|Year : 2015 | Volume
| Issue : 1 | Page : 7-11
Modified Cantwell-Ransley epispadias repair in children our experience
Rajendra B Nerli1, Vijay C Pujar2, Ranjeet A Patil1, Sujata M Jali3
1 Department of Urology, KLE Kidney Foundation, KLES Dr. Prabhakar Kore Hospital, Jawaharlal Nehru Medical College Campus, Belgaum, Karnataka, India
2 Department of Pediatric Surgery, KLES Dr. Prabhakar Kore Hospital, Jawaharlal Nehru Medical College Campus, Belgaum, Karnataka, India
3 Department of Pediatrics, KLES Dr. Prabhakar Kore Hospital, Jawaharlal Nehru Medical College Campus, Belgaum, Karnataka, India
|Date of Web Publication||16-Jan-2015|
Ranjeet A Patil
Department of Urology, KLE Kidney Foundation, KLES Dr. Prabhakar Kore Hospital, Jawaharlal Nehru Medical College Campus, Nehrunagar, Belgaum - 590 010, Karnataka
Source of Support: None, Conflict of Interest: None
Introduction: We retrospectively evaluated our experience with modified Cantwell-Ransley epispadias repair at our center to determine the complications and long-term results. Materials and Methods: We retrospectively reviewed the case records of 43 male children with a mean age of 9.13 ± 1.94 years who underwent primary epispadias repair at our center. The results of epispadias repair were assessed by both physical and endoscopic examination. All children who were old enough to opine as well as all parents/guardians were interviewed during the follow-up visits. Results: Urethrocutaneous fistulae occurred in 17.85% (5/28) children of the classic bladder exstrophy group and in 13.33% (2/15) children with penopubic epispadias. Postoperative cystoscopy done 12 weeks after repair revealed a smooth urethral tube in 81.39% (35/43) of children. With the patient in a standing position, the penis was dangling downward or in a horizontal position in 88.37% (38) of children, 85% of the patients ≥18 years of age were satisfied with both the functional and cosmetic outcome, as assessed by short form 36 and 93.02% (40/43) of the patients were continent during the daytime with voided volumes of more than 200 ml. Conclusion: In our experience, Cantwell-Ransley repair creates a functionally and cosmetically acceptable penis and produces a reliably tubularized neourethra with acceptable complication rates.
Keywords: Cantwell-Ransley repair, epispadias, exstrophy bladder, repair
|How to cite this article:|
Nerli RB, Pujar VC, Patil RA, Jali SM. Modified Cantwell-Ransley epispadias repair in children our experience. J Sci Soc 2015;42:7-11
| Introduction|| |
Isolated male epispadias is a rare anomaly, with a reported incidence of 1 in 11,700 males.  Most male children (70%) with epispadias present with complete epispadias with incontinence. The normal urethra is replaced by a broad mucosal strip lining the dorsum of the penis extending toward the bladder, with potential incompetence of the sphincter mechanism. The displaced meatus is free of deformity and occurrence of urinary incontinence is related to the degree of the dorsally displaced urethral meatus.  All types of epispadias are associated with varying degrees of the dorsal chordee. The corpora are of normal caliber, but the divergent pubis shortens the length of the penis. These penile deformities are corrected surgically by penile lengthening release of the dorsal chordee and urethral reconstruction. The penis is lengthened by mobilizing the crura from the inferior pubic attachments and joining the freed crura in the midline.  Additional penile length and release of the dorsal chordee can be obtained by excision of the fibrous chordee tissue between the corpora and pubis. Division and lengthening of the urethral groove may be necessary using paraexstrophy skin flaps. 
Several surgical procedures have been described in the literature, for the reconstruction of the urethra in patients with either complete epispadias or classical bladder exstrophy. Cantwell described the technique of reconstruction, wherein the dorsal urethral plate was mobilized completely, and the tubularized plate was transplanted ventrally between the separated corpora.  Young related the high fistula rates associated with the Cantwell procedure to excessive mobilization of the urethra and recommended limited mobilization during the reconstruction of the urethral tube.  Ransley subsequently successfully modified this technique. The Cantwell-Ransley procedure provides a pleasing glandular appearance, a ventral urethra free of fistulas and the opportunity to correct the dorsal chordee in the penopubic area and in the corpora themselves.  We reviewed the results of our experience with Cantwell-Ransley procedure performed at our center over the past 15 years.
| Materials and methods|| |
We retrospectively reviewed the case records of all male children undergoing epispadias repair at our center during the period July 1997 to June 2012. A total of 43 male children with a mean age of 9.13 ± 1.94 years underwent primary epispadias repair. Of these, 28 children had classic bladder exstrophy, and the remaining 15 had penopubic epispadias. All the 28 children with bladder exstrophy had undergone closure of the bladder alone previously (five at our center and the remaining 23 referred from other centers) and had presented for primary repair of epispadias. The modified Cantwell-Ransley technique was used for primary urethroplasty in all children as described in detail by Gearhart [Figure 1]a-c and [Figure 2]a-e. The urethral mucosal plate was dissected away from the corpora except for the distal most 1 to 1.5 cm so as to get the urethra deeper under the corpora at the glans level. All children received 25 mg testosterone enanthate injections 8 and 4 weeks preoperatively.
The results of epispadias repair were assessed by both physical and endoscopic examination. All children who were old enough to opine as well as all parents/guardians were interviewed during the follow-up visits.
| Results|| |
During the study period, 43 male children underwent primary repair of epispadias using the Cantwell-Ransley technique. All the children had a noticeable clinical response to preoperative testosterone injections. Preoperative cystoscopy was done in all children to assess the bladder neck. The mean operating time was 117 ± 24.6 min. During the procedure, iatrogenic buttonholes occurred in the urethral plate in two children.
Catheter was removed after 10 days in all children. Urethrocutaneous fistulae occurred in 17.85% (5/28) children of the classic bladder exstrophy group and in 13.33% (2/15) children with penopubic epispadias. Postoperative cystoscopy done 12 weeks after repair revealed a smooth urethral tube in 81.39% (35/43) of children. With a child in a standing position, the penis was dangling downward or in a horizontal position in 76.74% (33) of children. The appearance of the penis was acceptable to 69.76% (30) of the parents at 12-24 weeks after surgery.
Fistulas that occurred in seven children occurred at the base of penis in six children and at the midshaft in the remaining child. None of the two children who had developed buttonholes during surgery had a fistula. In none of the children, the fistulas closed spontaneously on insertion of the catheter. A separate procedure to close the urethrocutaneous fistulas was performed in all these seven children.
Dorsal skin incision, infection, and separation were seen in three other children. The neourethra was intact in all these three children. The skin wound healed by secondary intention and none required secondary suturing. Stricture urethra was noticed in 4 (9.3%) of children, all belonging to the classic bladder exstrophy group. The stricture was noted at the proximal anastomotic site in all these four children. The strictures could be managed successfully by urethral dilatation.
Twenty of these 43 children are more than or equal to 18 years of age today. Eight of these children have undergone multiple surgeries for repeat chordee correction and excision of scar tissue. Following these surgeries, 88.37% (38/43) of the patients have the penis that is dangling downward or in a horizontal position in a standing position. When assessed by short form 36 (SF-36) (v2) health questionnaire, 85% (17) of these patients were satisfied with the cosmetic and functional outcome with a mean SF-36 score of 2775.7 ± 27.18. All these patients are experiencing good erections. Two of these patients are married, and three others are in heterosexual relations. All these five patients are experiencing normal erections and sexual function.
The follow-up ranges from 12 to 180 months. Totally, 40/43 children are over 6 years of age, and most are dry during the daytime. Nearly, 60% of these patients do experience occasional nocturnal enuresis and also experience episodic grade I stress incontinence.
| Discussion|| |
Epispadias has been considered as the least severe defect of the exstrophy-epispadias complex; however, the treatment of this anomaly is far from easy and trivial.  The key concerns that need to be addressed during reconstruction of penis and urethra, so as to ensure a functionally and cosmetically acceptable penis are correction of dorsal chordee, creation of a straight urethra, glandular reconstruction, maintenance of erectile function, creation of urinary continence (proximal epispadias), satisfactory appearance and penile skin cover.
The Cantwell-Ransley technique is the most popular and widely used approach to epispadias repair.  Surer et al., Reporting on their 10 years' experience with the modified Cantwell-Ransley epispadias repair in 93 males (79 had classic bladder exstrophy and 14 had complete epispadias) had a 23% urethrocutaneous fistula rate in the immediate postoperative period and 19% at 3 months.  The success rates following epispadias repairs vary depending on the degree of epispadias, age of the child/patient, experience of the surgeon and the presence of previously operated tissues. Similarly, Baird et al., evaluated and updated the long term results of using this technique on 129 boys (97 had classic bladder exstrophy and 32 complete epispadias).  Urethrocutaneous fistulae were noted in 16% and 33% after primary and repeat urethral repair, respectively.
Penile cosmesis following repair is a subjective measurement. What appears as a satisfactory appearance to the surgeon may not equal that of the patient's expectations and vice-versa. The cosmetic success rates of reported series are thus difficult to validate and compare. Lottmann et al., reviewing their series of 40 patients reported a 90% successful anatomic and functional result following repair at a mean follow-up period of 3 years.  In our series, 69.76% of the parents accepted the appearance of the repaired penis at 12-24 weeks follow-up. Moreover, 85% of the patients more than or equal to 18 years of age were satisfied with both a functional and cosmetic outcome, as assessed by SF-36. The mean SF-36 score was 2775.7 ± 27.18.
In epispadias, as in the bladder exstrophy, bladder capacity is the predominant indicator of eventual continence.  Arap et al., reported higher continence rates in patients who had an adequate bladder capacity before bladder neck reconstruction than in those with inadequate capacity (71% vs. 20%, respectively). In addition, in Arap's group of patients with complete epispadias, most obtained continence within 2 years, similar to results in patients with classic bladder exstrophy. As in the exstrophy population, repair of the epispadiac deformity results in an increase of outlet resistance leading to continence. In our series, 93.02% (40/43) of the patients were continent during the daytime with voided volumes of more than 200 ml. For patients who do not gain sufficient urinary continence following the primary repair Grady and Mitchell, have been able to achieve urinary continence using a modification of the Leadbetter bladder neck procedure. 
Complications following epispadias repair include the development of urethrocutaneous fistula, persistent chordee, difficulty with urethral catheterization and erectile dysfunction. Fistula rates for the Cantwell-Ransley repair range from 5% to 20%. , In our series, we had a fistula rate of 16.27% (7/43). Several remedies have been suggested so as to reduce the fistula rate, including the use of tunica vaginalis pedicled wrap primarily along with the repair.  Pippi Salle et al., described a technical modification using a ventral penile skin flap, which facilitated dorsal skin closure, improved cosmesis and eliminated chordee.  In the 11 cases operated by them, all patients had an uneventful course after surgery and no patient developed recurrence of chordee or fistula.
Lottmann et al., reported several complications following primary epispadias repair that required further surgical reconstruction in 45% of their patients.  These complications were more common in patients who underwent this procedure as part of a staged exstrophy closure versus isolated epispadias. The high rate of re-operation reflects the technical difficulty involved in the repair, even in experienced hands. ,,,,,,,
| Conclusion|| |
Epispadias is a rare congenital disorder representing one end of the spectrum of the epispadias-exstrophy complex. Operative techniques involved in the repair of this condition are technically demanding. The care giver needs to be an experienced surgeon, with long-term commitment to these patients as many patients would require further more operations to achieve urinary continence, body image, and sexual function. In our experience, Cantwell-Ransley repair creates a functionally and cosmetically acceptable penis and produces a reliably tubularized neourethra with acceptable complication rates.
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[Figure 1], [Figure 2]