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Surgical Management of Complete Ureteral Duplication with Ectopic Ureter

Radu N. Balanescu¹² , Laura Topor¹, 2, Ruxandra Caragata¹, Andreea Moga¹, Gabriel Dragan¹
¹ “Grigore Alexandrescu” Clinical Emergency Hospital for Children
² “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania

 

Abstract

Complete ureteral duplication refers to a kidney with 2 ureters that drain separately and may be inserted anywhere into the bladder, the urethra or the genital system. We report the cases of three female patients diagnosed with complete ureteral duplication (two on the right side and one on the left) and ectopic insertion of the ureter into the urethra. While all patients had the same diagnosis, the surgical management in each case was different. Two patients underwent upper pole nephroureterectomy, while in the last case ureterovesical reimplantation of the ectopic ureter and inferior pole nephrectomy was performe. The postoperative evolution in each case was favorable, further follow-up having shown that all three patients are continent. The management of ectopic ureters should be decided based on a careful preoperative evaluation of both the upper and lower pole function.

Key words: operative management, nephroureterectomy, reimplantation, urinary tract infections

 

Correspondence

Laura Topor
Grigore Alexandrescu” Clinical Emergency Hospital for Children
Vaporul lui Assan Street No. 4, Bucharest Romania
Tel: +40722.984.237.
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Introduction

Complete ureteral duplication refers to a kidney with 2 ureters which drain separately and may be inserted anywhere into the bladder, the urethra or the genital system and is a rare defect, affecting less than 0.1% of children and mostly females (5:1 female – male ratio). Ureteral ectopia is associated with renal dysplasia and ureteral dilatation, resulting from reflux or obstruction. Usually these patients present with symptomatology resembling that of febrile urinary tract infections, dribbling and urinary incontinence[1,2]. The diagnosis of ureteral duplication is based on radiological exams: ultrasound, voiding cystourethrogram, intravenous pyelogram, magnetic resonance urography, CT scan or scintigraphy[3]. The type of intervention depends on the renal function of both pelvicaliceal systems, the presence of ureteral dilatation or vesico-ureteral reflux and the type of anatomical defect[1].

Case report

We report three female patients diagnosed with complete ureteral duplication (two on the right side and one on the left) and ectopic insertion of the ureter into the urethra.

Case 1: A 1 month-old baby girl presented to the pediatric ward of our clinic with repeated urinary tract infection. On admission, the examination of the external genital organs showed no vaginal opening. Cystography and urography were performed and showed vesicoureteral reflux III-IV degree and right ureterohydronephrosis (fig.1, 2). Abdominal ultrasound showed dilatation of the right kidney, with visible ureter in its proximal part and a suggestive image for right ureteral dilatation. The patient was referred to the surgical ward. The patient underwent surgery at the age of 1year. Intraoperative findings showed left ureter with a lower implantation into the bladder and 2 right ureters, one of which was dilated and opened into the urethra; on examination of the abdominal cavity, both ovaries and fallopian tubes were found, but no uterus was discovered. Ureterovesical reimplantation of the ectopic ureter and inferior pole nephrectomy was performed as primary surgery, due to the dysplasic aspect of the inferior pole.

Figure 1: Preoperative cystography

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Figure 2: Preoperative urography

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The patient’s postoperative evolution was favorable, follow up urography showed normal secretion and excretion of the kidneys and no pelvicaliceal dilatation and no reflux was visible on the cystography. A postoperative MRI confirmed the absence of the uterus and showed the presence of both ovaries. Taking into account the clinical, radiological and intraoperative findings the diagnosis of Rokitansky – Mayer – Kuster – Hauser syndrome was made and, at the age of 6, the patient underwent surgery for vaginoplasty, using the sigmoid colon.

Case 2: A 7 month old patient presented to the surgical ward with repeated urinary infections. Abdominal ultrasound showed a normal right kidney and 2 left kidney poles, the superior one measuring 3 cm and the inferior one 4.8 cm; the superior pole is drained by a 2nd ureter which is dilated, sinuous and seems to be inserted into the superior part of the urethra.

The urography showed the characteristical sign of a ,,drooping lily” (fig. 3); pelvicaliceal dilatation on the right side and duplication of the left ureter with hydronephrosis of the upper pole, while the cystography showed no reflux (fig. 4). The patient underwent surgery and an upper pole nephroureterectomy was performed. The patient’s postoperative evolution was favorable, with follow up investigations showing no reflux and no dilatation of the kidney or the ureter.

Figure 3: Preoperative urography. Drooping lily sign

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Figure 4: Preoperative cystography

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Case 3: A 2 year old patient presented with fever, pallor, right side abdominal mass, pyuria and dysuria. The urine cultures were positive for E. Coli infection. The clinical examination showed a palpable mass in the right flank (fig. 5 A, B). The ultrasound examination of the abdomen showed a normal left kidney and a 2nd degree right hydronephrosis in contact with a large polycystic mass (fig. 6). The suspicion of a possible tumor was raised and further investigations were performed.

Figure 5: A. Patient’s general appearance. B. Asymmetrical abdomen

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Figure 6: 2nd degree right hydronephrosis

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The CT scan and urography findings (fig. 7) were suggestive for complete ureteral duplication on the right side, with a right dilated, sinuous upper ureter and right ureterohydronephrosis.

Figure 6: Excretory urography

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Intraoperative findings showed pyoureteronephrosis of the upper pole, a dilated ureter which drained the superior pole of the right kidney and was inserted into the urethra and an inferior pole with secondary dilatation. Upper pole ureter nephrectomy was performed, the patient’s postoperative evolution being favorable.

Discussion

Ectopic ureters usually occur in females (female to male ratio 5:1), in 35% of cases the opening being in the urethra, in 30% in the vestibule and in 25% in the lower section of the vagina, with locations such as the uterus and the fallopian tube being rare [3]. In all our 3 cases, the ectopic ureter opened into the posterior urethra.

None of our patients had been diagnosed before birth as having any renal pathology. Age of presentation was different in each case, varying from 1 month old to 2 years old.

The symptoms for complete ureteral duplication are usually non-specific and they include: urinary tract infection, failure to thrive, abdominal pain, fever and urinary incontinence [1,4]. All our three patients presented with frequent urinary tract infections.

In ureteral duplication, the ureter draining the upper pole is usually predisposed to obstructive pathology, while the one draining the lower pole generally develops vesico-ureteral reflux[1]. Of the three, only patient 1 was diagnosed with vesicoureteral reflux.

Radiological investigation used in diagnosing ureteral duplication are ultrasound, voiding cystourethrogram, intravenous pyelogram or magnetic resonance urography; they help to assess the renal function, detect vesicoureteral reflux and exclude compression of the lower pole and urethral obstruction[3]. In all three cases ultrasound investigations were suggestive for ureteral duplication, with dilatation of the ureter draining the upper pelvicaliceal system, with the ectopic ureter opening into the posterior urethra.

The management of ectopic ureters should be decided based on a careful preoperative evaluation of both the upper and lower pole function. The standard method of treatment for complete duplication with ectopic ureter has been upper pole nephrectomy, the reason behind this being that the renal segment was considered as having no or very poor function, with the inferior pole usually having a better function[4].

In their study, Sen et al. suggest that heminephrectomy should only be performed when the upper pole appears unhealthy, but Smith et al. showed that the dysmorphic appearance of the upper pole does not necessarily mean that the histological examination will find dysplasia, thus suggesting that nephrectomy as first choice procedure is not entirely justified[6]. In all of our cases, histological examination of the upper pole nephrectomy specimens showed dysplasia. However, recent studies have shown that functional renal segments are not uncommon and thus should be preserved, usually by ureterovesical reimplantation of the ectopic ureter.

A subject of controversy in the surgical treatment of complete duplication with ectopic ureter is the management of the ureteral stump[4]. The ureter is usually sectioned as low as possible by using the same incision as that of the partial nephrectomy. By leaving behind a ureteral stump, there is a possibility of developing secondary infections. Plaire et. al revealed that in their study only 12 % of the patients who underwent heminephrectomy needed a secondary removal of the ureteral stump[7]. While all patients had the same diagnosis, the surgical management in each case was different. Patients 2 and 3 underwent surgery and the decision was made to perform an upper pole nephroureterectomy, while in patient number 1reterovesical reimplantation of the ectopic ureter and inferior pole nephrectomy was performed as primary surgery. The postoperative evolution in each case was favorable, further follow-up having shown that all three patients are continent.

Conclusions

The management of ectopic ureters is decided upon careful consideration of the function of both poles and the presence of ureteral dilatation or vesico-ureteral reflux, by either upper pole nephrectomy or vesicoureteral reimplantation.

 

 

References

1. Velaoras K. Renal duplication. Essentials in Pediatric Urology, 2012;89-94

2. Subramaniam R. Ureteral duplication and ureteroceles. In Coran AG, Caldamone A, Adzick SN et al, editors. Pediatric Surgery. 7th ed. Philadelphia: Elsevier Saunders; 2013. p. 1441-1451.

3. Temiz Y, Şimșek F, Güran S, et al. Ectopic ureter draining a poorly functioning renal moiety can be missed by IVP but not by MRU. Marmara Medical Journal 2007;20(2):118-121.

4. Ghoneimi AE, Miranda J, Truong T, Monfort G. Ectopic ureter with complete ureteric duplication: conservative surgical management. Journal of Pediatric Surgery 1996;31(4):467-472

5. Sen S, Ahmed S, Borghol M. Surgical management of complete ureteric duplication abnormalities. Pediatr Surg Int. 1998;13:61-64

6. Smith FL, Ritchie EL, Maizels M, et al. Surgery for duplex kidneys with ectopic ureters: ipsilateral ureteroureterostomy versus polar nephrectomy. J Urol 1989;142:532-534

7. Plaire JC, Pope John C. IV, Kropp BP, et al. Management of ectopic ureters: experience with the upper tract approach. J Urol 1997;158:1245-1247