PEDIATRIC UROLOGY Results of a Two-stage Technique for Treatment of Proximal Hypospadias with Severe Curvature: Creation of a Urethral Plate Using a Vascularized Preputial Island Flap Rahşan Özcan,1* Senol Emre,1 Pınar Kendigelen,2 Mehmet Eliçevik,1 Haluk Emir,1 Yunus Söylet,1 SN Cenk Büyükünal1 Purpose: To present the results of a two-stage technique used for the treatment of proximal hypospadias with severe curvature. Materials and Methods: The medical records of children with proximal hypospadias and severe curvature were retrospectively analyzed. A 2-stage procedure was performed in 30 children. In the first stage, the release of chor- dee was performed, and a well-vascularized preputial island flap was created. The vascularized island flap was brought anteriorly and sutured over the ventral surface of the glans and degloved penile shaft. The second stage was performed 6-8 months later. A neourethra was reconstructed by the tubularization of the preputial-urethral plate utilizing the principles of Duplay technique. All surgical procedures were performed between 2005 and 2011. Results: The mean age of the patients was 4.4 years (1–17 years). The mean duration of urethral catheterization was 6 days after the first stage and 10 days following the second stage. The flaps were viable in all of the chil- dren. There was no residual chordee. Following the second stage (n = 30), complications developed in 11 children (36%), namely, a fistula in 7, a pinpoint fistula in 3, and a diverticulum formation in 1. The cosmetic outcome was satisfactory. Uroflowmetry measurements were evaluated, and only one patient had a diverticulum formation at the late follow-up. Conclusion: Vascularized preputial island flap is an alternative to free grafts for the reconstruction of the urethra. The main advantage of this flap technique is the creation of a thick, healthy and well-vascularized urethral plate. The advantages of this technique include better aesthetic appearance, an acceptable complication rate, and a very low rate of diverticula formation. Keywords: hypospadias; surgery; postoperative complications; urologic surgical procedures; reconstructive surgi- cal procedures; surgical flaps; urethra. INTRODUCTION Division of the urethral plate is inevitable in cases of severe proximal hypospadias with a curvature of more than 35-40 degrees. During the last decade, “two- stage procedures” have increased in popularity for this group of patients.(1) In two-stage techniques for the cre- ation of a new urethral plate, the use of free preputial or buccal mucosal grafts is widely practice. After release of the curvature by excision of fibrotic tis- sues in the ventral part and midline dorsal plication, we prepared a rectangular island flap from the inner part of the dorsal preputium with its own vascular supply and transferred it to the ventral part to create a new ure- thral plate. The main advantage of this technique was the creation of a thick, healthy neourethral plate due to sufficient blood flow from the dorsal penile vessels. MATERIALS AND METHODS The medical records of patients with proximal hypospa- dias (penoscrotal, perineal) and severe curvature were retrospectively analyzed. The inclusion criteria for this two-stage technique were penoscrotal or perineal hy- pospadias with a severe curvature of more than 35-40 degrees. The degree of hypospadias was determined be- fore the release of the penile curvature. Those patients with a history of circumcision or loss of foreskin due to previous surgery to correct hypospadias were excluded. In spite of every effort to release the penile curvature and protect the urethral plate, division of the urethral plate was inevitable in this series. First stage (Figures 1-3): After complete degloving and the chordee test, fibrotic tissues in the ventral part were excised, and a midline dorsal plication was performed. 1 Department of Pediatric Surgery, Division of Pediatric Urology, Istanbul University, Cerrahpaşa Medical Faculty, Istanbul, 34098, Turkey. 2 Department of Pediatric Anesthesiology, Istanbul University, Cerrahpaşa Medical Faculty, Istanbul, 34098, Turkey. *Correspondence: Department of Pediatric Surgery, Division of Pediatric Urology, Istanbul University, Cerrahpaşa Medical Faculty, Istanbul, 34098, Turkey. Tel: +90 212 5870310. Fax: +90 212 4143320. E-mail: rozcan1@gmail.com. Received February 2016 & Accepted April 2016 Pediatric Urology 2629 Vol 13 No 02 March-April 2016 2630 A completely straight penis was created. Then, the dis- tance between the ectopic meatus and the glanular tip was measured. A transverse rectangular vascularized island flap (same length with the measured distance) was fashioned according to the principles of Standoli and Duckett (Figures 1 and 2).(2-5) This flap was rotat- ed ventrally and laid between the ectopic orifice and inner part of the glanular wings. The proximally locat- ed orifice was surrounded with 2 short wings from the preputial flap to position non-hair-bearing tissue around it (Figure 3). To prevent serum accumulation under the vascularized flap, 4-5 tiny incisions were made on the plate, and 5-6 fixation sutures were placed to ease the adhesion between the flap and tunica albuginea (Figure 3). The edges of the flap and the glanular wings and pe- nile skin were stitched with 7/0 Vicryl sutures. A Foley catheter was used for drainage for 6 days. The dressing consisted of a combination of bactigras and sponge. Second stage (Figures 4 and 5): Six to eight months af- ter the initial operation, a two-layer urethroplasty using Duplay’s principle was performed. A ventrally trans- posed thick and healthy dartos tissue near the plate were used to cover the neourethra. In cases of insufficient or thin Dartos tissue, a healthy, thick tunica vaginalis flap was used as a second layer coverage. Glanular wings were re-approximated by glanuloplas- ty, using 6/0 Vicryl sutures (Figures 4-7). A silicon catheter was inserted for 7-10 days, and a silicon foam dressing (Smith-Nephew Co. Cavi-Care, Hull, UK) was applied for 5 days. The follow-up studies included a physical examination, direct observation of the urinary stream, and review of mobile-phone videos of urination in the home environment. Postoperative cosmetic eval- uation was performed using the Hypospadias Objective Scoring Evaluation (HOSE) scoring system. The uro- flowmetry parameters of patients who had a uroflow- metre over 5 years were summarized. The maximum flow rate (Q) max values were compared to the uro- flowmetry normogram in healthy boys between 5 and 15 years of age.(6) In 4 of these 30 cases, there were minor (2 cases) and prominent (2 cases) forms of peno- scrotal transposition. Minor ones were corrected during the 2nd operation, while prominent ones were corrected with an additional 3rd operation. Under general anes- thesia, a caudal or pudendal block (using ultrasound microprobe) was administered during each stage by the pediatric anesthesiology team. RESULTS This 2-stage technique was used in 30 patients with the above-mentioned inclusion criteria. The mean age was Table 1. Clinical and demographic characteristics of study patients. Variables Values Age, years (mean) 4.4 (1-17) Type of Hypospadias, no. Penoscrotal 25 Perineal 5 Penoscrotal transposition, no. 4 Follow-up, years (mean) 6.5 (4-10.5) Reoperations, no. (%) Final Location of Neomeatus, no. Pinpoint fistula 3 Glanular region 22 Urethral fistula 7 Subcoronal region 8 Diverticulum formation 1 _____ Total reoperations 11 (36) _____ Table 2. Reoperations due to various complications and final location of the neomeatus in 30 patients. Figure 1. Penoscrotal hypospadias with severe chordee. Figure 2. Following the correction of the ventral curvature, a rectangular vascularized flap is prepared from the dorsal preputial mucosa. Two-stage Technique for Treatment of Proximal Hypospadias-Ozcan et al. 4.4 years (range, 1-17 years). The mean follow-up time was 6.5 years (range, 4-10.5 years) (Table 1). Early postoperative controls were performed on the 7th day and at 1 and 3 months. Late controls were performed at the end of the 1st year and the 5th to the 10th post- operative year (Figures 4-7). A healthy, thick and elastic urethral plate was created in all patients. Small tiny incisions and fixation sutures between the flap and corpora appeared to be responsible for the existence of a thick and healthy neourethral plate. Due to the nice, elastic and well-vascularized texture of the neourethral plate, it was easy to perform a Duplay urethroplasty in each case. The number of additional operations due to various types of complications and the position of the neomeatus is presented in Table 2. From the surgeons' perspective, end aesthetic results appeared to be much better than our previous experi- ence with patients treated with single-stage procedures. The satisfaction rate was similar for parents and sur- geons as well. The HOSE scoring system was used to conduct the postoperative cosmetic evaluation, and the mean HOSE score was 15 (range, 12-16). The results are presented in Table 3. A group of patients who had undergone the operation 5-10 years ago were investigated for urethral dilatation and /or diverticulum formation. We detected only one case of diverticulum formation (Table 2). Uroflowme- try findings are summarized in Table 4. We excluded five patients less than 5 years of age and compared the uroflowmetry parameters of 10 patients between 5-15 years of age based on nomograms for healthy boys. The Qmax value was between the 25th-50th percentile in 5 patients (mean age 6.2, range, 5-7 years) and was greater than the 50th percentile in 5 patients (mean age 10.8, range, 9-15 years). Due to the formation of the ne- ourethra using a well-vascularized thick urethral plate and the reinforcement of neourethra with dartos and/or a tunica vaginalis flap, neither diverticula formation nor urethral dilatation was observed in this series. We did not detect any urination problem with visual ex- amination of the patients during urination and a review of short voiding videos created by the parents. Because the follow-up did not exceed 10 years, we have no in- formation with regard to problems with ejaculation. DISCUSSION The choice of single- or double-stage operations for the Variables of HOSE HOSE Score Number of Patients (n = 30) Meatal location, no. Distal glanular 4 15 Proximal glanular 3 7 Coronal 2 8 Penile shaft 1 _____ Meatal shape Vertical slit 2 22 Circular 1 8 Urinary stream Single stream 2 30 Spray 1 _____ Erection Straight 4 25 Mild curvature (< 10) 3 5 Moderate curvature 2 _____ Severe curvature 1 _____ Fistula formation None 4 20 Single-subcoronal or more distal 3 7 Single-proximal 2 3 Multiple or complex 1 _____ Table 3. Postoperative cosmetic evaluation with the HOSE scoring system. Abbreviation: HOSE, Hypospadias Objective Scoring Evaluation. UFM Parameters Range Mean Qmax, mL/s 6-22 13.1 Time to maximum flow, s 7-37 17.4 Duration of flow, s 17-55 31.5 Mean flow, mL/s 4-17 13.2 Urine volume, mL 109-760 253 Abbreviation: UFM, uroflowmetry. Table 4. Results of late uroflowmetric analysis in 10 patients. Figure 3. This non-tubularized flap is laid between the original urethra and the tip of the glans. Two-stage Technique for Treatment of Proximal Hypospadias-Ozcan et al. Pediatric Urology 2631 Vol 13 No 02 March-April 2016 2632 treatment of severe hypospadias with a severe curvature problem is under debate. Duckett drew our attention to single-stage techniques in the early 80s.(3,4) According to and colleagues, there was only a 10% complication rate with the single-stage transverse island flap tech- nique.(7) However, over the past 10-15 years, two-stage tech- niques have been re-popularized, especially with the efforts of Braca who used free buccal mucosal grafts. (8,9) This was a type of revolution in the treatment of severe proximal cases of hypospadias with prominent curvature and that of patients with crippled hypospadi- as who had insufficient healthy penile skin. Today, the 2-stage techniques using free grafts from the oral cavity or inner prepuce appear to be the most popular methods for treating such cases. Based on clinical practice, the use of 2-stage techniques to treat patients with penoscrotal or perineal hypospadi- as with severe curvature, in whom division of urethral plate is inevitable, may provide better surgical and aes- thetic results. Zheng and colleagues compared the results of single- and two-stage techniques and reported similar compli- cation rates in 66 cases of proximal hypospadias treated by single or 2-stage procedures.(10) However, in 2 re- ports, Castagnetti and colleagues claimed that a lower complication rate and less favorable cosmetic results were associated with 2-stage techniques.(1,11) We used a well-vascularized preputial island flap with an attached healthy and thick dartos pedicle. This healthy and thick Figure 4. Urethroplasty is performed by tabularizing the neourethral plate. A urethral catheter is placed. Figure 6. Postoperative slit like meatus (first post-operative year). Figure 5. Silicone foam dressing (Smith-Nephew cavi-care) intact for 5 days. Figure 7. Postoperative coronal meatus (first post-operative year). Two-stage Technique for Treatment of Proximal Hypospadias-Ozcan et al. dartos tissue served as a safe, 2nd layer for the rein- forcement of the urethral tube in the 2nd stage. According to Snodgrass, better results can be obtained if free preputial grafts are used instead of vascularized flaps.(12) Conversely, Powell and colleagues. did not find any significant difference in complications rates when using free grafts or vascularized flaps.(13) Some have criticized the use of vascularized flaps be- cause of reports of a higher rate of diverticula forma- tion.(14) However, a tight adhesion can be created by inserting tiny incisions on the flap and placing sever- al stitches between the flap and corporal body. In case of insufficient or inadequate Dartos tissue, we always used tunica vaginalis flaps as a 3rd reinforcement layer. These additional techniques appeared to be effective in preventing diverticula formation, at least as of the 5-10 year follow-up. In this group, our aim was always to create a slit-like, wide meatus in the tip of the glans. However, if the me- atus was located in the coronal area and if there was no problem with the calibration and direction of urination, we did not attempt to advance the meatus to the tip of the glans penis. A wide neomeatus, located in the coro- nary sulcus, may occasionally be helpful in preventing possible complications such as diverticula formation, meatal stenosis or fistula formation. McNamara and colleagues reported a reoperation rate of 49% in 134 cases treated for proximal hypospadi- as.(15) Haxhirexha identified a 40% incidence of void- ing and ejaculation problems at long-term follow-up in those cases treated with 2 stage techniques.(16) Our reop- eration rate of 36% therefore appears to be reasonable for a very select group of patients with proximal hypo- spadias and remarkable curvature problems. Uroflowmetry findings are not trustworthy in patients less than 5 years of age because of difficulties in evalu- ation. We compared Qmax values from 10 patients with the nomograms of healthy boys.(6) The Qmax value was < 5th percentile in 2 patients (mean age, 6 years), between the 5-10th percentile in 4 patients (mean age, 6.5 years), between the 25-50th percentile in 2 patients (mean age, 8 years), and between the 50-75th percentile in 2 patients (mean age, 15.5 years). Though age-de- pendent improvements in uroflowmetry patterns and values after hypospadias repair have been reported,(17) we did not have sufficient data from consecutive uro- flowmetry studies to investigate this outcome. There are some limitations to this technique. For in- stance, previous circumcision or operations for hypo- spadias with a loss of foreskin make patients ineligi- ble for this procedure. Moreover, we were not able to obtain sufficient information regarding the incidence of sexual and ejaculation problems in this series. CONCLUSIONS Creation of a healthy urethral plate using a well-vascu- larized transverse rectangular island flap from the inner part of the foreskin is a nice alternative in cases of prox- imal hypospadias with severe curvature when division of the urethral plate is inevitable. Effective strategies to prevent diverticula formation at long-term follow-up include a) the development of dense adhesions created by tiny incisions and small fixation sutures between the tunica albuginea and the flap and b) the reinforcement of the neourethra using a thick and well-vascularized dartos patch and/or the tunica vaginalis. Prolonged fol- low-up is necessary for the evaluation of sexual and ejaculatory problems. CONFLICT OF INTEREST None declared. REFERENCES: 1. Castagnetti MA, El-Ghoneimi A. Surgical management of primary severe hypospadias in children: systematic 20-year review. J Urol. 2010;184:1469-74. 2. Standoli L. One-stage repair of hypospadias: preputial island flap technique. Ann Plast Surg. 1982;9:81-8. 3. Standoli L. Vascularized urethroplasty flaps. The use of vascularized flaps of preputial and penopreputial skin for urethral reconstruction in hypospadias. Clin Plast Surg. 1988;15:355- 70. 4. Duckett JW Jr. Transverse preputial island flap technique for repair of severe hypospadias. Urol Clin North Am. 1980;7:423-30. 5. Duckett JW. The island flap technique for hypospadias repair. Urol Clin North Am. 1981;8:503-11. 6. Gupta DK, Sankhwar SN, Goel A. Uroflowmetry Nomograms for Healthy Children 5 to 15 Years Old. J Urol. 2013;190:1008-13 7. Singal AK, Dubey M, Jain V. Transverse preputial onlay island flap urethroplasty for single-stage correction of proximal hypospadias. World J Urol. 2015 Sep 22 [Epub ahead of print]. 8. Bracka A. Hypospadias repair: the two-stage alternative. Br J Urol. 1995;76 Suppl 3:31-41. 9. Bracka A. A versatile two-stage hypospadias repair. Br J Plast Surg. 1995;48:345-52. 10. Zheng DC, Yao HJ, Cai ZK, et al. Two-stage urethroplasty is a better choice for proximal hypospadias with severe chordee after urethral plate transection: a single-center experience. Two-stage Technique for Treatment of Proximal Hypospadias-Ozcan et al. Pediatric Urology 2633 Vol 13 No 02 March-April 2016 2590Vol 13 No 02 March-April 2016 2634 Asian J Androl. 2015;17:94-7. 11. Castagnetti M, Zhapa E, Rigamonti W. Primary severe hypospadias: comparison of reoperation rates and parental perception of urinary symptoms and cosmetic outcomes among 4 repairs. J Urol. 2013;189:1508-13. 12. Snodgrass W, Bush N. Surgery for Primary Proximal Hypospadias with Ventral Curvature > 30 degrees. Curr Urol Rep.2015;16:69. 13. Powell CR, Mcaleer I, Alagiri M, Kaplan GW. Comparison of flaps versus grafts in proximal hypospadias surgery. J Urol. 2000;163:1286- 8. 14. Divarci E, Dökümcü Z, Ergün R, Elekberova V, Ulman I ̇, Avanoglu A. Comparison of Bracka and TIPU techniques for proximal hypospadias repair. Forth World Congress of Pediatric Surgery, 2013; Abstract book. 12/4. 15. McNamara ER, Schaeffer AJ, Logvinenko T, et al. Management of Proximal Hypospadias with 2-Stage Repair: 20-Year Experience. J Urol. 2015;194:1080-5. 16. Haxhirexha KN, Castagnetti M, Rigamonti W, Manzoni GA. Two-stage repair in hypospadias. Indian J Urol. 2008;24:226-32. 17. Andersson M, Doroszkiewicz M, Arfwidsson C, Abrahamsson K, Sillen U, Holmdahl G. Normalized Urinary Flow at Puberty after Tubularized Incised Plate Urethroplasty for Hypospadias in Childhood. J Urol. 2015;194:1407-13. Two-stage Technique for Treatment of Proximal Hypospadias-Ozcan et al.