frequently associated with ectopia of the corresponding kidney and a variable degree of renal
dysplasia. [8] It is also postulated that failure of vascular supply during embryologic development
prevents the kidney from developing normally and causes formation of a small primary organ that
contains embryonal tissues. Some researchers believe that it is the obstruction of the ureter during an
early phase of the embryo that stops development of the kidney. [13] All 13 cases had renal dysplasia,
in five cases kidney was located at normal site whereas in eight cases it was at lower location, ureter
was narrow, non fibrosed and non obstructive, except in one case where the ureter was dilated and
tortuous.
Depending on the location of the ostium, ectopic ureters may produce a highly variable clinical
character which delays the diagnosis and therapeutic result. An ectopic opening into the bladder neck
may produce obstructive uropathy, vesicoureteral reflux, recurrent infections or enuresis ureterica;
that is, urinary dribbling associated with an otherwise normal voiding pattern results from ectopia
distal to the bladder sphincter in female patients. [13],[14]
Highlights of clinical and pathologic features in our series are: A) Our series consists of all girls,
similar to reports by Chowdhary et al. [15] and Li et al. [16] though others had reported that singlesystem ureteral ectopia is more commonly in males. [6] B) All the patients were under the age of three
years of which two were infants. C) in 38% of cases, kidney was located at normal position. Was not
dilated, distorted or fibrosed in 12 cases. D) Strong association with ipsilateral renal dysplasia noted
by others was confirmed in our series. [6],[16],[17],[18],[19] E) The ureter orifice was ectopic but the ureter
was not dilated or distorted, not fibrosed in 12 cases, which is different from the dilated and distorted
ectopic ureter seen with duplex system. F) We used IVU primarily for locating the kidney as well as for
contralateral functional hypertrophy and renal scan to confirm the diagnosis which also helps in
decision making of the management, where as other have used computed tomography for the same.
G) Except for mild wetting and dribbling, these patients had no other symptoms and the severity of
wetting and dribbling was far milder than that of patients with duplicated kidney (indirect evidence of
dysplastic kidney) and an ectopic ureter.
Intravenous urography and ultrasonography are the most common examinations for diagnosis of
ectopic ureter. In the present series in six cases, affected kidney was localized by ultrasonography
whereas Borer et al. were not able to detect single ectopic kidney in their series though Li J et
al. [16] were able to locate the kidney in seven out of eight of the cases. In present series in 61%
cases the affected kidney was located by IVU which is contrary to the report of Li J et al. Kawamura
N et al. [20] used renal scan where IVU was not able to detect the ectopic kidney in the cases of poor
functioning single ectopic ureter but in our series all of these patients were examined by renal scan
which also helped us in decision making. Recently laparoscopy is used to localize the position of
ectopic kidney and was able to detect the position of ectopic kidney in all cases.
Treatment can be in form of ureteral reimplantation when relative renal function is 10% or more [3] or
nephroureterectomy when relative renal function is less than 10% [16],[21] . In patients with bilateral
anomalies or marginal renal function, reimplantation is of choice for salvaging renal mass. [22] In our
series, excision was a simple and valid treatment. Before ureteronephrectomy, it is better to fill the
bladder with methylene blue saline either via a thin needle or perurethrally and observe whether blue
liquid comes from the perineum so as to confirm the diagnosis. Because the dysplastic kidney is
usually located abnormally in the retroperitoneum below the normal kidney location, care is needed
not to misdiagnose it as a solitary kidney by exploring only the normal kidney location near the lumbar
vertebrae. [23],[24] Its merits include minor trauma, less time spent, rapid recovery, short hospitalization
and low cost and it may be especially suitable when the precise location of the dysplastic kidney
cannot be predicted preoperatively.
Conclusion
Continuous urinary incontinence in females with a normal voiding pattern should prompt an evaluation
for ureteric ectopia. When the initial evaluation yields the diagnosis of a solitary kidney, clinicians
should be aware of the possibility of a hypoplastic and/or dysplastic, often ectopic, contralateral
kidney with an ectopically draining ureter. Nephroureterectomy is the treatment of choice for unilateral
single system ectopic ureter with renal dysplasia of affected side.
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Tables
[Table - 1]
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