Modified penile disassembly technique for boys with epispadias and those undergoing complete primary repair of exstrophy: Long-term outcomes

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1 bs_bs_banner International Journal of Urology (2014) 21, doi: /iju Original Article: Clinical Investigation Modified penile disassembly technique for boys with epispadias and those undergoing complete primary repair of exstrophy: Long-term outcomes Ahmed Zaki Mohamed Anwar, Mamdouh Abdel-Hamid Mohamed, Alayman Hussein and Alaa Mohammed Shaaban Department of Urology, Faculty of Medicine, El-Minia University, El-Minia, Egypt Abbreviations & Acronyms BE = bladder exstrophy BNR = bladder neck reconstruction BUR = bilateral ureteral reimplantation CPRE = complete primary repair of exstrophy VUR = vesicoureteral reflux Correspondence: Ahmed Zaki Mohamed Anwar M.D., Department of Urology, Faculty of Medicine, El-Minia University, El-Minia 61111, Egypt. zakiuro67@yahoo.com Received 13 September 2013; accepted 19 March Online publication 15 April 2014 Objectives: To describe our experience performing the modified penile disassembly technique for boys with epispadias and for those undergoing complete primary repair of exstrophy. Methods: Between January 2004 and July 2009, 34 boys underwent the modified penile disassembly technique at our institution. The first group included 15 boys with bladder exstrophy who underwent complete primary repair of exstrophy. The second group comprised 11 boys with penopupic epispadias after previous closure of bladder exstrophy. The third group included 8 boys with isolated complete epispadias. Results: The age range was 3 months to 8 years (median, 9 months). The follow-up time ranged from 36 months to 8 years (mean, 63 months). A conical-shaped glans with the absence of any ischemic changes occurred in 94% of patients. A mild degree of penile dorsal tilt occurred in 11.7% of patients, urethrocutanous fistula in 17.6% and meatal stenosis 5.8%. In cases of complete primary repair of exstrophy, hydronephrosis occurred in 66.6% of patients. Vesicoureteral reflux appeared in 60% of patients; despite suppressive antibiotic therapy, 33.3% are awaiting reimplantation. Continence with volitional voiding with dry intervals of 3 h was achieved in 40% of patients. Conclusions: The modified penile disassembly technique can be used in epispadias and complete primary repair of exstrophy with excellent cosmetic results. Preservation of the distal urethral plate along with both hemiglans avoids shortening and prevents occurrence of hypospadias. Complete primary repair of exstrophy is a feasible technique with positive effects on continence with preservation of kidney function. Key words: bladder exstrophy, continence, epispadias, penile, urologic surgical procedures. Introduction The incidence of epispadias has been calculated to be 1 per males. 1 Epispadias repair presents a surgical challenge to the urologist. The goals of epispadias reconstructive procedures are to achieve cosmetic and functional outcomes with a straight penis, glandular meatus, and acceptable continence. 2 Ransley et al. were the first to develop the incomplete penile disassembly technique for epispadias. 3 Separation of the three penile components was an attractive idea proposed by Mitchell and Bagli for use when performing the complete penile disassembly technique to improve epispadias repair. 4 Grady and Mitchell extended the complete disassembly technique to be applied in the CPRE. 5 This dramatically changed the treatment of exstrophy-epispadias complex to a single-stage procedure. 5 7 Despite the versatility of Mitchell technique, 4,5 ischemic changes at the distal glans, 8 loss of the glans or corpora 9 and shortening of the urethral plate with resultant hypospadias 4,7,10 have been reported. Perovic et al. described a variation of the Mitchell technique 4 for epispadias that involves leaving the distal end of the urethral plate attached to both hemiglans to circumvent the problem of shortening. 11 El-Sherbiny and Hafez extended this modification to CPRE and were successful in preventing hypospadias. 12 Herein, we describe our long-term experience with and outcomes from a large series of surgeries performed using a modified penile disassembly technique (Perovic method) 11 in boys with epispadias and in those undergoing CPRE The Japanese Urological Association

2 Modified disassembly epispadias repair (a) (b) (c) (d) (e) Fig. 1 Complete primary repair of exstrophy. (a) Preoperative appearance showing the outlining of the incision. (b) Complete separation between the urethral plate and corpora cavernosa, except for the distal end attached to the hemiglans. (c) Bladder and urethral closure (d) Corporal rotation; the arrow points to symphyseal closure (e) Skin closure with an orthotopic meatus. Methods Between January 2004 and July 2009, 34 boys underwent the modified penile disassembly technique at our institution. The patients were divided into three groups; the first group included 15 boys with BE who underwent CPRE. The second group comprised 11 boys with penopupic epispadias as a second stage following previous closure of BE. The third group included eight boys with isolated complete epispadias. During the study period, we excluded five patients with BE from undergoing CPRE. These included patients after failure of primary bladder closure and those with a small bladder template. All the boys who underwent CPRE were younger than 1 year of age. Surgical technique The preliminary skin incisions, bladder dissection, and bladder closure were consistent with those described by Grady and Mitchell. 5 At the start of the epispadias repair, the urethral plate was outlined dorsally. The ventral penile skin was degloved to the scrotum. The urethral plate was separated from corporal bodies with special care taken to preserve the mesentery, which carries the blood supply. The unique step in this modified disassembly technique was leaving the distal end of the urethral plate attached to both hemiglans. 11 This prevented shortening of the urethral plate and achieved an orthotopic meatus. In cases of CPRE, a pelvic osteotomy was performed and immobilization was maintained by a hip spica cast for 21 days. Ureteral stents were retrieved through the urethra, and were maintained for days. Drainage of the bladder was accomplished through a suprapubic tube for 3 weeks. The modified penile disassembly technique is outlined in Figures 1 3. Follow-up Follow-up was performed 1 month after surgery, 3 months later, and every 6 months. Follow-up visits focused on the cosmetic results in terms of the meatal position and appearance, glans configuration, penile skin changes, fistula, and penile shaft angulation. Evaluation of the reconstructed urethra was assessed by asking the parents if there had been any straining during voiding and by inspection of the urinary stream. In patients treated with CPRE, assessment was expanded to include any history of recurrent urinary tract infection and continence condition. Evaluation of upper tract condition was evaluated by serial renal ultrasound and serum creatinine concentration. The presence or absence of VUR was detected by voiding cystourethrography. Suppressive antibiotics were offered to all patients exhibiting reflux The Japanese Urological Association 937

3 AZM ANWAR ET AL. (a) (b) (c) (d) Fig. 2 Second stage epispadias repair. (a) Preoperative appearance. (b) Complete separation between the urethral plate and corpora cavernosa except for the distal end attached to the hemiglans. (c) Glanuloplasty and corporal rotation completed. (d) Skin closure with an orthotopic meatus. (a) (b) (c) (d) Fig. 3 Isolated epispadias repair. (a) Preoperative appearance. (b) Complete separation between the urethral plate and corpora cavernosa except for the distal end attached to the hemiglans. (c) Glanuloplasty and corporal rotation completed (d) Skin closure with an orthotopic meatus. Results The modified disassembly technique was applied in 34 boys. The age ranged from 3 months to 8 years (median, 9 months). Fifteen boys underwent CPRE, 11 underwent epispadias repair as a secondary procedure after previous bladder closure, and eight underwent peno-pubic epispadias repair as the sole primary procedure. The follow-up time ranged from 36 months to 8 years (mean, 63 months). The cosmetic appearance after surgery was excellent and was pleasing to the parents. A conical-shaped glans with the absence of any ischemic changes occurred in 32 patients (94%). An orthotopic meatus was achieved in all patients. A mild degree of penile dorsal tilt occurred in four patients (11.7%) and was observed during penile erection. Skin-related complications included mild dehiscence of the dorsal skin of the penis that healed without problems in three boys (8.8%) and urethrocutanous fistula in six (17.6%). The location of the fistula was penopupic in four patients and middle penile in two. Three fistulas occurred after staged exstrophy-epispadias closure, two after CPRE and one after isolated epispadias. During the follow-up, the fistula closed spontaneously within 3 months in one patient. The remaining five patients (14.7%) were scheduled for later closure. Meatal stenosis occurred in two patients (5.8%); one responded to postoperative dilatation and the other was relived by an external meatotomy. In cases of CPRE, renal function was preserved based on serial serum creatinine concentration and renal growth observed by ultrasound during the follow-up. During the early follow-up period, hydronephrosis occurred in 10 patients (66.6%): mild hydronephrosis in seven patients, moderate in two, and severe in one. During the long-term follow-up, hydronephrosis remained in four patients (26.6%): mild hydronephrosis in three patients and moderate in one. VUR appeared in nine patients (60%). Despite the use of suppressive antibiotic therapy, five patients (33.3%) developed recurrent breakthrough infections and are awaiting reimplantation; during the follow-up, two of these patients were found to have a small bladder capacity and augmentation and reimplatation will be performed in the same session. In our series of CPRE, six patients (40%) achieved continence with volitional voiding with dry intervals of 3h. Discussion The repair of exstrophy epispadias complex has progressed considerably since the novel technique first reported by Cantwell 13 to more advanced techniques and modifications such as CPRE. 5 7,10 Gearhart summarized the steps for successful epispadias repair as correction of dorsal chordee, urethroplasty, glanuloplasty, and finally skin closure. 14 Among various types of epispadias repair the complete penile disassembly technique proposed by Mitchell and Bagli 4 is the most recent and one of the best. 11 It separates the penile components effectively, returns the urethra to the orthotopic position, and provides successful chordee removal and sound The Japanese Urological Association

4 Modified disassembly epispadias repair glandular closure. 4 The feasibility of this technique in CPRE have been confirmed. 5 7,10 However, it carries inherent risk of shortening of the urethral plate with resultant hypospadias, which has a reported incidence of 22.7% to 68%. 4,10,12,15 The higher incidence occurs in older children and after failure of initial bladder closure. 10,16 However, Hammouda and Kotb achieved an orthotopic meatus in all cases. 6,8 To reduce the likelihood of hypospadias, Perovic et al. left the distal end of the urethral plate attached to the hemiglans by a small tissue bridge. 11 Borer et al. used interrupted urethral sutures that reduced the incidence to 20%. 7 Caione et al. performed multiple Z-plasties along the entire urethral plate and bladder neck to ensure urethral plate lengthening. 17 In the current study, the modified disassembly (Perovic method) was used in all cases. This provided better vascular supply to the distal urethral strip and prevented shortening. Our technique produced an orthotopic meatus and the absence of any ischemic changes at the distal glans in all cases. This is in agreement with previous reports. 11,12 The claim that penile length may be affected by using the modified rather than the complete penile disassembly technique is incorrect. El-Sherbiny and Hafez found no significant difference in mean penile length between the two techniques. 12 This was explained by the fact that the anterior corporeal length was reduced in BE whereas the posterior corporeal length was normal. 18 In our work, we made every attempt to ensure penile lengthening using standard lengthening techniques. In addition, for patients requiring epispadias repair after previous bladder closure, all remnants of the suspensory ligament and scarring from the previous repair were excised. There are two established methods for the treatment of classic BE; modern staged repair of exstrophy and the more recent CPRE. The choice depends on the experience of staff, bladder template characteristics, and the preference of the patient s family. 19 The proposed advantages of CPRE include the limited operative procedures, better bladder growth, improved continence even without BNR, and favorable outcomes. 5,6,15 CPRE can also be used after failure of initial bladder closure of exstrophy. 16,20 However, CPRE carries a risk of serious problems, especially with the use of the complete disassembly technique. Reported complications include wound dehiscence, bladder prolapse, pubic separation, 21 and loss of the hemiglans or urethra. 9 The suggested etiologies for these complications include technical inexperience and venous and arterial problems. 9 Use of the modified disassembly technique in CPRE 12 may carry some technical concerns, such as interference with proper mobilization of the vesicourethral unit. 22 In addition, the deep position of the bladder and urethra in the pelvis, especially during symphyseal approximation, may lead to corporal angulations and shortening. 12 However, this is not always the case. Based on the bony anomalies associated with BE, symphyseal approximation lead to simultaneous rotation of the corpora and inward rotation of pubic bones and thus shortening and angulations can be prevented. 12 Our preference is now to perform CPRE. In the present study, the modified disassembly technique was used in 15 patients. Complete mobilization of the vesicourethral unit and proper placement of the bladder and the urethra in the pelvis were achieved. An orthotopic meatus was achieved in all patients, and this result compares favorably with other results. 12 Proper patient selection before CPRE is helpful in this regard. The reported incidence of fistula after Mitchell epispadias repair is 2.4% to 17.6%. 4,8,23 After CPRE, fistula occurs in 3.0% to 19.3% of patients. 5 7,15,24 The highest incidence (30%) occurs after failure of initial bladder closure. 20 In the present study, fistula occurred in 12.5% of patients with isolated epispadias, in 13.3% of those treated with CPRE, and in 27% of those receiving staged surgery. The overall fistula rate after spontaneous closure was 14.7% and this compared favorably with previously reported rates. 5,15,23 The presence of hydronephrosis after CPRE varies between series and from early to late follow-up. The incidences of hydronephrosis is 9.0% to 69.6% during early follow-up, 6,15,16,20 and late-onset hydronephrosis occurs in 21.8% to 33.9% of patients. 15,25 In the present series, renal function as determined by serial serum creatinine concentration and renal growth observed with ultrasound was preserved during the follow-up. Early-onset hydronephrosis occurred in nine patients (60%), with follow-up, hydronephrosis remained in four (26.6%). This is in agreement with previous reports. 15,16,20 The incidence of VUR after CPRE varies and the reported incidence is 18.1% to 73.9%. 5 7,16 Re-implantation is needed in 48 to 66% of cases. 5,15,20,25 The absence of febrile urinary tract infection has been reported only by Hammouda and Kotb. 6 Recently, Braga et al. performed BUR in conjugation with CPRE to reduce the possibility of associated reflux. 25 They achieved a complete absence of reflux and reduction in the incidence of febrile urinary tract infection to 7%. In the present study, VUR appeared in nine patients (60%) and re-implantation was needed in 33.3% of these patients. further experience on BUR together with CPRE is needed in the future. One of the proposed advantages of CPRE is the achievement of acceptable continence without BNR, although this cannot always be attained. Many factors affect continence after CPRE including gender, follow-up duration, location of the surgery, and history of previous bladder closure. 16,20 Dry intervals of 3 h are achieved in 19% to 80% of patients, 5,6,15,26 but this value decreases to 9% to 16% after previous failed closure. 9,16 Nevertheless, the long-term results after CPRE reveal unsatisfactory continence results because BNR is needed in 63% to 86%. 7,15,16,27 In the current series, continence with volitional voiding with dry intervals of 3 h was achieved in six patients (40%). Further follow-up is needed to verify the long-term continence results and the need of BNR. The repair of BE is a difficult issue that needs expertise. Although CPRE is advocated as a single-stage operation 4 to solve this problem, it is not performed as such. Incontinence 7 and VUR 21 remain issues to be solved in the future. Conclusions The modified penile disassembly technique can be used to treat epispadias and with CPRE with excellent cosmetic results. Preservation of the distal urethral plate in conjugation with both hemiglans avoids shortening and prevents the occurrence of hypospadias. CPRE is a feasible technique that positively influences continence and preserves kidney function. Proper patient selection is important to achieve good results The Japanese Urological Association 939

5 bs_bs_banner AZM ANWAR ET AL. Conflict of interest None declared. References 1 Dees JE. Congenital epispadias with incontinence. J. Urol. 1949; 62: Gearhart JP, Sciortino C, Ben-Chaim J, Peppas DS, Jeffs RD. The Cantwell-Ransley epispadias repair in exstrophy and epispadias: lessons learned. Urology 1995; 46: Ransley PG, Duffy PG, Wollin M. Bladder exstrophy closure and epispadias repair. In: Spitz L, Nixon HH (eds). Rob and Smith s Operative Surgery. Pediatric Surgery, 4th edn. Buttenvorths, Boston, MA, 1988; Mitchell ME, Bagli DJ. Complete penile disassembly for epispadias repair: Mitchell technique. J. Urol. 1996; 155: Grady RW, Mitchell ME. Complete primary repair of exstrophy. J. Urol. 1999; 162: Hammouda HM, Kotb H. Complete primary repair of bladder exstrophy: initial experience with 33 cases. J. Urol. 2004; 172: Borer JG, Gargollo PC, Hendren WH et al. Early outcome following complete primary repair of bladder exstrophy in the newborn. J. Urol. 2005; 174: Hammouda HM. Results of complete penile disassembly for epispadias repair in 42 patients. J. Urol. 2003; 170: Husmann DA, Gearhart JP. Loss of the penile glans and/or corpora following primary repair of bladder exstrophy using the complete penile disassembly technique. J. Urol. 2004; 172: El-Sherbiny M, Hafez A, Ghoneim M. Complete repair of exstrophy: further experience with neonates and children after failed initial closure. J. Urol. 2002; 168: Perovic S, Vukadinovic V, Djordjevic ML, Diakovic NG. Penile disassembly technique for epispadias repair: variants of technique. J. Urol. 1999; 162: El-Sherbiny M, Hafez A. Complete repair of bladder exstrophy in boys: can hypospadias be avoided. Eur. Urol. 2005; 47: Cantwell FV. Operative treatment of epispadias by transplantation of the urethra. Ann. Surg. 1895; 22: Gearhart JP. Evolution of epispadias repair, timing, techniques and results. J. Urol. 1998; 160: Shnorhavorian M, Grady RW, Andersen A, Joyner BD, Mitchell ME. Long-term follow-up of complete primary repair of exstrophy: the Seattle experience. J. Urol. 2008; 180: Hafez A, EL-Sherbiny M, Shorrab A. Complete primary repair of bladder exstrophy in children presenting late and those with failed initial closure: single center experience. J. Urol. 2005; 174: Caione P, Nappo SG, Matarazzo E, Aloi IP, Lais A. Penile repair in patients with epispadias-exstrophy complex can we prevent resultant hypospadias? J. Urol. 2013; 189: Silver RI, Yang A, Ben-Chaim J, Jeffs RD, Gearhart JP. Penile length in adulthood after exstrophy reconstruction. J. Urol. 1997; 157: Gearhart JP. The exstrophy-epispadias complex in the new milennum-science, practice and policy. J. Urol. 1999; 162: Baird AD, Mathews RI, Gearhart JP. The use of combined bladder and epispadias repair in boys with classic bladder exstrophy: outcomes, complications and consequences. J. Urol. 2005; 174: Scaeffer AJ, Stec AA, Purvus JT, Cervellione RM, Nelson CP, Gearhart JP. Complete primary repair of bladder exstrophy: a single institution referral experience. J. Urol. 2011; 186: Caione P, Capozza N. Evolution of male epispadias repair experience. J. Urol. 2001; 165: Zaontz MR, Steckler RE, Shortliffe LM, Kogan BA, Baskin L, Tekgul S. Multi-center experience with the Mitchell technique for epispadias repair. J. Urol. 1998; 160: Surer I, Baker LA, Jeffs RD, Gearhart JP. The modified Cantwell-Ransley repair for exstrophy and epispadias: 10-year experience. J. Urol. 2000; 164: Braga LH, Lorenzo AJ, Jrearz R, Bagli DJ, Salle JL. Bilateral ureteral reimplantation at primary bladder exstrophy closure. J. Urol. 2010; 183: Gargollo PC, Borer JG, Diamond DA et al. Prospective follow-up in patients after complete primary repair of bladder exstrophy. J. Urol. 2008; 180: Grady R, Joyner BD, Roedel M et al. The complete primary repair of exstrophy (CPRE) technique for repair of bladder exstrophy and epispadias: long-term follow-up. Presented at meeting of American Academy of Pediatrics, New Orleans, Louisiana, November 1 5, Editorial Comment Editorial Comment to Modified penile disassembly technique for boys with epispadias and those undergoing complete primary repair of exstrophy: Long-term outcomes The modified penile disassembly originated because of concerns about penile soft tissue loss with the complete disassembly procedure and the fact that the hypospadias created by this procedure are often difficult to repair due to a paucity of penile skin. This article shows that the modified technique can be done in most cases. 1 However, 94% of patients had a conical glans and no ischemia, but it is unclear whether the other 6% had ischemia. This reviewer was also confused by the continence data. In the results section, outcomes were reported both by medians and sometimes by mean with the range, but usually this is reported as mean ± standard error, and this requires clarification. Three groups of patients were reported, but the continence results were only reported for the first group, not all of the patients. The continence status should be reported in all three groups, so that comparisons can be made. Trying to link continence to epispadias repair alone is risky, as many reliable groups have reported the need for bladder neck reconstruction in almost 80% of patients who underwent penile disassembly as part of a complete primary repair of exstrophy. John P Gearhart M.D., F.A.A.P., F.A.C.S., F.R.C.S.(Hon.)(Ed.) Pediatric Urology Division, James Buchanan Brady Urological Institute, Johns Hopkins School of Medicine, Charlotte R. Bloomberg Children s Center, Baltimore, Maryland, USA jgearha2@jhmi.edu Conflict of interest None declared. Reference DOI: /iju Anwar AZM, Mohamed MAH, Hussein A, Shaaban AM. Modified penile disassembly technique for boys with epispadias and those undergoing complete primary repair of exstrophy: long-term outcomes. Int. J. Urol. 2014; 21: The Japanese Urological Association