European Urology European Urology 41 2002) 323±327 Current Role of Simpli ed Upper Tract Approach in the Surgical Treatment of Ectopic Ureteroceles: A Single Centre's Experience J. Gomes a,*, M. Mendes a, R. Castro b, A. Reis b a Department of Urology, St. JoaÄo Central Hospital, Alameda Prof. HernaÃni Monteiro, 4200 Porto, Portugal b Department of Urology, Hospital Maria Pia, Porto, Portugal Accepted 8 January 2002 Abstract Objective: Despite renewed interest in either endoscopic decompression or complete reconstruction as primary approaches in ectopic ureterocele's EU's) treatment, we advocate that a simpli ed upper tract approach is an optimal choice in a large subset of these patients. We review our surgical results to evaluate the effectiveness of different surgical procedures used in the treatment of EU, based on an individualised approach. Patients and Methods: We retrospectively analysed all patients with EU and without previous treatment n ˆ 59) operated in this centre between the years 1991 and 2000. This disorder was bilateral in three patients 62 EU) and associated with duplex ureters in 60 cases. Vesicoureteral re ux VUR) was diagnosed pre-operatively in 49.1% of patients. Treatment and choice of procedure was based on patients' age, clinical manifestations, associated abnormalities and function of the ureterocele-bearing moiety. Patients were divided in three separate groups according to the initial surgical approach. Group 1±11 patients submitted to endoscopic puncture six urgent ureterocele decompression, four infants with obstruction of a functional ureterocele-bearing moiety and another with both lower pole ureter and mild bladder out ow obstruction). Group 2±30 patients 31 EU) submitted to a simpli ed upper tract approachðpyeloureterostomy 1), heminephrectomy 29) and nephrectomy 1) all patients but one with non-viable renal segments; VUR, grade III, present in six cases). Group 3±18 patients 20 EU) submitted to complete primary reconstruction all patients with high-grade and/or contralateral re ux, with or without function of the ureterocele-bearing moiety). Success clinically asymptomatic patients, without obstruction or VUR) and complication rates from the different approaches were analysed. For statistical evaluation, we used Fisher's exact test with p 0:05 considered signi cant. Results: The success rate of endoscopic treatment, simpli ed upper tract approach and complete reconstruction were 18.2%, 80% and 83.3%, respectively. Major complications occurred in two patients from group 3. Conclusion: Endoscopic puncture is our rst option when immediate ureterocele decompression is required, although it seldom affords de nitive treatment. Complete primary reconstruction is necessary in complex cases. Cure rates are high but there is a potential risk for serious complications. A simpli ed upper tract approach is curative in most patients without associated re ux. Heminephrectomy remains our rst choice in a large subset of patients with non-viable or hypofunctional renal segments and without high-grade re ux. # 2002 Elsevier Science B.V. All rights reserved. Keywords: Ectopic ureterocele; Individualised approach; Simpli ed upper tract approach 1. Introduction Ectopic ureterocele EU) accounts for the great majority of ureteroceles diagnosed in children. It's clinical * Corresponding author. Tel.: 351-22-422-3342. E-mail address: semogarierom@hotmail.com J. Gomes). manifestations are quite variable and include urinary tract infections UTI), vesicoureteral re ux VUR), ureteral and bladder obstruction, renal insuf ciency and continence problems. This pathology's accurate natural history is still unknown. However, in most cases the clinical manifestations mentioned above force a surgical intervention in infancy or childhood [1±3]. 0302-2838/02/$ ± see front matter # 2002 Elsevier Science B.V. All rights reserved. PII: S0302-2838 02)00042-8
324 J. Gomes et al. / European Urology 41 2002) 323±327 There is still some controversy about the best surgical management for EU. Different choices include an endoscopic puncture [4], a simpli ed upper tract approach total or upper pole nephrectomy combined with partial ureterectomy, or pyeloureterostomy) [5] or complete reconstruction ureterocele excision and ureteral reimplantation with or without ablative surgery of the upper tract) [6]. In recent years, the role of an isolated upper tract approach consisting in partial nephrectomy in most cases) has been questioned by its high reoperation rates in an unselected group of patients. There has been a growing interest with either early endoscopic minimally invasive surgery to preserve/improve moiety function and reduce the risk of severe infection [4,7] or primary complete reconstruction, as the most de nitive approach, with cure rates over 85% [8±10]. An individualised approach based on clinical manifestations, patients' age, associated abnormalities presence and grade of VUR, ureteral or urethral obstruction) and function of the ureterocelebearing moiety allows the selection of the least invasive and in many cases de nitive surgical procedure. We reviewed our surgical results to evaluate the effectiveness of different surgical procedures, based on an individualised approach. Results are presented considering rst intervention's success rates, although the main objective was not to ensure resolution with a single procedure in all cases. 2. Material and methods In a period of 10 years 1991±2000), we operated 59 patients 46 girls, 13 boys) with the diagnosis of EU and without previous surgical treatment. Age at surgery varied between 7 days and 10 years mean age 21 months). Clinical presentation is shown in Table 1. The diagnosis included the performance of a kidney±bladder ultrasonography US) in all patients, complemented in doubtful cases with intravenous urography IVU), 99m technetium diethilenetriaminepentaacetic acid DTPA) renography or 99m technetium dimercaptosuccinic acid DMSA) scintigraphy. Voiding cystourethrography VCUG) was performed pre-operatively in all but two patients two cases of urgent ureterocele puncture). Urethrocystoscopy was performed when there were doubts in the characterisation of the ureterocele. Table 1 Clinical presentation leading to the diagnosis of ectopic ureterocele Foetal US 31 UTI 26 Acute urinary retention 1 Renal failure 1 US: ultrasonography; UTI: urinary tract infection. No. of patients The ureteroceles were classi ed according to the classi cation system proposed by the Urology Section of the American Academy of Paediatrics [11]. The EU involved the left side in 30 patients, the right side in 26 and both sides in three. From a total of 62 EU, 60 were associated with a duplex-system double ureters) and two with single-system. VUR was identi ed in 28 children 28/57, 49.1%), in all the cases involving the ipsilateral lower pole ureter and in ve patients with contralateral involvement as well. Indications for surgery included breakthrough febrile UTI, bladder or ureteral obstruction and persistent high-grade re ux. We performed endoscopic puncture mainly when rapid decompression of the EU was required. We also used this approach in some infants with obstruction of viable ureterocele-bearing moieties in one patient, without associated abnormalities, we performed a pyeloureterostomy). Partial nephrectomy was our rst choice in all patients with hypofunctional renal segments when re ux was not a major part of the clinical picture both in magnitude or number of affected ureters). Otherwise signi cant moiety function associated with re ux or high-grade re ux) we proceeded with a complete reconstruction. Whenever possible, we limit this surgery to older children. When deciding between salvaging or ablative upper tract surgery in complete reconstruction, we use a 10% limit of split renal function on DMSA scintigraphy. However, in patients with decreased glomerular ltration rate we try to preserve the affected renal segment even with slightly inferior percentages. Patients were separated in three different groups according to the initial surgery performed. Group 1 included 11 patients 11 EU) submitted to endoscopic puncture of the most distal intravesical portion of the ureterocele. VUR was present pre-operatively in four out of nine patients evaluated. This surgery was performed in four patients with infected ureterocele and urosepsis, one acute urinary retention, one obstructive renal failure, four infants with obstruction of a viable ureterocele-bearing moiety and another with both lower pole ureter and mild bladder out ow obstruction. Group 2 included 30 patients 31 EU) submitted to a simpli ed upper tract approachðheminephrectomy with sub-total ureterectomy up to the iliacvessels or bladder) n ˆ 29), pyeloureterostomy n ˆ 1) and nephroureterectomy n ˆ 1, a single-system ureterocele). VUR was present before surgery in six cases grade III in two). Group 3 included 18 patients 20 EU), all with ipsilateral re ux to the lower pole ureter grade III±V in 16 cases and involving the contralateral ureter in ve). We performed complete ureterocele excision with trigonal and bladder neck reconstruction in all cases, associated with upper pole ureter tailoring and reimplantation of twin ureters using a Cohen advancement technique n ˆ 9) or heminephrectomy with total ureterectomy n ˆ 11). Contralateral ureter reimplantation was performed in four cases. Patients were rst observed 1 month post-intervention. Thereafter, the periodicity depended on the outcome of the surgery, and in successful cases it included observations at 6, 12 and 24 months. In younger children, there is still another observation around age ve. We performed a clinical evaluation, analytical renal function assessment, urine bacteriology and kidney-bladder US. A VCUG was done 6±12 months after surgery in all cases. Its repetition and periodicity depended on the results of the rst examination. Screening for bladder lling defects urinary incontinence with or without bladder instability) was based on anamnesticsymptoms in children who have achieved toilet training. Urodynamic studies were performed when clinically indicated. Cure was de ned as clinically asymptomatic patients, without neither ureteral nor bladder out ow obstruction and without VUR.
J. Gomes et al. / European Urology 41 2002) 323±327 325 For statistical evaluation, we used Fisher's exact test with p 0:05 considered signi cant. 3. Results Mean follow-up time was 33 months minimal 12 months and maximal 9 years) considering the latest observation Table 2). In group 1, ureterocele's re ux occurred in four patients. Re ux to the lower pole ureter persisted in all four patients diagnosed pre-operatively. Breakthrough febrile UTI, persistent high-grade re ux and reobstruction motivated reintervention in eight patients. A total of 10 surgeries, including two repunctures, two heminephrectomies, one nephroureterectomy and ve bladder reconstructions combined with upper pole resection in two cases were performed in these patients between 2 and 36 months after the rst intervention, mean 24 months). Only three children remained clinically asymptomatic, without obstruction and without further need for surgery. One of these patients is on prophylactic antibiotics for low-grade re ux. The success rate of the rst intervention was 18.2% 2/11). However, immediate ureterocele's decompression was achieved in all cases and short-term decompression was successful in nine children, allowing patient's stabilisation in all cases it had an urgent setting. Upper pole's function preservation was achieved in three out of four patients in whom the early endoscopic treatment had this speci c goal. All three patients underwent ureteral reimplantation. New onset VUR occurred in four patients from group 2, including re ux to the ureteral stump in two, one of which was with previous involvement of the lower pole ureter as well. In three cases 3/9) VUR subsequently disappeared over a period of 22 months two patients with pre-operatory and one with de novo re ux). A fourth patient is still on prophylactic antibiotics for low-grade re ux 15 months follow-up). The other ve patients four of which with pre-operatory re ux) required a secondary lower tract reconstruction, for breakthrough febrile UTI associated with VUR 4) and persevering high-grade re ux 1). Mean duration of conservative treatment in these patients who underwent a second operation was 25.4 months range 9±30 months). These results con rm that pre-operatory re ux is a risk factor for an unsuccessful outcome with this approach p ˆ 0:007; relative risk ˆ 8:00, range 1.89±33.85, 95% con dence limits). Overall upper tract approach was successful in 24 patients 24/30, 80%). Although, there is no signi cant difference in pre-operatory re ux in patients from groups 1 and 2 p ˆ 0:41), we nd that an upper tract approach is signi cantly more successful in treating this patients two-tailed p ˆ 0:0005). Fifteen patients from group 3 were cured by a single intervention success rate 15/18, 83.3%). VUR resolved in all patients but occurred de novo in one contralateral low-grade re ux) managed by prophylactic antibiotics. We had two major complications in this group, both in patients younger than 30 months at the time of surgery: one case of bilateral ureteral obstruction submitted to bilateral ureteroneocystostomy and one case of dysfunctional voiding managed by clean intermittent catheterization. Using our selection criteria in choosing an upper tract approach or a complete reconstruction, there are no differences in the expectation of a successful outcome between these two approaches two-tailed p ˆ 1). There is no record of stress urinary incontinence. All cases of monosymptomatic enuresis resolved by the age of 11. Besides the case mentioned above in group 3, three other children one from group 2, one from group 3 and one from group 1 subsequently submitted to complete reconstruction) underwent urodynamic evaluation for urgency and diurnal frequency in two and urge incontinence in one the latter). All three presented uninhibited detrusor contractions, with no Table 2 General results after the first intervention Group No. of patients No. of EU) VUR a Cure %) No. of patients with long-term complications Pre-operatory b New onset c No. of patients submitted to reintervention 1 11 11) 4/± d 4/± 18.1 ± 8 2 30 31) 6/2 4/1 80 ± 5 3 18 20) 18/18 1/± 83.4 1 1 a VUR: vesicoureteral reflux. b Number of patients with pre-operatory VUR/Spontaneous or surgical resolution. c Number of patients with new onset VUR/Spontaneous resolution. d In this group, only nine patients were evaluated pre-operatively with voiding cystourethrography.
326 J. Gomes et al. / European Urology 41 2002) 323±327 other remarkable bladder abnormality. All cases were asymptomaticwithout medication by the age of 9 years. 4. Discussion Like in other reports [8,12,13], our results suggest that endoscopic puncture is seldom a de nitive procedure in the management of EUs. In our series, 72.7% of patients required a second intervention on long-term follow-up, mainly for symptomaticre ux. Its simplicity and ef ciency in accomplishing immediate ureterocele decompression makes this approach our rst choice in urgent settings, not only in cases of urosepsis but also in patients presenting with renal failure. We don't advocate performing endoscopic puncture to preserve or improve function in hypofunctional renal segments unless renal reserve is decreased, since in most cases of true EU the involved renal segment is hypodysplastic[1]. Still, there are exceptions to this correlation between ureteral ori ce position and kidney morphology. In some cases the metanephric mesenchyme is well induced despite ureteral ectopy and renal moiety function is worth preserving. This assumption led us to perform an endoscopic puncture in four infants one of which was without associated abnormalities). However, new onset re ux is a major concern when operating uncomplicated ureteroceles, especially at an age when renal scarring is a problem. The usefulness of upper pole preservation has to be balanced against the necessity or anticipation of a bladder approach. Ureterocele excision and correction of all associated abnormalities is considered the most de nitive approach, with the highest success rates independent of patients' age at surgery [8±10]. A success rate of 83.3% in a group with six infants seems to support this point of view. However, we feel this is a technically demanding surgery, with potential serious complications, especially when performed in immature bladders, as it happened with two of our patients, though fortunately without long-term sequels in one case. More important, in a large group of patients this is not an essential procedure to attain cure. We reserve a complete primary reconstruction when high-grade VUR is present, when more than one ureter is affected or when continence problems ensue due to muscle de cit of the bladder neck and urethra although no such case was found in our review). Others have demonstrated that in these complex cases, less invasive approaches exceptionally attain cure [8,12]. Excision of non-viable renal segments and ureterocele's decompression is usually curative in patients without associated abnormalities, since non-re uxing ureteral stumps rarely become infected [2]. In group 2, 23 patients without VUR were submitted to partial or complete nephrectomy and only one needed reoperation while another is still on antibioticprophylaxis 91.3% success rate). Yet, from six patients with preoperatory re ux grade III in two cases) four underwent a second operation. Still, a bladder surgery was obviated in two of these patients 33.3%) with a low morbidity procedure. Since clinical evolution dictated the time of reintervention, the other four patients suffered little or no morbidity in terms of pyelonephritis, urosepsis or deterioration of renal function) during the period between the two operations. We feel this justi es our policy regarding patients with lowgrade re ux. Considering most patients present with non-viable or hypofunctional ureterocele-bearing moieties, heminephrectomy remains our rst choice in patients without high-grade re ux. Obstruction of viable renal segments without VUR seems appropriately managed by pyeloureterostomy. However, at our institution this surgery is rarely a rst option for fear of interfering with lower pole ureter's integrity. Reports on urinary incontinence and voiding dysfunction in this complex pathology are controversial. Several studies show that a high percentage of patients with EU have bladder dysfunction. Most frequent alterations include high capacity bladders with incomplete emptying in around 50% of cases and urinary incontinence in about 10% [2,14]. There is no agreement on whether such ndings are due to pre-existing anatomical defects or the result of the surgical procedure. Since no urodynamic evaluation was performed pre-operatively in our patient diagnosed with detrusor± sphincter dis-coordination, we remain doubtful. Otherwise our results suggest that problems such as voiding dysfunction and urinary incontinence are not prevalent in these patients. Like others, we believe preserving bladder continence is not a major problem in most patients treated for EU [15]. 5. Conclusions Endoscopic puncture is an optimal choice in urgent settings, such as urosepsis, acute urinary retention and obstructive renal insuf ciency. Although seldom de nitive, it's a simple uneventful approach and thus probably useful in a staged approach, when an early attempt to preserve or improve ureterocele-bearing moiety function is desirable. Complete reconstruction of all EU's associated anomalies is considered the most de nitive approach. Its highly ef cient and it appears to be safe
J. Gomes et al. / European Urology 41 2002) 323±327 327 even in infants. Still it encloses potentially serious complications. We use it in complex cases and preferably around the age of 3 years. A simpli ed upper tract approach is curative in most patients without associated re ux. A signi cant minority of patients with low/ moderate grade VUR can still be managed by heminephrectomy with partial ureterectomy alone. This approach remains our rst choice in a large subset of patients with EU: those with non-viable or hypofunctional renal segments and without high-grade re ux. References [1] Schlussel RN, Retik AB. Anomalies of the ureter. In: Walsh PC, Retik AB, Vaughan ED, Wein AJ, editors. Campbell's Urology. vol. 2. 7a Ed. Philadelphia: Saunders, 1998. p. 1841±57. [2] Coplen DE, Barthold JS. Controversies in the management of ectopic ureteroceles. Urology 2000;56:665±8. [3] Shankar KR, Vishwanath N, Rickwood MK. Outcome of patients with pre-natally detected duplex system ureterocele. Natural history of those managed expectantly. J Urol 2001;165:1226± 8. [4] P ster C, Ravasse P, Barret E, Petit T, Mitrofanoff P. The value of endoscopic treatment for ureteroceles during the neonatal period. J Urol 1998;159 3):1006±9. [5] Cendron J, Bonhomme C. 31 Cas d'ureter abondement ectopique sons sphincterien chez l'enfant du sexe femenin. J Urol Nephrol 1968;74:1±4. [6] Hendren WH, Mitchell ME. Surgical treatment of ureteroceles. J Urol 1979;121:590±7. [7] Chertin B, Fridmans A, Hadas-Halpren I, Farkas A. Endoscopic puncture of ureterocele as minimally invasive and effective long-term procedure in children. Eur Urol 2001;39 3):332±6. [8] Shekarriz B, Updhayay J, Flemig P, Gonzalez R, Barthold JS. Longterm outcome based on the initial surgical approach to the ureterocele. J Urol 1999;162:1072±6. [9] de Jong TP, Dik P, Klijn AJ, Viterwaal CS, van Gool JD. Ectopic ureterocele: results of open surgical therapy in 40 patients. J Urol 2000;164:2040±3. [10] Decter RM, Sprunger JK, Holland RJ. Can a single individualized procedure predictably resolve all the problematic aspects of the pediatricureterocele? J Urol 2001;165:2308±10. [11] Glassberg KI, Braren V, Duckett JW, Jacobs EC, King LR, Lebowitz RL, Perlmutter AD, Stephens D. Report of the committee on terminology, nomenclature and classi cation, Section on Urology, American Academy of Paediatrics. J Urol 1984;132:1153±4. [12] Husmann D, Strand B, Ewalt D, Clement M, Kramer S, Allen T. Management of ectopic ureterocele associated with renal duplication: a comparison of partial nephrectomy and endoscopic decompression. J Urol 1999;162:1406±9. [13] Jayanthi VR, Koff SA. Long-term outcome of transurethral puncture of ectopic ureteroceles: initial success and late problems. J Urol 1999;162:1077±80. [14] Abrahamsson K, Hansson E, Sillen U, Hermansson G, Hjalmas K. Bladder dysfunction: an integral part of the ectopic ureterocele complex. J Urol 1998;160:1468±70. [15] Vereecken RL, Proesmans W. Extensive surgery on the trigone for complete ureteral duplication does not cause incontinence or voiding problems. Urology 2000;55 2):267±70.