Surgical treatment for ureterocele with special ... - Wiley Online Library

International Journal of Urology (2007) 14, 1063–1067

doi: 10.1111/j.1442-2042.2007.01903.x,

Original Article: Clinical Investigation

Surgical treatment for ureterocele with special reference to lower urinary tract reconstruction Kenji Shimada, Fumi Matsumoto and Futoshi Matsui Department of Urology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan

Objectives: We reviewed the results of surgical treatment for children with ureterocele, especially addressing the importance of the lower urinary tract reconstruction. Methods: We present medical records of 91 children with ureterocele (31 with the intravesical type, and 60 with the ectopic type) treated during the last 14 years. As the initial treatment, we carried out transurethral incision of the ureterocele (TUI). In patients with persistent reflux, breakthrough urinary tract infection (UTI), or signs of bladder outlet obstruction due to a collapsed cele wall, we recommended that patients undergo lower urinary tract reconstruction irrespective of the renal function involved in ureterocele. The average follow-up period was 5 years (ranging 1 year and 6 months to 14 years). Results: Transurethral incision of the ureterocele was carried out as the initial treatment on 68 patients (75%). A total of 34 patients (21 intravesical [68%] and 13 ectopic [22%]) were followed medically after TUI alone. Reconstruction of the lower urinary tract was carried out in 59 patients (65%). Nephroureterectomy combined with bladder level reconstruction was carried out in four children with single system and non-functioning kidney. Follow-up voiding cystourethrography showed that only one girl had reflux, which disappeared after the first follow-up examination. Postoperative UTI occurred in 12 patients (20%) with ectopic ureterocele. Voiding dysfunction was suspected in eight patients. Conclusion: We believe that the primary objective for patients with ureterocele, especially of the ectopic type, is to reconstruct the original pathology of the lower urinary tract that may give rise to reflux, obstruction, or abnormalities of urination. Although surgery at the bladder level can be challenging, the lower urinary tract reconstruction successfully corrects the vesicoureteral reflux and bladder outlet pathology under a cosmetically acceptable incision. Key words:

lower urinary tract reconstruction, surgical treatment, ureterocele.

Introduction A ureterocele is a cystic dilatation of the terminal ureter. Ureteroceles have a broad spectrum of clinical presentation, function of the affected renal moiety, and pathophysiology. Although the aims of treatment of this disorder are preservation of renal function, prevention of infection from obstruction or reflux, and maintenance of urinary continence, there is no single method of surgical repair sufficient to all cases. There have been increasing reports on the transurethral incision (TUI) of the cele wall with cold knife or puncture with a laser. About 70–80% of intravesical ureteroceles can successfully be treated with TUI alone, although one-third to one-fourth of this type require re-operation mainly as a result of persistent reflux.1 On the other hand , TUI of ectopic ureteroceles has resulted in a new creation of vesicoureteral reflux in about half or more of the patients, probably because of the poor backing at the bladder neck and trigone of the involved side.2 The open surgical approach of patients with ureterocele includes upper tract intervention, lower tract reconstruction, and a combination of the two.3 The upper tract approach consists of upper pole nephrectomy and partial ureterectomy in a duplex system, because the upper pole usually contributes little to the total renal function, or less commonly, to ureteropyelostomy. Although early reports of this approach showed attractive results with a re-operation rate of about 20% or less,4 the incidence for re-operation increased up to 50–65% in long-term reports.5,6 Lower urinary tract reconstruction, mainly for ectopic ureterocele, consists of excision of the ureterocele, reconstruction of the trigone and Correspondence: Kenji Shimada MD PhD, Department of Urology, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo-cho, Izumi, Osaka 594-1101, Japan. Email: [email protected]. osaka.jp Received 5 April 2007; accepted 16 August 2007.

© 2007 The Japanese Urological Association

bladder neck, which is needed mainly in ectopic ureterocele, and ureteral reimplantation with or without tapering under a cosmetically acceptable lower abdominal transverse incision. The most important pathophysiology to correct in this entity is the defect of the detrusor backing behind the ureterocele.7 If this defect is left untreated , weak anatomy at the ureterovesical junction would not resolve spontaneously and create ureteral reflux leading to high re-operation rates seen after the upper tract approach. We reviewed our results of surgical treatment for children with ureterocele, with particular emphasis on the importance of the lower urinary tract reconstruction.

Methods We retrospectively analyzed medical records of 91 children (32 males and 59 females) who underwent surgical treatment for ureterocele between August 1991 and December 2005. Clinical presentations consisted of febrile urinary tract infection (UTI) in 41 patients (45%), and prenatal detection of hydronephrosis in 34 (38%) (Table 1). Average age at diagnosis was 1 year 4 months (range 0 months to 11 years). Patients were evaluated by ultrasonography, voiding cystourethrography (VCUG), 99mTc-dimercaptosuccinic acid (DMSA) renal scintigraphy, and in some patients by intravenous urography, computed tomography (CT), or magnetic resonance imaging (MRI). Ureterocele was classified as intravesical and ectopic according to endoscopic findings.8 There were 31 patients with intravesical ureteroceles (24 with a single system, and seven with a duplex system), and 60 patients with ectopic ureteroceles (nine with a single system, and 51 with a duplex system) (Table 2). Patients who had small intravesical ureteroceles without pyelocalyectasis were followed non-surgically, and were excluded. Our principal management of ureterocele consists of initial endoscopic incision or puncture of the cele wall with either cold knife or 1063

K SHIMADA ET AL.

Table 1 Clinical presentations of patients who underwent transurethral incision of the ureterocele Clinical presentation

n (%)

Febrile UTI Cystitis Hematuria Prenatal diagnosis Postnatal ultrasound screening Others

41 (45%) 8 3 34 (40)% 3 3

}

Table 2

Intravesical type

Single system Duplex system

24 (30) 7 (7)

Single system Duplex system

9 (9) 51 (55)

31 pts (37 ureters) Ectopic type 60 pts (64 ureters)

Table 3 (TUI)

YAG-laser. In patients with single system upper tract and nonfunctioning kidney, nephrectomy together with bladder level reconstruction was carried out without TUI. After TUI, patients were followed while under prophylactic medications with regular check-ups of urinalysis and ultrasonography. Patients were evaluated by VCUG at 6-month to 1-year intervals to check changes of urination and reflux. In patients with persistent reflux, breakthrough UTI, or signs of bladder outlet obstruction due to a collapsed cele wall, we recommended that patients undergo lower urinary tract reconstruction irrespective of the renal function involved in ureterocele. In brief, lower urinary tract reconstruction in ectopic ureterocele begins with the circumcision of the cele wall, taking care of the contralateral ureteral orifice. In cases of a duplex system, the upper and the lower pole ureters are mobilized as a unit. The posterior mucosal layers of the ureterocele are then dissected away from the underlying thinned detrusor wall. This dissection is continued to the bladder neck or into the posterior urethra. The thinned-out bladder wall including bladder neck or even posterior urethra is repaired with interrupted sutures to provide muscular backing and adequate urinary continence. The ureters are re-implanted into the new orifice via the Cohen method. Tapering was required in dilated ureters involved in ureterocele. Common sheath re-implantation was carried out in patients with duplex systems. Follow-up periods ranged from 1 year and 6 months to 14 years (average 5 years). Children were followed with regular urine examinations and ultrasonography. VCUG was re-evaluated 6 months after surgery. Voiding habit was obtained from parents until the children reached 4–5 years of age. Good to fair renal function was defined as those possessing apparent radio-isotope (RI)-uptake on DMSA scintigram, while in nonfunctioning kidney or moiety no appreciable uptake was demonstrated.

Classification of ureterocele

Persistent reflux after transurethral incision of the ureterocele

Intravesical Ectopic

Single Duplex Single Duplex Total

(n)

TUI

No other Tx

VUR persist

24 7 9 51 91

19 6 3 40 68

17 (68)% 4 2 (22)% 11 34

2 1 1 3 7

}

}

Tx, treatment; VUR, vesicoureteral reflux.

Table 4 Radio-isotope (RI)-uptake of the kidney and/or renal moiety with ureterocele (n)

RI-uptake +



24 7 9 51 91

22 7 2 15 45

– 2 7 36 46

RI-uptake of the kidney More than 90% of the kidneys involved in intravesical ureterocele had function on scintigraphy, while more than 70% in ectopic ureterocele was non-functioning (Table 4).

Definitive reconstruction at the bladder level

Results TUI of ureterocele Transurethral incision of the ureterocele was carried out as the initial treatment on 68 patients (75%) (Table 3). Average age at TUI was 12 months (ranging from 0 months to 11 years). Twenty-five of 31 children with intravesical ureterocele (81%) and 43 of 60 children with ectopic ureterocele (72%) were treated by the endoscopic procedure. A total of 34 patients (21 intravesical [68%], and 13 ectopic [22%]) have been followed medically after TUI alone. Reflux persisted in seven patients. 1064

Reconstruction of the lower urinary tract was carried out in 59 patients (65%); 10 with intravesical ureterocele (32%), and 49 with ectopic ureterocele (82%) (Table 5). Nephroureterectomy in combination with bladder level reconstruction was carried out in four children with single system and non-functioning kidneys (two of intravesical ureterocele, and two of ectopic ureterocele). Among seven patients with intravesical single-system ureterocele who underwent bladder level surgery, five had everting ureterocele and two had non-functioning kidney. Three patients with intravesical double-system ureterocele had moderate to severe reflux to the mate ureter. Parents of one girl chose one-stage definitive repair, and in another two, persistent reflux was the indication for bladder level surgery. Average interval between TUI and definitive © 2007 The Japanese Urological Association

Surgical results of pediatric ureterocele

Table 5

Definitive reconstruction at the bladder level



(n)

Definitive repair

(N–Ux)

24 7 9 51 91

7 (32)% 3 9 (82)% 40 59 (65%)

}

(2)

}

(2) (4)

Definitive repair is cele wall resection and bladder neck trigonal reconstruction with or without ureterocystoneostomy. N–Ux, nephroureterectomy with bladder level reconstruction.

Table 6 Outcome reconstruction


of


vesicoureteral

reflux

after

definitive

(n)

DR

UCN

VUR persist

24 7 9 51 91

7 3 9 40 59

5 3 7 40 55

0 0 0 0 0

DR, definitive repair; UCN, ureterocystoneostomy; VUR, vesicoureteral reflux.

repair was 14 months (ranging 2 months to 3 years and 7 months). Ureteral tapering was carried out in 85% of the patients, mainly on the dilated ureter with ureterocele.

Outcome of vesicoureteral reflux Of 59 patients who underwent definitive reconstruction, ureterocystoneostomy was carried out in 55. Single system re-implantation was carried out in 12 patients, and the duplex system in 43 (Table 6). Eight patients underwent reimplantation of contralateral ureter either due to coexisting reflux or risks to impairment of ureterovesical junction at the time of definitive repair. Follow-up VCUG revealed persistent reflux in only one girl. Her reflux subsided spontaneously on repeated VCUG one year later. No patient has required subsequent upper tract surgery after definitive reconstruction.

Postoperative UTI Postoperative UTI occurred in 12 patients (20%) with ectopic ureterocele, nine with non-febrile, two with febrile, and one with both (Table 7). Febrile UTI was seen within 6 months after definitive reconstruction, and did not recur in each patient.

Voiding habit Voiding dysfunction was suspected in eight patients (Table 8). Among them, infrequent voiding was seen in six. Urodynamics of these patients showed underactive detrusor with normal sphincter mechanisms. Two of them had a history of perinatal urinary retention from infravesical obstruction by ureterocele. Urinary incontinence continued © 2007 The Japanese Urological Association

Table 7 Complications after definitive reconstruction for ureterocele: recurrence of UTI



(n)

Cystitis

Febrile UTI

24 7 9 51 91

0 0 3 7 10

0 0 1 2 3

UTI, urinary tract infection.

in four patients; one was mentally disabled , and the other patient was associated with anorectal malformations. Mild stress incontinence was seen in one girl with bilateral cecoureterocele. In the last girl, mild incontinence may have been attributed to overflow from infrequent voiding.

Discussion Although the treatment goals of ureterocele are preservation of renal function, prevention of infection from obstruction or reflux, and maintenance of urinary continence, there is no single method of surgical repair that is sufficient to all cases. Many published reports on the surgical management of this disorder have concurred that the initial treatment of TUI effectively decompresses the upper urinary tract and decreases the risk of infection. It is generally accepted that TUI is definitive in children with intravesical ureterocele. However, there was heterogeneity in regard to the re-operation rate after TUI in patients with intravesical ureterocele.9,10 Several studies have demonstrated that a quarter to approximately half of patients with this type required a secondary surgical procedure due to persistent high-grade reflux from wide hiatus or everting ureterocele. In our study, about one third of patients with intravesical ureterocele required bladder level surgery. We presume that intravesical ureterocele detected in young childhood is not as simple as is seen in adults, but has a broad spectrum of pathology and renal function. We recommend that patients undergo bladder level surgery if high-grade reflux persists after TUI, or if intravesical ureterocele is associated with evertion or a single-system non-functioning kidney. We believe that the primary purpose for patients with ureterocele, especially of the ectopic type, is to reconstruct the original pathology of the lower urinary tract that gives rise to reflux, obstruction, and abnormalities of urination.7 The morphological findings of ectopic ureterocele indicate that musculature at the bladder neck and posterior urethra is normal in anterior, but severely compromised in posterior quadrants.11 In cases of broad based ureteroceles, weakness of musculature extends cranially in the involved trigone and medially to the contralateral ureterovesical junction. A muscle defect may predispose to incontinence and , paradoxically, to outlet obstruction from protrusion of the bladder neck and posterior urethra similar to congenital bladder diverticulum in neonates or infants. The hypomuscularity of the trigone and the bladder outlet that backs the ectopic ureterocele should be repaired by adaptation of the normal detrusor muscle bordering. High re-operation rate following the upper tract approach has been considered to result from leaving these anatomical defects at the bladder level with persistent or delayed onset reflux or bladder outlet obstruction.6,12 1065

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Table 8

Complications after definitive reconstruction for ureterocele: voiding dysfunction



(n)

Dysfunctional voiding

(Incontinence)

24 7 9 51 91

0 0 4 4 8

(3) (1) (4)

Surgery at the bladder level on ectopic ureterocele can be technically challenging in that it consists of several procedures required by expert pediatric urologists. Management of the distal lip of the ureterocele and realignment of defective muscle at the bladder neck and deep in the posterior urethra differs in each case. In addition, tapering of the cele ureter is needed in most patients. Concerns about lower urinary tract reconstruction include persistent reflux or obstruction from re-implantation procedure, urinary incontinence from bladder outlet injury, and risks to leave hypofunctioning or dysplastic renal segments. Ureteral re-implantation via Cohen’s method is highly successful to stop reflux. Our institute has reported excellent results of ureterocystoneostomy in ureters with high grade reflux or megaureter.13 At the beginning of our practice we were concerned about leaving dilated ureters belonging to non-functioning or hypofunctioning renal segments in place, because ureteral musculature of ureterocele was reported to be dysmorphic and was not expected to sustain peristalsis.14 Our review, however, revealed good results with no persistent reflux or obstruction. The function of the renal moiety does not seem to affect the reliability for ureteral re-implantation. One of the demanding points of reconstruction is the removal of the posterior mucosal wall inside the distal ureterocele and the repair of thinned-out posterior bladder wall to provide adequate muscular backing. The complete mobilization of the distal lip of ureterocele is in some cases difficult because it extends deep in the urethra, and removal of the mucosa brings risks to injure sphincter mechanisms or even create urethrovaginal fistulas in female patients. In these instances we cut the distal edge of the ureterocele in order not to leave valvular structure, and fulgulate posterior mucosal epithelium instead of complete removal of the mucosal layer. High incidences of bladder dysfunction has been reported.15 Infrequent voiding, large capacities, and underactive detrusor on urodynamic study were commonplace.16 Bladder dysfunction was suspected in eight of the patients who underwent bladder level reconstruction (13%). Infrequent voiding was apparent in six patients. Two of them had a history of perinatal urinary retention from infravesical obstruction by ureterocele. Four patients were infrequent voiders for unknown reasons. Urodynamics of these patients showed underactive detrusors with normal sphincter mechanisms. It was not clear whether the bladder dysfunction seen in our patients was the consequence of bladder level surgery or an inherent component of this entity. We presume that surgical injury of pelvic plexus is not possible because extravesical manipulation is not needed in the bladder level reconstruction. In addition, they could retain urine on postoperative VCUG. Urinary incontinence continued in four patients, one of them was mentally disabled , and the other patient had a possible neurogenic bladder associated with anorectal malformations. Mild stress incontinence was seen in one girl with bilateral coecoureterocele. Weakness of her sphincter function could be congenital in origin. In addition to 1066

diminished muscularity at the bladder neck and proximal urethra, the muscle deficit at the intrinsic sphincter from bilateral coecoureterocele may predispose to urinary incontinence.11 Developmental abnormality of normal sphincter mechanism at or distal to the bladder neck is postulated to cause urinary incontinence in ureterocele. The hypomuscularity of the trigone, internal sphincter, and proximal urethra that backed the ureterocele should be repaired in bladder level reconstruction. As for the risks for leaving a hypofunctioning renal segment in place, we have been traditionally taught that such a kidney or renal segment would bring risks of intractable urinary tract infection if the urethra was re-implanted to the bladder. Postoperative febrile UTI, however, was seen in only 5% of patients after lower urinary tract reconstruction, although it was significantly higher than those with primary vesicoureteral reflux.17 From the results of our review, function of the kidney or renal segment appeared to affect little, if at all, both postoperative UTI or the success rate for re-implantation surgery. Although concerns have been considered about increasing risks of hypertension and malignancy when leaving behind non-functioning dysplastic renal segments, we are unaware of any reports where patients have developed these risks caused by a non-functioning moiety cases, including the present study.18 Reports of malignancy in the multicystic dysplastic kidney are limited in number, and to date, no evidence exists of increasing prevalence over that of the general population.19 Reviews of the current medical published reports on the risks associated with leaving dysplastic tissue in situ confirm that UTI, hypertension, and malignancy are rare occurrences for patients retaining duplicated renal segments.20 In conclusion, we believe that the primary purpose for patients with ureterocele is to reconstruct the original pathology of the lower urinary tract, which, if left untreated , gives rise to reflux, obstruction, and abnormalities of urination. Although surgery at the bladder level can be challenging, the procedure successfully corrects the vesicoureteral reflux and bladder outlet pathology under a cosmetically acceptable incision.

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Surgical results of pediatric ureterocele

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13 Matsumoto F, Shimada K, Harada Y, Naitoh Y. Split renal function does not change after successful treatment in children with primary vesico-ureteric reflux. BJU Int. 2003; 92: 1006–8. 14 Shimada K. A histological study of the dilated ureters in children. Jpn. J. Urol. 1983; 74: 61–75. 15 de Jong TP, Dik P, Klijn AJ, Uiterwaal CS, van Gool JD. Ectopic ureterocele: Results of open therapy in 40 patients. J. Urol. 2000; 164: 2040–43. 16 Abrahamsson K, Hansson E, Sillen U et al. Bladder dysfunction: An integral part of the ectopic ureterocele complex. J. Urol. 1998; 160: 1468–70. 17 Matsumoto F, Tohda A, Shimada K. Effect of ureteral reimplantation on prevention of urinary tract infection and renal growth in infants with primary vesicoureteral reflux. Int. J. Urol. 2004; 11: 1065–9. 18 Gran CD, Kropp BP, Cheng EY, Kropp KA. Primary lower urinary tract reconstruction for nonfunctioning renal moieties associated with obstructing uretercelec. J. Urol. 2005; 173: 198–201. 19 Wacksman J, Phipps L. Report of the Multicystic Kidney Registry: Preliminary findings. J. Urol. 1993; 150: 1870–72. 20 Husmann DA. Renal dysplasia: The risk and consequences of leaving dysplastic tissue in situ. Urology 1998; 52: 533–6.

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