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Etiology and outcome of the perineal repair of posterior and bulbar urethral strictures in children: A single surgeon experience
Journal of Pediatric Urology (2013) 9, 769e774
Etiology and outcome of the perineal repair of posterior and bulbar urethral strictures in children: A single surgeon experience Daniel Pfalzgraf a,*, Hendrik Isbarn a, Wolf-Hartmut Meyer-Moldenhauer b, Margit Fisch a, Silke Riechardt a a
University Hospital Hamburg-Eppendorf, Department of Urology, Martinistr. 52, 20246 Hamburg, Germany b Asklepios Clinic Hamburg-Harburg, Department of Urology, Eißendorfer Pferdeweg 52, 21075 Hamburg, Germany Received 22 May 2012; accepted 14 September 2012 Available online 13 October 2012
KEYWORDS Urethral stricture; Urethroplasty; Buccal mucosa; Pediatric urology
Abstract Objective: To evaluate the etiology of posterior and/or bulbar strictures in children in an industrialized country and assess the outcome of its repair by perineal approach. Urethral strictures in children are rare and often challenging to treat. Trauma is the main etiology in developing countries. However, data for industrialized countries are sparse. Material and methods: Retrospective analysis of 17 patients treated with perineal urethroplasty 2001e2010. Data were assessed by chart review and non-validated standardized questionnaire. Hypospadias cases were excluded. Reconstruction was performed by stricture excision and primary anastomosis, or a single-staged or two-staged buccal mucosa graft. Results: Mean age at surgery was 7.9 years (range 1e13) and mean follow-up was 42.6 months (4e115). Eight patients (47.1%) had post-traumatic strictures, five (29.4%) had a history of posterior valves, and previous transurethral catheterization and irradiation each accounted for one patient (5.9%). In the remaining two (11.8%), the etiology was unknown. The success rate was 88.9%. All but one patient were continent postoperatively. Conclusions: Most common etiology for open urethral reconstruction in children was trauma and previous valve treatment. In our hands the perineal approach for stricture repair is safe
* Corresponding author. Tel.: þ49 621 43626749; fax: þ49 621 2184. E-mail addresses: [email protected] (D. Pfalzgraf), [email protected] (H. Isbarn), [email protected] (W.-H. Meyer-Moldenhauer), [email protected] (M. Fisch), [email protected] (S. Riechardt). 1477-5131/$36 ª 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.jpurol.2012.09.007
770
D. Pfalzgraf et al. and successful. Stricture recurrence rate is low, and incontinence is only associated with additional bladder neck trauma. ª 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction Several causes for the development of proximal urethral strictures have been described, including post-traumatic, post-inflammatory, and iatrogenic circumstances [1]. In children, however, proximal urethral strictures are a rare entity, and there have been only a few series with a significant number of patients treated by open reconstruction. The number of patients ranges from 2 to 75 [2e4]. Trauma is the main etiology in developing countries and the transpubic approach is often used for repair [5]. However, there are limited data for industrial countries, with most published series reporting on small numbers of patients. Clinical signs for urethral stricture in children can be incontinence or bed-time wetting, as well as common symptoms like difficulties at micturition, weak urine flow and recurrent urinary tract infection. Pelvic fracture posterior distraction defects frequently lead to obliterative strictures and cause acute urinary retention. Therapeutic options are similar to those in adults, ranging from endoscopic treatment for short strictures [6] to open reconstruction in long strictures or failed endoscopic treatment. In open reconstruction, access is gained via the perineum [7] or in a combined perineal and transpubic approach [8]. In short strictures, excision and primary anastomosis (EPA) with en bloc resection of scar tissue can be performed [6]; longer strictures can be treated using scrotal flaps [3,9], appendix [2], tubularized penile skin [6] or buccal mucosa [10]. However, the delicateness of the pediatric urethral tissue as well as the small anatomy poses a considerable surgical challenge. The aim of this study was to evaluate the etiology of posterior (post-traumatic) and anterior bulbar strictures in children in an industrialized country and to assess the intermediate-term outcome of a repair by perineal approach in a European tertiary centre in a single-surgeon series. Anterior penile strictures e mainly due to complications of hypospadias repair e were excluded from this series.
hypospadias were excluded. Stricture etiology, previous surgeries, the postoperative recurrence rates, complications and postoperative incontinence were assessed.
Preoperative diagnostics Preoperative workup included routine uroflowmetry and a combined voiding cystourethrogram (VCUG) and retrograde urethrogram. If the length of the stricture remained unclear, a retrograde and rarely an antegrade cystoscopy through a suprapubic tube site (under anesthesia) was additionally performed.
Surgical technique Urethral reconstruction was performed by stricture excision and primary anastomosis (EPA), or single-staged or twostaged buccal mucosa graft (BMG). A posterior post-traumatic stricture and a bulbar stricture of 1 cm were treated by end-to-end anastomosis
Material and methods We relied on the data of 17 patients with posterior (posttraumatic) or bulbar urethral strictures who were treated with open perineal repair at our institution between 2001 and 2010. All surgeries were performed by a single surgeon (MF), no other pediatric urethroplasties were performed within the indicated time, and direct visual internal urethrotomy (DVUI) was performed for posterior urethral valves (PUV) but not for urethral strictures. A retrospective analysis of the patients was conducted by means of chart review and a non-validated standardized questionnaire. This questionnaire was mailed to the patients at time of follow-up. Children with meatal stenosis or a history of
Figure 1 Inspecting the proximal urethral using a children’s nose speculum.
Etiology and outcome of the perineal repair whereas bulbar strictures of >1 cm were treated by buccal mucosa onlay or e if severe spongiofibrosis or scarring was present e by two-staged buccal mucosa inlay. All children were treated via a perineal approach. In short strictures, the scar tissue was removed and an end-to-end anastomosis was performed. In longer strictures the stricture was passed with a small probe and opened along this guiding line until healthy tissue was reached. Subsequently, the proximal urethral margin was inspected using a children’s nose speculum (Fig. 1) and the three posterior sutures for the buccal mucosa onlay urethroplasty were placed. The stricture length was then measured and buccal mucosa of the required length was harvested from the lower lip, and if more tissue was needed from the inner cheek as well. Next, the buccal mucosa was sutured into the opened urethra under vision. Special attention was paid to prepare a well-vascularized bed for the graft. In singlestaged reconstruction this was the corpus spongiosum; in the two-staged procedures, the graft bed was prepared from the surrounding subcutaneous tissue. Once the buccal mucosa was fixed to the proximal urethra, in the singlestaged reconstructions the urethra was closed and a transurethral catheter inserted (Fig. 2). In the two-staged reconstructions the buccal mucosa was placed in the defect after resection of the scar tissue and fixed with several sutures. Finally a compression dressing was placed and remained in situ for 10 days. No further measures were required to immobilize the graft on the graft bed.
771 All children received a transurethral as well as a suprapubic catheter intraoperatively. After end-to-end anastomosis, the transurethral catheter was removed 7 days after surgery and healing and success were verified by a VCUG. After reconstruction with buccal mucosa, the transurethral catheter was left in place for 10 days postoperatively. In buccal mucosa onlay, patients kept their suprapubic catheter for 21 days after surgery. Subsequently, healing and success were verified by VCUG as well. In the two-staged procedures (buccal mucosa inlay), three months after the initial surgery, the second stage was performed tubularizing the buccal mucosa over a transurethral catheter. After seven days, a VCUG was performed and micturition was allowed after catheter removal, if no extravasation was present. The suprapubic catheter was removed, if voiding without residual urine was possible.
Postoperative routine follow-up Assessment of the surgical results at stricture repair was obtained by measuring uroflow and residual urine, and by inquiring for straining or recurrent urinary tract infections. If any of these factors were found, another VCUG was performed. In the absence of such circumstances, and if no further intervention was required, the repair was considered successful. The follow-up was obtained at our institution at three months after surgery and e due to widespread living of the patients e by the attending urologist at the child’s home thereafter. Follow-up for this study was obtained by a non-validated standardized questionnaire. In this study, the principles outlined in the Declaration of Helsinki were followed.
Results
Figure 2 Once the buccal mucosa is fixed to the proximal urethra, in the single-staged reconstructions the urethra is closed and a transurethral catheter inserted.
Median age at surgery was 9 years (range 1e13) and the median follow-up was 30 months (4e115). Stricture etiology could not be assessed in all patients. Eight patients (47.1%) had post-traumatic strictures, five (29.4%) had a history of posterior valves, and previous transurethral catheterization and irradiation accounted for one patient (5.9%) each. In the remaining two patients (11.8%), the reason for the stricture formation was unknown. Table 1 shows patient baseline characteristics and surgical procedures. Mean stricture length was 2.5 cm (range 0.5e4 cm). Three children presented with complete obliteration of the urethra. Two of these were after treatment of PUV, of whom one had undergone a failed endoscopic treatment and another a failed endoscopic as well as open treatment. The third child had a history of urethral dislocation after complex pelvic fracture. Four patients (23.5%) had undergone previous transurethral surgeries for stricture treatment and three patients (17.6%) had undergone open reconstructions before (Table 2). All but one patient were continent postoperatively. The one individual who was not continent had presented with
772 Table 1
D. Pfalzgraf et al. Patient baseline characteristics and surgical procedures. Bulbar n (%)
No. pts. Median age (range) Stricture etiology Trauma History of PUV Catheter Irradiation Unknown Previous DVUI Previous urethroplasties Technique of reconstruction EPA BMG (single-staged) BMG (two-staged) Median mos. follow-up (range)
13 (76.5) 9 (1e13) 4 5 1 1 2 3 2
(30.8) (38.5) (7.7) (7.7) (15.4) (23.1) (15.4)
5 6 2 28
(38.5) (46.2) (15.4) (4e91)
Membranous n (%)
Penile n (%)
Totals n (%)
3 (17.6) 9 (8e11)
1 (5.9) 10
17 (100) 9 (1e13)
3 0 0 0 0 0 1
(100) (0.0) (0.0) (0.0) (0.0) (0.0) (33.3)
1 0 0 0 0 1 0
(100) (0.0) (0.0) (0.0) (0.0) (100) (0.0)
8 5 1 1 2 4 3
(100) (0.0) (0.0) (36e115)
0 (0.0) 1 (100) 0 (0.0) 30
8 7 2 30
3 0 0 115
a severe traumatic posterior urethral disruption with additional bladder neck trauma. Eight children (47.1%) were treated by excision and primary anastomosis (EPA), and in seven children a singlestaged buccal mucosa urethroplasty was performed. In two children with complete urethral stricture an open twostaged reconstruction with buccal mucosa was required. During follow-up, two stricture recurrences occurred, resulting in an overall stricture recurrence-free survival rate of 88.9%. One of the recurrences occurred in a child
Table 2 Outcome of urethroplasty, previous treatment and technique of reconstruction stratified according to stricture localization. Success rate Bulbar (n)
Membranous (n)
Penile (n)
Median age (range) Stricture etiology Trauma
9 (1e13)
9 (8e11)
10
75% (3/4)
History of PUV Catheter Irradiation Unknown Previous DVUI Previous urethroplasties Technique of reconstruction EPA BMG (single-staged) BMG (two-staged) Median mos. follow-up
80% (4/5) 100% (1/1) 100% (1/1) 100% (2/2) 3 2
100% (3/3) e e e e e 1
100% (1/1) e e e e 1 e
5 6
3 0
0 1
2
0
0
28 (4e91)
115 (36e115)
30
(47.1) (29.4) (5.9) (5.9) (11.8) (23.5) (17.6) (47.1) (41.2) (11.8) (4e115)
with previous traumatic urethral disruption and one in a child with a history of PUV (Table 3). As postoperative complications, an extravasation with the need for prolonged suprapubic diversion was noted in two cases (11.7%). Both eventually subsided without further problems and the suprapubic tube was removed 2 weeks after the first VCUG. One urinary tract infection occurred. Incontinence was seen in one child with additional bladder neck trauma. Once micturition was allowed, all children had a urinary flow >15 ml/s with no residual urine.
Discussion Different etiologies for development of urethral strictures have been described. Apart from hypospadias and meatal stenosis, trauma and transurethral manipulation are most often responsible for stricture occurrence. If hypospadias and meatal stenosis are not considered, trauma is identified as the main cause for pediatric urethral stricture in most reported series. In the current study also, most strictures were posttraumatic (47.1%). However a history of prior urethral valve treatment was found in 29.4%. This number is higher then expected, considering that, of the more recent publications, only Diamond et al. [6] reported on this phenomenon in 9% of stricture cases and Rourke et al. [10] described 12% of their pediatric patients with stricture as having a history of PUV. The cause for this remains unclear; however, with a small sample size, single patients influence the percentages considerably and cannot be generalized as a shift in etiology. Over the last years, there has been a shift towards valve treatment at an early age, with instruments getting smaller [11]. Accordingly, in 2009 Sudarsanan et al. reported a mean age of 45 days at urethral valve treatment [12], while Lal et al. in 1998 reported urinary diversion until the age of 9e12 months, if the urethral caliber was too small for a 9 Fr resectoscope [13]. The other change in valve treatment in recent years is the more liberal use of lasers instead of cold-knife incision. Whether any of these
Etiology and outcome of the perineal repair
773
Table 3 etiology.
Surgical
Stricture etiology
Success Technique of reconstruction rate n (success rate)
outcomes
EPA Trauma
87.5% (7/8) History 80.0% of PUV (4/5) Catheter 100% (1/1) Irradiation 100% (1/1) Unknown 100% (2/2)
stratified
according
to
BMG BMG (single-staged) (two-staged)
6 1 (100%) (100%) 2 2 (50%) (100%) e 1 (100%)
1 (0%)
e
1 (100%)
e
e
2 (100%)
e
1 (100%) e
factors influence stricture formation after valve treatment remains unclear and will need further investigation. In the case of a suspected stricture, Garibay et al. found that uroflow examination is an important non-invasive tool to evaluate the functional results of urethroplasty in children and detect asymptomatic strictures [14]. In suspicious uroflow, further diagnostic measures should always include radiologic or endoscopic assessment. At this time, we feel that our approach of performing a VCUG 3 weeks postoperatively to assess for good wound healing and to rule out early failure of stricture repair, followed by conservative measures, is the likeliest follow-up to be conducted in everyday life. These conservative measures consisted of uroflow, assessment of residual urine, and inquiry as to whether straining or recurrent urinary tract infections were observed after surgery. Regardless of etiology, open reconstruction for proximal strictures in children following the same algorithms as in adults leads to excellent results, comparable to larger series for adult urethral reconstruction. Some authors report the need for a transpubic access to complex posterior urethral strictures to achieve a successful repair [15,16]. However, as all patients in this series were treated via a perineal access with good results and Rourke et al. [10] report a success rate of 94.4% with a perineal approach as well, the need for transpubic surgery needs to be reconsidered. Usually even longer strictures can be managed via this incision, and a pubectomy may only be required in selected cases with complex post-traumatic strictures if a tension-free anastomosis via the perineal approach cannot be achieved. Again, as in adult urethral reconstruction, we prefer EPA and single- or two-staged buccal mucosa reconstruction over other techniques. The timing of the second stage procedure for tubularization at 3 months after the initial procedure is the same as in adults; indeed we find this a suitable timing in children as well due to the possibly more rapid healing of pediatric tissue. Poor results have been reported for transurethral stricture treatment in children, with success rates of 42% for dilation with guide wire [17] and 33e50% for DVUI [18,19]. However, some techniques of open reconstruction have shown unfavorable results as well. While for
augmentation with tube graft a success rate of 33% was reported [5], Koraitim [3] found a success rate of 46% with scrotal inlay urethroplasty. In the present study, the success rate with EPA was 100% and for single-staged buccal mucosa urethroplasty it was 85.7%. For twostaged procedures, the success rate in this group was only 50%, as one of the two performed staged procedures failed. However, due to the very small number of patients in this group, a larger series is required to help determine the success rate for two-staged urethroplasty in children. Surgical outcomes stratified according to etiology shows that for the post-traumatic strictures, reconstruction could mostly be achieved by EPA with excellent results, while a single- and a two-staged BMG was required in one case each. While in the case of single-staged reconstruction the patient remained recurrence free, the two-staged urethroplasty failed. Though no single reason for stricture recurrence could be established in this patient, local tissue conditions after traumatic disruption are suspected to have influenced the outcome. Special attention needs to be paid to anatomical differences in children as opposed to adults in urethral reconstruction. Due to the small urethral caliber and tissue delicacy, pediatric stricture repair poses a considerable challenge to the surgeon. The urethra is less elastic and, together with the small size of the urethra, we believe that in children an EPA should not be performed in strictures of more than 1 cm. Moreover, the risk of erectile dysfunction has to be accounted for in particular, and surgery with magnifying glasses is mandatory. The true risk for the development of erectile dysfunction in children will require further investigation, as patient numbers in studies are still relatively small and mostly the follow-up is not long enough (i.e. children are still too young) to allow for reliable adjustment of erectile function. Furthermore, covering the reconstructed part of the urethra with the fragile, still developing corpus spongiosum requires careful dissection in the beginning and adaption at the end of surgery. However, considering the poor results of transurethral stricture treatment in children [18,19], open reconstruction should be discussed in selected cases even in small children. The reason to perform reconstructive surgery on a child as young as 1 year in the bulbar stricture group was a complete obliteration of the bulbar urethra, requiring either open reconstruction or suprapubic cystostomy. In this child, the operative result was favorable with no stricture recurrence on follow-up. The two stricture recurrences occurred in a child with previous traumatic urethral disruption and in one with a history of PUV. The reasons for stricture recurrence are difficult to establish: while in the case of traumatic disruption the local tissue conditions and impaired microvascularization might be suspected to have influenced the outcome, a single reason for stricture recurrence could not be established for either of these patients. One of the limitations of this study is the shorter follow-up in some cases, somewhat limiting the informative value of the stricture recurrence rates. However, with a median follow-up of 30 months and a range of 4e115 months, most patients are surely to be counted as long-term follow-up. Another is the retrospective nature of the study.
774
Conclusion The most common etiology for open urethral reconstruction in children was trauma and previous valve treatment. In our hands, the perineal approach for stricture repair was safe and successful. The stricture recurrence rate was low, and incontinence only associated with additional bladder neck trauma. Accordingly, open reconstruction even in small children should be considered feasible and does not have to be postponed until later in life.
Conflict of interest None.
Funding None.
References [1] Lumen N, Hoebeke P, Willemsen P, De Troyer B, Pieters R, Oosterlinck W. Etiology of urethral stricture disease in the 21st century. J Urol 2009;182:983e7. [2] Aggarwal SK, Goel D, Gupta CR, Ghosh S, Ojha H. The use of pedicled appendix graft for substitution of urethra in recurrent urethral stricture. J Pediatr Surg 2002;37:246e50. [3] Koraitim MM. Posttraumatic posterior urethral strictures in children: a 20-year experience. J Urol 1997;157:641e5. [4] Sunay M, Karabulut A, Dadali M, Bagbanci S, Emir L, Erol D. Single-institution outcomes of open reconstruction techniques for management of pediatric and adolescent post-traumatic urethral strictures. Urology 77:706e10. [5] Orabi S, Badawy H, Saad A, Youssef M, Hanno A. Post-traumatic posterior urethral stricture in children: how to achieve a successful repair. J Pediatr Urol 2008;4:290e4. [6] Diamond DA, Xuewu J, Bauer SB, Cilento Jr BG, Borer JG, Nguyen H, et al. What is the optimal surgical strategy for bulbous urethral stricture in boys? J Urol 2009;182:1755e8.
D. Pfalzgraf et al. [7] Shenfeld OZ, Gdor J, Katz R, Gofrit ON, Pode D, Landau EH. Urethroplasty, by perineal approach, for bulbar and membranous urethral strictures in children and adolescents. Urology 2008;71:430e3. discussion 3e4. [8] Podesta ML. Use of the perineal and perineal-abdominal (transpubic) approach for delayed management of pelvic fracture urethral obliterative strictures in children: long-term outcome. J Urol 1998;160:160e4. [9] Quentel P, Beurton D, Baron JC, Cukier J. Urethral stricture in children. Long term results of surgical treatments. Ann Pediatr (Paris) 1990;37:387e9. [10] Rourke KF, McCammon KA, Sumfest JM, Jordan GH, Kaplan GW. Open reconstruction of pediatric and adolescent urethral strictures: long-term followup. J Urol 2003;169:1818e21. discussion 21. [11] Chatterjee SK, Banerjee S, Basak D, Basu AK, Chakravarti AK, Chatterjee US, et al. Posterior urethral valves: the scenario in a developing center. Pediatr Surg Int 2001;17:2e7. [12] Sudarsanan B, Nasir AA, Puzhankara R, Kedari PM, Unnithan GR, Damisetti KR. Posterior urethral valves: a single center experience over 7 years. Pediatr Surg Int 2009;25:283e7. [13] Lal R, Bhatnagar V, Mitra DK. Urethral strictures after fulguration of posterior urethral valves. J Pediatr Surg 1998;33:518e9. [14] Garibay JT, Reid C, Gonzalez R. Functional evaluation of the results of hypospadias surgery with uroflowmetry. J Urol 1995; 154:835e6. [15] Das K, Charles AR, Alladi A, Rao S, D’Cruz AJ. Traumatic posterior urethral disruptions in boys: experience with the perineal/perineal-transpubic approach in ten cases. Pediatr Surg Int 2004;20:449e54. [16] Gupta NP, Mishra S, Dogra PN, Hemal AK, Seth A, Kumar R. Outcome of end-to-end urethroplasty: single-center experience. Urol Int 2009;82:179e82. [17] Chiang DT, A Dewan P. Guide wire-assisted urethral dilation in pediatric urology: experience of a single surgeon. Urol J 2007; 4:226e9. [18] Hosseini SY, Safarinejad MR. Early versus delayed internal urethrotomy for recurrent urethral stricture after urethroplasty in children. Urol J 2005;2:165e8. [19] Hsiao KC, Baez-Trinidad L, Lendvay T, Smith EA, Broecker B, Scherz H, et al. Direct vision internal urethrotomy for the treatment of pediatric urethral strictures: analysis of 50 patients. J Urol 2003;170:952e5.
Etiology and outcome of the perineal repair of posterior and bulbar urethral strictures in children: A single surgeon experience Daniel Pfalzgraf a,*, Hendrik Isbarn a, Wolf-Hartmut Meyer-Moldenhauer b, Margit Fisch a, Silke Riechardt a a
University Hospital Hamburg-Eppendorf, Department of Urology, Martinistr. 52, 20246 Hamburg, Germany b Asklepios Clinic Hamburg-Harburg, Department of Urology, Eißendorfer Pferdeweg 52, 21075 Hamburg, Germany Received 22 May 2012; accepted 14 September 2012 Available online 13 October 2012
KEYWORDS Urethral stricture; Urethroplasty; Buccal mucosa; Pediatric urology
Abstract Objective: To evaluate the etiology of posterior and/or bulbar strictures in children in an industrialized country and assess the outcome of its repair by perineal approach. Urethral strictures in children are rare and often challenging to treat. Trauma is the main etiology in developing countries. However, data for industrialized countries are sparse. Material and methods: Retrospective analysis of 17 patients treated with perineal urethroplasty 2001e2010. Data were assessed by chart review and non-validated standardized questionnaire. Hypospadias cases were excluded. Reconstruction was performed by stricture excision and primary anastomosis, or a single-staged or two-staged buccal mucosa graft. Results: Mean age at surgery was 7.9 years (range 1e13) and mean follow-up was 42.6 months (4e115). Eight patients (47.1%) had post-traumatic strictures, five (29.4%) had a history of posterior valves, and previous transurethral catheterization and irradiation each accounted for one patient (5.9%). In the remaining two (11.8%), the etiology was unknown. The success rate was 88.9%. All but one patient were continent postoperatively. Conclusions: Most common etiology for open urethral reconstruction in children was trauma and previous valve treatment. In our hands the perineal approach for stricture repair is safe
* Corresponding author. Tel.: þ49 621 43626749; fax: þ49 621 2184. E-mail addresses: [email protected] (D. Pfalzgraf), [email protected] (H. Isbarn), [email protected] (W.-H. Meyer-Moldenhauer), [email protected] (M. Fisch), [email protected] (S. Riechardt). 1477-5131/$36 ª 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.jpurol.2012.09.007
770
D. Pfalzgraf et al. and successful. Stricture recurrence rate is low, and incontinence is only associated with additional bladder neck trauma. ª 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction Several causes for the development of proximal urethral strictures have been described, including post-traumatic, post-inflammatory, and iatrogenic circumstances [1]. In children, however, proximal urethral strictures are a rare entity, and there have been only a few series with a significant number of patients treated by open reconstruction. The number of patients ranges from 2 to 75 [2e4]. Trauma is the main etiology in developing countries and the transpubic approach is often used for repair [5]. However, there are limited data for industrial countries, with most published series reporting on small numbers of patients. Clinical signs for urethral stricture in children can be incontinence or bed-time wetting, as well as common symptoms like difficulties at micturition, weak urine flow and recurrent urinary tract infection. Pelvic fracture posterior distraction defects frequently lead to obliterative strictures and cause acute urinary retention. Therapeutic options are similar to those in adults, ranging from endoscopic treatment for short strictures [6] to open reconstruction in long strictures or failed endoscopic treatment. In open reconstruction, access is gained via the perineum [7] or in a combined perineal and transpubic approach [8]. In short strictures, excision and primary anastomosis (EPA) with en bloc resection of scar tissue can be performed [6]; longer strictures can be treated using scrotal flaps [3,9], appendix [2], tubularized penile skin [6] or buccal mucosa [10]. However, the delicateness of the pediatric urethral tissue as well as the small anatomy poses a considerable surgical challenge. The aim of this study was to evaluate the etiology of posterior (post-traumatic) and anterior bulbar strictures in children in an industrialized country and to assess the intermediate-term outcome of a repair by perineal approach in a European tertiary centre in a single-surgeon series. Anterior penile strictures e mainly due to complications of hypospadias repair e were excluded from this series.
hypospadias were excluded. Stricture etiology, previous surgeries, the postoperative recurrence rates, complications and postoperative incontinence were assessed.
Preoperative diagnostics Preoperative workup included routine uroflowmetry and a combined voiding cystourethrogram (VCUG) and retrograde urethrogram. If the length of the stricture remained unclear, a retrograde and rarely an antegrade cystoscopy through a suprapubic tube site (under anesthesia) was additionally performed.
Surgical technique Urethral reconstruction was performed by stricture excision and primary anastomosis (EPA), or single-staged or twostaged buccal mucosa graft (BMG). A posterior post-traumatic stricture and a bulbar stricture of 1 cm were treated by end-to-end anastomosis
Material and methods We relied on the data of 17 patients with posterior (posttraumatic) or bulbar urethral strictures who were treated with open perineal repair at our institution between 2001 and 2010. All surgeries were performed by a single surgeon (MF), no other pediatric urethroplasties were performed within the indicated time, and direct visual internal urethrotomy (DVUI) was performed for posterior urethral valves (PUV) but not for urethral strictures. A retrospective analysis of the patients was conducted by means of chart review and a non-validated standardized questionnaire. This questionnaire was mailed to the patients at time of follow-up. Children with meatal stenosis or a history of
Figure 1 Inspecting the proximal urethral using a children’s nose speculum.
Etiology and outcome of the perineal repair whereas bulbar strictures of >1 cm were treated by buccal mucosa onlay or e if severe spongiofibrosis or scarring was present e by two-staged buccal mucosa inlay. All children were treated via a perineal approach. In short strictures, the scar tissue was removed and an end-to-end anastomosis was performed. In longer strictures the stricture was passed with a small probe and opened along this guiding line until healthy tissue was reached. Subsequently, the proximal urethral margin was inspected using a children’s nose speculum (Fig. 1) and the three posterior sutures for the buccal mucosa onlay urethroplasty were placed. The stricture length was then measured and buccal mucosa of the required length was harvested from the lower lip, and if more tissue was needed from the inner cheek as well. Next, the buccal mucosa was sutured into the opened urethra under vision. Special attention was paid to prepare a well-vascularized bed for the graft. In singlestaged reconstruction this was the corpus spongiosum; in the two-staged procedures, the graft bed was prepared from the surrounding subcutaneous tissue. Once the buccal mucosa was fixed to the proximal urethra, in the singlestaged reconstructions the urethra was closed and a transurethral catheter inserted (Fig. 2). In the two-staged reconstructions the buccal mucosa was placed in the defect after resection of the scar tissue and fixed with several sutures. Finally a compression dressing was placed and remained in situ for 10 days. No further measures were required to immobilize the graft on the graft bed.
771 All children received a transurethral as well as a suprapubic catheter intraoperatively. After end-to-end anastomosis, the transurethral catheter was removed 7 days after surgery and healing and success were verified by a VCUG. After reconstruction with buccal mucosa, the transurethral catheter was left in place for 10 days postoperatively. In buccal mucosa onlay, patients kept their suprapubic catheter for 21 days after surgery. Subsequently, healing and success were verified by VCUG as well. In the two-staged procedures (buccal mucosa inlay), three months after the initial surgery, the second stage was performed tubularizing the buccal mucosa over a transurethral catheter. After seven days, a VCUG was performed and micturition was allowed after catheter removal, if no extravasation was present. The suprapubic catheter was removed, if voiding without residual urine was possible.
Postoperative routine follow-up Assessment of the surgical results at stricture repair was obtained by measuring uroflow and residual urine, and by inquiring for straining or recurrent urinary tract infections. If any of these factors were found, another VCUG was performed. In the absence of such circumstances, and if no further intervention was required, the repair was considered successful. The follow-up was obtained at our institution at three months after surgery and e due to widespread living of the patients e by the attending urologist at the child’s home thereafter. Follow-up for this study was obtained by a non-validated standardized questionnaire. In this study, the principles outlined in the Declaration of Helsinki were followed.
Results
Figure 2 Once the buccal mucosa is fixed to the proximal urethra, in the single-staged reconstructions the urethra is closed and a transurethral catheter inserted.
Median age at surgery was 9 years (range 1e13) and the median follow-up was 30 months (4e115). Stricture etiology could not be assessed in all patients. Eight patients (47.1%) had post-traumatic strictures, five (29.4%) had a history of posterior valves, and previous transurethral catheterization and irradiation accounted for one patient (5.9%) each. In the remaining two patients (11.8%), the reason for the stricture formation was unknown. Table 1 shows patient baseline characteristics and surgical procedures. Mean stricture length was 2.5 cm (range 0.5e4 cm). Three children presented with complete obliteration of the urethra. Two of these were after treatment of PUV, of whom one had undergone a failed endoscopic treatment and another a failed endoscopic as well as open treatment. The third child had a history of urethral dislocation after complex pelvic fracture. Four patients (23.5%) had undergone previous transurethral surgeries for stricture treatment and three patients (17.6%) had undergone open reconstructions before (Table 2). All but one patient were continent postoperatively. The one individual who was not continent had presented with
772 Table 1
D. Pfalzgraf et al. Patient baseline characteristics and surgical procedures. Bulbar n (%)
No. pts. Median age (range) Stricture etiology Trauma History of PUV Catheter Irradiation Unknown Previous DVUI Previous urethroplasties Technique of reconstruction EPA BMG (single-staged) BMG (two-staged) Median mos. follow-up (range)
13 (76.5) 9 (1e13) 4 5 1 1 2 3 2
(30.8) (38.5) (7.7) (7.7) (15.4) (23.1) (15.4)
5 6 2 28
(38.5) (46.2) (15.4) (4e91)
Membranous n (%)
Penile n (%)
Totals n (%)
3 (17.6) 9 (8e11)
1 (5.9) 10
17 (100) 9 (1e13)
3 0 0 0 0 0 1
(100) (0.0) (0.0) (0.0) (0.0) (0.0) (33.3)
1 0 0 0 0 1 0
(100) (0.0) (0.0) (0.0) (0.0) (100) (0.0)
8 5 1 1 2 4 3
(100) (0.0) (0.0) (36e115)
0 (0.0) 1 (100) 0 (0.0) 30
8 7 2 30
3 0 0 115
a severe traumatic posterior urethral disruption with additional bladder neck trauma. Eight children (47.1%) were treated by excision and primary anastomosis (EPA), and in seven children a singlestaged buccal mucosa urethroplasty was performed. In two children with complete urethral stricture an open twostaged reconstruction with buccal mucosa was required. During follow-up, two stricture recurrences occurred, resulting in an overall stricture recurrence-free survival rate of 88.9%. One of the recurrences occurred in a child
Table 2 Outcome of urethroplasty, previous treatment and technique of reconstruction stratified according to stricture localization. Success rate Bulbar (n)
Membranous (n)
Penile (n)
Median age (range) Stricture etiology Trauma
9 (1e13)
9 (8e11)
10
75% (3/4)
History of PUV Catheter Irradiation Unknown Previous DVUI Previous urethroplasties Technique of reconstruction EPA BMG (single-staged) BMG (two-staged) Median mos. follow-up
80% (4/5) 100% (1/1) 100% (1/1) 100% (2/2) 3 2
100% (3/3) e e e e e 1
100% (1/1) e e e e 1 e
5 6
3 0
0 1
2
0
0
28 (4e91)
115 (36e115)
30
(47.1) (29.4) (5.9) (5.9) (11.8) (23.5) (17.6) (47.1) (41.2) (11.8) (4e115)
with previous traumatic urethral disruption and one in a child with a history of PUV (Table 3). As postoperative complications, an extravasation with the need for prolonged suprapubic diversion was noted in two cases (11.7%). Both eventually subsided without further problems and the suprapubic tube was removed 2 weeks after the first VCUG. One urinary tract infection occurred. Incontinence was seen in one child with additional bladder neck trauma. Once micturition was allowed, all children had a urinary flow >15 ml/s with no residual urine.
Discussion Different etiologies for development of urethral strictures have been described. Apart from hypospadias and meatal stenosis, trauma and transurethral manipulation are most often responsible for stricture occurrence. If hypospadias and meatal stenosis are not considered, trauma is identified as the main cause for pediatric urethral stricture in most reported series. In the current study also, most strictures were posttraumatic (47.1%). However a history of prior urethral valve treatment was found in 29.4%. This number is higher then expected, considering that, of the more recent publications, only Diamond et al. [6] reported on this phenomenon in 9% of stricture cases and Rourke et al. [10] described 12% of their pediatric patients with stricture as having a history of PUV. The cause for this remains unclear; however, with a small sample size, single patients influence the percentages considerably and cannot be generalized as a shift in etiology. Over the last years, there has been a shift towards valve treatment at an early age, with instruments getting smaller [11]. Accordingly, in 2009 Sudarsanan et al. reported a mean age of 45 days at urethral valve treatment [12], while Lal et al. in 1998 reported urinary diversion until the age of 9e12 months, if the urethral caliber was too small for a 9 Fr resectoscope [13]. The other change in valve treatment in recent years is the more liberal use of lasers instead of cold-knife incision. Whether any of these
Etiology and outcome of the perineal repair
773
Table 3 etiology.
Surgical
Stricture etiology
Success Technique of reconstruction rate n (success rate)
outcomes
EPA Trauma
87.5% (7/8) History 80.0% of PUV (4/5) Catheter 100% (1/1) Irradiation 100% (1/1) Unknown 100% (2/2)
stratified
according
to
BMG BMG (single-staged) (two-staged)
6 1 (100%) (100%) 2 2 (50%) (100%) e 1 (100%)
1 (0%)
e
1 (100%)
e
e
2 (100%)
e
1 (100%) e
factors influence stricture formation after valve treatment remains unclear and will need further investigation. In the case of a suspected stricture, Garibay et al. found that uroflow examination is an important non-invasive tool to evaluate the functional results of urethroplasty in children and detect asymptomatic strictures [14]. In suspicious uroflow, further diagnostic measures should always include radiologic or endoscopic assessment. At this time, we feel that our approach of performing a VCUG 3 weeks postoperatively to assess for good wound healing and to rule out early failure of stricture repair, followed by conservative measures, is the likeliest follow-up to be conducted in everyday life. These conservative measures consisted of uroflow, assessment of residual urine, and inquiry as to whether straining or recurrent urinary tract infections were observed after surgery. Regardless of etiology, open reconstruction for proximal strictures in children following the same algorithms as in adults leads to excellent results, comparable to larger series for adult urethral reconstruction. Some authors report the need for a transpubic access to complex posterior urethral strictures to achieve a successful repair [15,16]. However, as all patients in this series were treated via a perineal access with good results and Rourke et al. [10] report a success rate of 94.4% with a perineal approach as well, the need for transpubic surgery needs to be reconsidered. Usually even longer strictures can be managed via this incision, and a pubectomy may only be required in selected cases with complex post-traumatic strictures if a tension-free anastomosis via the perineal approach cannot be achieved. Again, as in adult urethral reconstruction, we prefer EPA and single- or two-staged buccal mucosa reconstruction over other techniques. The timing of the second stage procedure for tubularization at 3 months after the initial procedure is the same as in adults; indeed we find this a suitable timing in children as well due to the possibly more rapid healing of pediatric tissue. Poor results have been reported for transurethral stricture treatment in children, with success rates of 42% for dilation with guide wire [17] and 33e50% for DVUI [18,19]. However, some techniques of open reconstruction have shown unfavorable results as well. While for
augmentation with tube graft a success rate of 33% was reported [5], Koraitim [3] found a success rate of 46% with scrotal inlay urethroplasty. In the present study, the success rate with EPA was 100% and for single-staged buccal mucosa urethroplasty it was 85.7%. For twostaged procedures, the success rate in this group was only 50%, as one of the two performed staged procedures failed. However, due to the very small number of patients in this group, a larger series is required to help determine the success rate for two-staged urethroplasty in children. Surgical outcomes stratified according to etiology shows that for the post-traumatic strictures, reconstruction could mostly be achieved by EPA with excellent results, while a single- and a two-staged BMG was required in one case each. While in the case of single-staged reconstruction the patient remained recurrence free, the two-staged urethroplasty failed. Though no single reason for stricture recurrence could be established in this patient, local tissue conditions after traumatic disruption are suspected to have influenced the outcome. Special attention needs to be paid to anatomical differences in children as opposed to adults in urethral reconstruction. Due to the small urethral caliber and tissue delicacy, pediatric stricture repair poses a considerable challenge to the surgeon. The urethra is less elastic and, together with the small size of the urethra, we believe that in children an EPA should not be performed in strictures of more than 1 cm. Moreover, the risk of erectile dysfunction has to be accounted for in particular, and surgery with magnifying glasses is mandatory. The true risk for the development of erectile dysfunction in children will require further investigation, as patient numbers in studies are still relatively small and mostly the follow-up is not long enough (i.e. children are still too young) to allow for reliable adjustment of erectile function. Furthermore, covering the reconstructed part of the urethra with the fragile, still developing corpus spongiosum requires careful dissection in the beginning and adaption at the end of surgery. However, considering the poor results of transurethral stricture treatment in children [18,19], open reconstruction should be discussed in selected cases even in small children. The reason to perform reconstructive surgery on a child as young as 1 year in the bulbar stricture group was a complete obliteration of the bulbar urethra, requiring either open reconstruction or suprapubic cystostomy. In this child, the operative result was favorable with no stricture recurrence on follow-up. The two stricture recurrences occurred in a child with previous traumatic urethral disruption and in one with a history of PUV. The reasons for stricture recurrence are difficult to establish: while in the case of traumatic disruption the local tissue conditions and impaired microvascularization might be suspected to have influenced the outcome, a single reason for stricture recurrence could not be established for either of these patients. One of the limitations of this study is the shorter follow-up in some cases, somewhat limiting the informative value of the stricture recurrence rates. However, with a median follow-up of 30 months and a range of 4e115 months, most patients are surely to be counted as long-term follow-up. Another is the retrospective nature of the study.
774
Conclusion The most common etiology for open urethral reconstruction in children was trauma and previous valve treatment. In our hands, the perineal approach for stricture repair was safe and successful. The stricture recurrence rate was low, and incontinence only associated with additional bladder neck trauma. Accordingly, open reconstruction even in small children should be considered feasible and does not have to be postponed until later in life.
Conflict of interest None.
Funding None.
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