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 Table of Contents  
ORIGINAL ARTICLE
Year : 2012  |  Volume : 2  |  Issue : 1  |  Page : 24-28

Ureterocele in adults: Management of patients in Zaria, Nigeria


Department of Surgery, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria

Date of Web Publication22-Sep-2012

Correspondence Address:
Ahmed Muhammed
Division of Urology, Department of Surgery, Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.101262

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  Abstract 

Background: Ureterocele is a cystic dilatation of the distal ureter. It remains a rare urologic condition in non-Caucasians and is even less common in adults. Single-system ureteroceles are usually found in adults, and thus the name "adult" ureterocele. Though uncommon, urologists practicing in this region should be acquainted with the management.
Materials and Methods: A retrospective review of folders of 10 adult patients who were admitted for surgical treatment of ureterocele in our center between 2000 and 2009 was done. The information obtained included: age, sex, type and number of ureterocele, associated disease, type of surgical intervention, complications, and follow-up.
Results: The age range was 20-49 years, with a mean age of 31 years. Of these patients, seven were females and three were males. Six patients had unilateral ureterocele while four had bilateral disease, making a total of 14 ureteroceles. The presenting symptoms were flank pain and painful micturition, each occurring in 60% of the patients. Genitourinary tuberculosis was diagnosed in 4 (40%) patients. Other associated pathologies were impacted stone in the ureterocele and vesical schistosomiasis. The treatment was mainly by open method (70%), while the rest had endoscopic treatment. All the patients did well and there were no significant perioperative complications.
Conclusion: The finding of associated pathology in our patients supports the theory of an acquired origin. Urologists practicing in this region need to have a high index of suspicion and be acquainted with the variable clinical presentations, radiographic features, and treatment options in order to be able to effectively manage these occasional cases.

Keywords: Adult, management, ureterocele


How to cite this article:
Muhammed A, Hussaini MY, Ahmad B, Hyacinth MN, Garba KD. Ureterocele in adults: Management of patients in Zaria, Nigeria. Arch Int Surg 2012;2:24-8

How to cite this URL:
Muhammed A, Hussaini MY, Ahmad B, Hyacinth MN, Garba KD. Ureterocele in adults: Management of patients in Zaria, Nigeria. Arch Int Surg [serial online] 2012 [cited 2019 Jun 25];2:24-8. Available from: http://www.archintsurg.org/text.asp?2012/2/1/24/101262


  Introduction Top


Ureterocele is a cystic dilatation of the distal ureter. It remains a challenging urologic condition with a wide spectrum of anatomic anomalies and variable clinical presentation. It occurs in 1:4000 children in America and Europe, [1] and is 4-7 times more common in females and found almost exclusively in the Caucasians. [1],[2] It is rare among non-Caucasians and the incidence in adults is unknown. There is debate about its etiology; while it is commonly accepted that it is congenital, some believe the adult type is acquired. The fact that it occurs mainly in children and is often associated with other congenital upper tract anomalies like duplex collecting system supports the congenital origin. It is thought to arise from incomplete dissolution of the Chwelle's membrane. [3],[4] Advocates of the origin being acquired claim that inflammation in or around the vesical wall or passage of stones is responsible for ureterocele formation. [4],[5],[6],[7] It is variously classified as single or duplex system, orthotopic (intravesical) or ectopic, while the orifices were classified by Stephens in 1954 as stenotic, sphincteric, sphincterostenotic, and cecoureterocele. [1],[3],[4] It is commonly unilateral, but is bilateral in 10% of cases. [2],[3] Diagnosis may be in utero with perinatal ultrasonography or in early childhood. These children often present with recurrent urinary tract infection, failure to thrive, incontinence, urinary tract calculus, and urethral prolapse with bladder outlet obstruction. [4],[5] Single-system ureterocele is usually found in adults, and thus the name "adult" ureterocele. It is less prone to obstruction and renal dysplasia. Adults often present with recurrent flank pain from infection, obstruction, or calculus, and the diagnosis is only made on radiologic investigations. [2],[8],[9] Despite the rarity of this condition among other races and, in this case, Blacks in our own environment, this study aims to remind urologists of the occasional occurrence of this condition, and the need for a high index of suspicion and to be acquainted with the multiple presentations, radiologic appearances, and treatment options.


  Materials and Methods Top


A retrospective review of folders of 10 adult patients admitted for surgical treatment of ureterocele to the Division of Urology, Department of Surgery of Ahmadu Bello University Teaching Hospital, Zaria, Nigeria, between 2000 and 2009, was performed. The information obtained included age, sex, type and number of ureterocele, associated disease, type of surgical intervention, complications, and follow-up. The data were displayed in tables and figures.


  Results Top


The age range was 20-49 years, with a mean age of 31 years. Of these patients, seven were females and three were males. Six patients had unilateral ureterocele while four had bilateral, making a total of 14 ureteroceles. All the males had only unilateral lesions, while all the four bilateral lesions occurred in the female patients and the only patient with a duplex system with the ureterocele found in the ureter draining the upper pole was also a female, as shown in [Table 1].
Table 1: Summary of types and number of ureteroceles

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The patients presented with flank pain and painful micturition each occurring in 60%; four of the patients with flank pain also had painful urination. Lower urinary tract symptoms (LUTS) accounted for 50% and were predominately irritative. Fever and hematuria was the reason for presentation in 40% and 30%, respectively, as shown in [Table 2]. More than 60% of the patients had one or more of these symptoms. Urine examination showed ova of Schistosoma haematobium in two patients. The entire urine specimen from patients with either flank pain and/or painful urination had pyuria, and a positive culture of bacteria was obtained in 80% percent of them. All patients were screened for genitourinary tuberculosis and 4 (40%) were found positive. Other associated pathologies found were impacted stone in the ureterocele in 5 (50%) patients, of which one each had either associated schistosomiasis or genitourinary TB [Table 3].
Table 2: Presenting symptoms

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Table 3: Associated pathologies

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Abdominopelvic ultrasound scan showed unilateral hydronephrosis in six patients and bilateral in four patients. A stone in the kidney was seen in two patients and in some a cystic vesical lesion was seen. Intravenous urography is diagnostic and the findings were hydroureter and hydronephrosis in all patients with unilateral or bilateral cystic dilatation of the distal ureter. The distal ureteric cystic dilatation is diagnostic and is described radiologically as "cobra head" or "spring onion" deformity with peripheral halo within the trigonal area [Figure 1]a, b, [Figure 2]. Impacted calculus in the ureterocele was found in four patients, bilateral in one [Figure 3]. The findings in [Figure 1] and [Figure 2] are typical of single-system intravesical ureteroceles (bilateral and unilateral, respectively).
Figure 1: (a and b) Ureteroceles with impacted stone. They also show the typical "cobra head" or "spring onion" deformity with peripheral halo in the trigonal area

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Figure 2: KUB of the same patient in figure 1 showing the stones in the trigonal and left kidney areas.

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Figure 3: Right unilateral ureterocele with impacted stone

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Treatment of the ureteroceles was mainly by open method (70%). Intraoperatively, all the ureteric orifices were found to be stenosed on one or both sides in unilateral and bilateral cases, respectively, with proximal cystically dilated distal ureter. The specific procedure included excision with ureteric reimplantation, and incision with marsupialization [Table 4].
Table 4: Type of surgical treatment

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All the patients did well, and there were no significant perioperative complications. Those who had endoscopic treatment were discharged within 48 h, while all those treated by open method were discharged between 5 th and 14 th postoperative days. Those who had ureteric reimplantation stayed longer. Follow-up period ranged between 3 months and 1 year depending on patient's compliance; they all reported remarkable improvement in symptoms. Two patients had an episode of urinary tract infection (UTI) which was treated with antibiotics. Repeat ultrasound scan showed marked resolution of hydronephrosis in all patients. Because of financial constraints, micturating cystourethrogram was not done in any of the patients to exclude reflux post-treatment.


  Discussion Top


Ureterocele is the cystic dilatation of the distal ureter. It is a congenital developmental anomaly. Several theories of its origin have been proposed. These include abnormal muscular development of the distal ureter, leading to weakness and dilatation. Another theory is that an abnormal developmental stimulus is responsible for the dilation. [1],[4] However, the most accepted mechanism is the incomplete dissolution of the Chwelle's membrane, which is usually present before the 37 th day of gestation as a division between the urogenital sinus and the developing ureteral bud. It is commonly associated with duplex upper tract and often ectopic. [3] It has varied effects as regards to obstruction, reflux, continence, and renal function.

The incidence is 1:4000 children in North America and Europe. It occurs most frequently in females (4:1) and almost exclusively in the Caucasians. [1],[3] There are a few reports about the incidence in other races, and the incidence among Blacks is not known. It is unilateral in 80% of cases and bilateral in 10% of cases. [4] The single-system ureteroceles are usually found in adults, and are thus termed "adult" ureteocele. These are usually less prone to obstruction and renal dysplasia associated with duplicated systems. [2],[9] This will likely explain why the patient in this study presented late in adulthood (age range was 20-49 years, with a mean age of 31 years). Thus, the diagnosis was not suspected prior to radiological investigations. The late presentation is not only due to the rarity of the condition among Blacks, but also because the patients were adults with a condition that commonly presents in early childhood.

Ericsson in 1954 classified ureteroceles as either simple or ectopic. They may be associated with a single or double system. The simple type is usually associated with the single system while ectopic is associated with duplex system. [2] Subsequently, Stephens in 1958 characterized ureteroceles based on the nature of their opening as: stenotic (a narrowed intravesical opening), sphincteric (when the opening is into the bladder neck), sphincterostenotic (a narrowed opening into the bladder neck), and cecoureterocele (intravesical ureterocele with submucosal extension into the urethra). [1] Ureteroceles have also been described as orthotopic or ectopic. These numerous classifications and terminologies are confusing. They are neither very clear nor helpful in guiding management decisions. In an attempt to eliminate the ambiguity, the committee on terminology, nomenclature, and classification of the section of urology of the American Academy of Pediatrics proposed standardized terms. They include: intravesical ureterocele (ureterocele contained wholly in the bladder), ectopic if any portion extends to the bladder neck or urethra, single or duplex systems and in term or the orifices as terms stenotic, sphincteric, sphincterostenotic, and cecoureterocele were recommended [4],[6] In this study, six patients had unilateral ureterocele while four had bilateral, making a total of 14 ureteroceles. All the males had only unilateral lesions, while all the four bilateral lesions occurred in the female patients and the only patient with a duplex system with the ureterocele found in the ureter draining the upper pole was also a female. Thus, 93% of these patients had single-system ureterocele which is the typical "adult" type ureterocele. Studies by Aas and Chtourou showed a similar pattern. [2],[9]

In childhood, diagnosis may be made following a child's presentation with recurrent UTI or urosepsis, incontinence, failure to thrive, urinary tract calculus, abdominal mass, bladder outlet obstruction, and vaginal or urethral prolapse. [10],[11] "Adult" ureteroceles are less prone to obstruction and dysplasia, [2] and thus are generally asymptomatic. This explains why they are detected in adulthood either incidentally or when a patient presents with recurrent flank pain, calculus, or UTI. In this study, the presenting symptoms were flank pain and painful micturition, each occurring in 60% of the patients; four of the patients with flank pain also had painful urination. Subsequent studies showed these patients with flank pain or painful urination had UTI. LUTS accounted for 50% and were predominately irritative. Fever and hematuria was the reason for presentation in 40% and 30% patients, respectively.

A number of articles have reported ureteroceles associated with other genitourinary diseases; they have been called acquired ureteroceles and are commonly found in adults. [2],[6],[7] Ureteroceles associated with schistosomiasis was reported by Umerah [6] and Elem and Sinha. [7] In this study, all patients were screened for genitourinary tuberculosis, and 4 (40%) were found positive. Other associated pathologies found were impacted stone in the ureterocele in 5 (50%) patients, of which one each had either associated schistosomiasis or genitourinary TB [Table 3]. The high incidence of associated pathologies in this study makes a case for a possible acquired origin of these ureteroceles.

Ultrasonography can confirm the diagnosis even in utero, as a cystic mass can be seen within the bladder or close to the proximal urethra. [6] Intravenous urography may demonstrate poor function on the affected side with delayed excretion or no excretion at all. It may also demonstrate hydroureteronephrosis, the dilated distal ureter becomes evident, appearing as a "cobra head" or "spring onion" deformity with peripheral halo. [3],[5] These findings were noted in the intravenous urograph (IVU) [Figure 1]. A voiding cystourethrogram (VCUG) demonstrates the size and location of the ureterocele as a filling defect or may demonstrate reflux into the ureters. Nuclear scan with DMSA (dimercapto succinic acid), DTPA (diethelene triamine pentaacetic acid), or MAG3 (Mercaptoacetyl triglycine) may demonstrate subtle changes in renal function and presence or absence of obstruction. [11],[12],[13]

Early surgical intervention is required for cure and prevention of long-term sequelae. [14] The goals of treatment are the preservation of renal function, and elimination of infection, obstruction, or reflux. Surgical treatment can be endoscopic or open. [14] Endoscopic treatment includes transurethral puncture and transurethral incision; these are applicable mainly to the intravesical types and may be curative in up to 90% of cases. [15],[16],[17] The open procedures are often reserved for the more complex types. The operative procedures include upper pole nephrectomy and partial ureterectomy in cases associated with dysplastic upper pole in a duplicated system. Intravesical excision with common sheath reimplantation is done when the upper tracts are normal or there is no indication for partial nephrectomy. [1],[14] Treatment of the ureteroceles in this study was mainly by open method (70%). The specific procedures included excision with ureteric reimplantation, and incision with marsupialization [Table 4].

The patients did well and there were no significant perioperative complications. Those who had endoscopic treatment were discharged within 48 h, while all those treated by open method were discharged between 5 th and 14 th postoperative days. The short hospital stay and probable less morbidity in those treated by endoscopic method make it an attractive choice whenever possible. Those who had ureteric reimplantation stayed the longest. Follow-up period ranged between 3 months and 1 year depending on patient's compliance, and they all reported remarkable improvement in symptoms. Two patients had an episode of UTI which was treated with antibiotics. Repeat ultrasound scan showed marked resolution of hydronephrosis in all patients. Micturating cystourethrogram was not done in any of the patients to exclude reflux post-treatment. These patients require long-term follow-up to monitor renal function, symptoms, and occurrence of vesicoureteric reflux, especially in patients treated with endoscopic method or simple open incision. [14],[16]


  Conclusion Top


The few cases reported in this study attest to the uncommon occurrence of ureterocele in non-Caucasians, but the incidence is not known. The finding of associated pathology in all but one of these patients may supports the theory of an acquired origin. Because of its rarity, physicians practicing in such areas need to have a high index of suspicion and be acquainted with the variable clinical presentations, radiographic features, and the treatment options in order to be able to effectively manage these occasional cases.

 
  References Top

1.Stephens FD. Aetiology of ureteroceles and effects of ureteroceles on the urethra. Br J Urol 1968;40:483-7.  Back to cited text no. 1
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2.Aas TN. Ureterocele. A clinical study of sixty-eight cases in fifty-two adults. Br J Urol 1960;32:133-44.  Back to cited text no. 2
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3.Chwalla R. The process of formation of cystic dilatation of the vesical end of the ureter and of diverticula at the ureteral ostium. Urol Cutan Rev 1927;31:499-504.  Back to cited text no. 3
    
4.Jeffrey PW. Embryogenesis of ureteral anomalies: A unifying theory. Aust N Z J Surg 1988;58:631-8.  Back to cited text no. 4
    
5.Richard ES. Ureterocele. Br J Surg 1973;60:337-42.  Back to cited text no. 5
    
6.Umerah BC. The less familiar manifestations of Schistosomiasis of the urinary tract. Br J Radiol 1977;50:105- 9.   Back to cited text no. 6
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7.Elem B, Sinha SN. Ureterocele in Bilharziasis of the urinary tract. Br J Urol 1981;53:428-9.  Back to cited text no. 7
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8.Tanagho EA. Anatomy and management of ureteroceles. J Urol 1972;107:729-36.  Back to cited text no. 8
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9.Chtourou M, Sallami S, Rekik H, Binous MY, Kbaier I, Horchani A. Ureterocele in adults complicated with calculi: Diagnostic and therapeutic features. Report of 20 cases. Prog Urol 2002;12:1213-20.  Back to cited text no. 9
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10.Hutch J, Chisholm ER. Surgical repair of ureterocele. J Urol 1996;96:445-50.  Back to cited text no. 10
    
11.Coplen DE, Duckett JW. The modern approach to ureteroceles. J Urol 1995;153:166-71.  Back to cited text no. 11
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12.Thompson GJ, Kelalis PP. Ureterocele: clinical appraisal of 176 cases. J Urol 1964;91:488-92.  Back to cited text no. 12
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13.Conlin MJ, Skoog SJ, Tank ES. Current management of ureteroceles. Urology 1995;45:357-62.  Back to cited text no. 13
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14.Byun E, Merguerian PA. A meta-analysis of surgical practice pattern in endoscopic management of ureteroceles. J Urol 2006;176:1871-7.  Back to cited text no. 14
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15.Rickwood AM, Reiner I, Jones M, Pournaras C. Current management of duplex-system ureteroceles, experience with 41 patients. Br J Urol 1992;70:196.   Back to cited text no. 15
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16.Monfort G, Morisson-Lacombe G, Coquet M. Endoscopic treatment of ureterocele revisited. J Urol 1985;133:1031-3.  Back to cited text no. 16
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17.Blyth B, Passerini-Glazel G, Camuffo C, Snyder HM, Duckett JW. Endoscopic incision of ureteroceles: intravesical versus ectopic. J Urol 1993;149:556-9.  Back to cited text no. 17
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]


This article has been cited by
1 An adult ureterocele complicated by a large stone: A case report
Omar N. Atta,Hussein H. Alhawari,Muayyad M. Murshidi,Emad Tarawneh,Mujalli M. Murshidi
International Journal of Surgery Case Reports. 2018;
[Pubmed] | [DOI]



 

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