|
 
 |
| CASE REPORT |
|
| Year : 2015 | Volume
: 2
| Issue : 4 | Page : 179-183 |
|
Penopubic epispadias repair in adulthood: Surgical and functional outcome with modified Cantwell-Ransley procedure
Terkaa Atim, A Mustapha
Department of Surgery, University of Abuja Teaching Hospital, Gwagwalada, Abuja, Nigeria
| Date of Submission | 18-May-2015 |
| Date of Acceptance | 02-Nov-2015 |
| Date of Web Publication | 22-Dec-2015 |
Correspondence Address:
Terkaa Atim
P.O. Box10978, Garki 900001, Abuja
Nigeria
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2384-5147.172448
Isolated primary penopubic epispadias is a rare anomaly with a reported incidence of one in 11,700 males. It is unusual for patients with this anomaly to present for the first time in adulthood due to the fact that the clinical presentation is striking at birth. The hallmark of surgical repair is the reconstruction of a functionally and cosmetically appealing genitalia as well as proper citing of the neomeatus. Several surgical procedures for complete epispadias have been described in the literature, but the modified Cantwell-Ransley technique and the Mitchell-Bagli repair (complete penile disassembly) are the most widely used. We report a case of a 25-year-old man who presented to our facility for the first time for surgical treatment. The patient underwent the modified Cantwell-Ransley procedure based on surgeon's preference and expertise. The delay in presentation was a thing of concern to us as it is well-known that boys born with epispadias suffer poor self-image as they grow older and become aware of their genitalia. Following a successful modified Cantwell-Ransley procedure our patient's self-esteem, confidence, and sexual appeal greatly improved. We, therefore, conclude that surgical procedures such as the Cantwell-Ransley repair are appropriate even in cases presenting late in adulthood. Keywords: Epispadias, genitalia, neomeatus, penopubic
How to cite this article:
Atim T, Mustapha A. Penopubic epispadias repair in adulthood: Surgical and functional outcome with modified Cantwell-Ransley procedure. Sub-Saharan Afr J Med 2015;2:179-83 |
How to cite this URL:
Atim T, Mustapha A. Penopubic epispadias repair in adulthood: Surgical and functional outcome with modified Cantwell-Ransley procedure. Sub-Saharan Afr J Med [serial online] 2015 [cited 2023 Dec 3];2:179-83. Available from: https://www.ssajm.org/text.asp?2015/2/4/179/172448 |
| Introduction | |  |
Complete male epispadias is a rare and grotesque congenital anomaly which occurs as a result of failed closure of the entire penopubic dorsal urethra. [1],[2],[3],[4],[5] It has a reported incidence of one in 11,700 males. [2],[3],[4],[5],[6] Although rare, this malformation causes great physical, functional, social, sexual, and psychological burdens on patients and families. The clinical appearance of this deformity is so obvious that missing it at birth is unimaginable. Epispadias in adults is extremely rare since such anomalies are usually treated surgically in early childhood. Reconstructive surgery for epispadias is best started early in childhood with decent results. [7],[8] For the fact that there has been no reported case of isolated epispadias in adults in the literature in the West African sub-region, we present this 25-year-old young man with penopubic epispadias whom we managed in our center. Challenges in the treatment of adult patients include long-term changes affecting the urethral mucosa and surrounding tissues as well as concerns about sexual potentials. [7] He was evaluated and successfully had modified Cantwell-Ransley procedure with acceptable cosmetic and functional outcome.
| Case Report | | |
Mr. MM, a 25-year-old single male presented to our facility with an abnormal looking penis, stress urinary incontinence, and splaying of urine. He had no formal education due to the social stigma of his condition. His sister who accompanied him to the hospital claimed ignorance and financial constraints were reasons for not seeking medical aid earlier.
Examination revealed a short, deformed penis with a dorsal penopubic meatal opening and dorsal chordee [Figure 1] and [Figure 2]. The exposed ventral urethral plate was 3 cm wide and adequate. Scrotal examination revealed normal testes. He had an intravenous urogram which showed normal upper tract with a smooth-walled capacious bladder [Figure 3]. The kidneys and other intra-abdominal organs were normal on ultrasonography. His pack cell volume was 42%; serum electrolytes and urinalysis were normal. Renal function tests were also within normal limits, and he was seronegative for human immunodeficiency virus. At urethrocystoscopy, a normal posterior urethra and the verumontanum were seen. The ureteric orifices, bladder mucosa, and estimate of the bladder volume (500 ml) were normal. | Figure 3: Intravenous urogram showing a capacious bladder and normal upper tract
Click here to view |
He was prepared for and had a modified Cantwell-Ransley epispadias repair under general anesthesia. A reverse meatal advancement and glanuloplasty were done using the heineke-mikulicz technique. A penile degloving incision about 5 mm from the coronal sulcus was made and the penis degloved to the scrotum [Figure 4]. The glans wings were developed, and a U-shaped incision was made to raise the urethral plate for tubularization. The two corporal bodies were separated and mobilized from the urethral plate. The urethral plate was tabularized (urethroplasty) over a size 16 silicone catheter using running vicryl 4/0 suture [Figure 5]. Caverno-cavernostomy was done on both sides, and the tubularized urethra was repositioned ventrally by suturing the corpora together above the urethra. This maneuver also corrected the dorsal chordee. A W-flap was raised in the pubic region and the suspensory ligament released thus lengthening the penis from 6 cm to about 8 cm flaccid length. Penile skin was approximated and pubic skin flaps closed [Figure 6]. | Figure 5: The tabularized urethral plate (urethroplasty) over a size 16 silicone catheter
Click here to view |
He had routine parenteral broad-spectrum antibiotics and analgesics postoperatively. On the second postoperative day, he commenced intake of food and oral medications including tolterodine for bladder spasms. The wound dressings were changed as indicated. The urethral catheter was removed on the 14 th postoperative day, and he was noticed to have developed a vesicocutaneous fistula and surgical site infection at the suprapubic wound. Wound care was done with povidone iodine dressings. We reinserted a urethral catheter for continuous drainage, and the fistula closed spontaneously after 9 days with a more cosmetically-acceptable penis [Figure 7].
The catheter was removed, and he was observed to have voided with a good stream and satisfactory urinary continence. He was discharged home and seen in clinic 5 weeks later for follow-up. He was happy with his outlook and control of urine.
| Discussion | | |
Epispadias is a rare congenital condition and is often described as part of bladder exstrophy complex. [9],[10],[11] Epispadias repair is usually carried out during infancy, and the attendant results could have a significant impact on the adults' genitourinary function. [1] It is very unusual for patients with this anomaly to present for the first time as adults. [2],[3] For this reason, we deem it fit to report this case and remind ourselves of the operative techniques used to treat this disorder. Isolated male epispadias accounts for only ten percent of cases of the epispadias-exstrophy complex. [3],[4],[5],[12],[13] It is characterized by failure of tubularization of the urethral plate on the dorsum. [1] This anomaly ranges from a distal glandular to a more proximal penopubic location, the latter as in our patient. [1]
The hallmark of surgical repair is the reconstruction of a functionally- and cosmetically-appealing genitalia as well as proper citing of the neomeatus. [11],[14],[15] Although epispadias is regarded as the milder form of the exstrophy-epispadias complex, the surgical correction of this anomaly could be tasking. In cases of isolated epispadias, only the urethra is affected. Involvement of the bladder neck occurs in only a few occasions. Urinary incontinence may thus result if the defect is proximal enough to affect the urinary sphincter mechanism. [16] Several surgical procedures for complete epispadias have been described in the literature. [9] The modified Cantwell-Ransley technique and Mitchell-Bagli repair (complete penile disassembly) are the most widely described procedures in the repair of epispadias. [9],[14],[17],[18] Cantwell popularized the technique of reconstruction, wherein the dorsal urethral plate was mobilized completely, and the tubularized plate was transplanted ventrally between the separated corpora. [9]
The modified Cantwell-Ransley technique; however, entails partial disassembly of the penis and repositioning of the urethra in its normal anatomical location. In the Mitchell-Bagli repair, a complete penile disassembly is required. The major drawback of the modified Cantwell-Ransley method is the persistence of a short penis and residual dorsal chordee. This setback is usually eliminated in the Mitchell-Bagli complete penile disassembly repair. Aggressive dissection and likelihood of a resulting hypospadias meatus are attendant limitations of the Mitchell-Bagli method. The explanation for this problem is because the urethral plate is usually shorter than the corpora cavernosa. [13] The Mitchell-Bagli procedure (complete penile disassembly) allows tubularization and ventralization of the entire distal urethra, thus making glans and urethral repair independent. Urethrocutaneous fistulas which our patient developed and was managed conservatively are among the most common complications after the modified Cantwell-Ransley procedure is performed. [13] A fistula rate of four percent and urethral stricture rate of 5.3%, respectively, was reported by Gearhart John Philip. [10]
In our patient, we elected to carry out the modified Cantwell-Ransley procedure based on surgeon's preference and expertise.
The delay in presentation and age at repair was a thing of concern to us in the index patient. Boys born with epispadias suffer poor self-image as they grow older and become aware of their genitalia. [6],[19],[20] Whereas Braga et al. reported mean patient age at epispadias surgery using the Cantwell-Ransley repair to be 16.8 months
(range: 12-24) in their series. [15] Our patient was brought to the hospital for the first time at the age of 25 years by a relative citing ignorance and poverty as reasons for the delay. Another series; however, reported complete penile disassembly in postpubertal patients with penopubic epispadias even though they all had previous failed repairs. [21] Similarly, Mansour et al. described the primary surgical management of five adult males with bladder exstrophy-epispadias complex at their center. [2],[3]
In most boys (up to 70%) with penopubic epispadias, the bones of the pelvis are widely separated. This affects the bladder neck and external sphincter leading to incontinence and constant or stress urinary dribbling, a problem our patient had. However, there was no abnormal widening of the pubic symphysis in our case. Therefore, we did not consider bladder neck reconstruction as being indicated. [10]
Our patient had a single stage repair which is favored by most contemporary authors. [22] This is easily desirable especially when urinary continence is achieved after the single stage repair. It further mitigates the problem of defaulting from the hospital which is prevalent in our environment when procedures are staged. Following the successful surgery our patient's self-image and confidence greatly improved. This is in keeping with the findings of Venkatramani et al. and Wittmeyer et al. on improvement in the quality of life of patients with exstrophy-epispadias in adulthood. [20],[23] Our patient is presently in a relationship with a female partner 2 years after surgery. This further underscores the importance of surgical repair regardless of late presentation. Reddy et al. in their assessment of sexual health and fertility outcomes in adults with complete male epispadias postreconstruction noted that 47% and 80% of their patients, respectively, were in a relationship and had overall satisfactory sexual intercourse. [1]
| Conclusion | | |
The management of epispadias could be challenging, especially in poor resource settings associated with ignorance and poverty. Nevertheless, appropriate surgical procedures, such as the modified Cantwell-Ransley repair in adulthood, offer the patient an improved quality of life.
Financial Support and Sponsorship
Nil.
Conflicts of Interest
There are no conflicts of interest.
| References | | |
| 1. | Reddy SS, Inouye BM, Anele UA, Abdelwahab M, Le B, Gearhart JP, et al. Sexual health outcomes in adults with complete male epispadias. J Urol 2015;194:1091-5.  |
| 2. | Mansour AM, Sarhan OM, Helmy TE, Awad B, Dawaba MS, Ghali AM. Management of bladder exstrophy epispadias complex in adults: Is abdominal closure possible without osteotomy? World J Urol 2010;28:199-204. |
| 3. | Perovic S, Scepanovic D, Sremcevic D, Vukadinovic V. Epispadias surgery - Belgrade experience. Br J Urol 1992;70:674-7. |
| 4. | Mokhless I, Youssif M, Ismail HR, Higazy H. Partial penile disassembly for isolated epispadias repair. Urology 2008;71:235-8. |
| 5. | Maitama HY, Ahmed M, Bello A, Mbibu HN. Epispadias with complete prepuce. Afr J Urol 2012;18:90-2. |
| 6. | Wood D, Woodhouse C. Penile anomalies in adolescence. ScientificWorldJournal 2011;11:614-23. |
| 7. | Perlin DV, Sapozhnikov AD, Darenkov SP, Efremov EA. Treatment of exstrophy of bladder in adults. Urologiia 2014;5:121-4. [ PUBMED] |
| 8. | Nerli RB, Kamat GV, Alur SS, Koura A, Prabha V, Amarkhed SS. Bladder exstrophy in adulthood. Indian J Urol 2008;24:164-8. [ PUBMED]  |
| 9. | Gearhart JP, Sciortino C, Ben-Chaim J, Peppas DS, Jeffs RD. The Cantwell-Ransley epispadias repair in exstrophy and epispadias: Lessons learned. Urology 1995;46:92-5. |
| 10. | Gearhart John Philip, Mathews RI. Exstrophy-epispadias complex. In: AJ, editor. Campbell-Walsh Urology. 10 th ed. Philadelphia: Elsevier Saunders; 2010. p. 3325-78. |
| 11. | Sujijantararat P, Chotivichit A. Surgical reconstruction of exstrophy-epispadias complex: Analysis of 13 patients. Int J Urol 2002;9:377-84. |
| 12. | Wiersma R. Overview of bladder exstrophy: A third world perspective. J Pediatr Surg 2008;43:1520-3. |
| 13. | Lodh B, Khumukcham S, Bernard Amer KA. Modified Cantwell-Ransley repair of male penopubic epispadias: Report of two cases and review of the literature. J Case Rep 2013;3:344-8. |
| 14. | Frimberger D. Diagnosis and management of epispadias. Semin Pediatr Surg 2011;20:85-90. |
| 15. | Braga LH, Lorenzo AJ, Bägli DJ, Khoury AE, Pippi Salle JL. Outcome analysis of isolated male epispadias: Single center experience with 33 cases. J Urol 2008;179:1107-12. |
| 16. | Grady RW, Mitchell ME. Management of epispadias. Urol Clin North Am 2002;29:349-60, vi. |
| 17. | Baird AD, Gearhart JP, Mathews RI. Applications of the modified Cantwell-Ransley epispadias repair in the exstrophy-epispadias complex. J Pediatr Urol 2005;1:331-6. |
| 18. | Borzi PA, Thomas DF. Cantwell-Ransley epispadias repair in male epispadias and bladder exstrophy. J Urol 1994;151:457-9. |
| 19. | VanderBrink BA, Stock JA, Hanna MK. Esthetic outcomes of genitoplasty in males born with bladder exstrophy and epispadias. J Urol 2007;178(4 Pt 2):1606-10. |
| 20. | Venkatramani V, Chandrasingh J, Devasia A, Kekre NS. Exstrophy-epispadias complex presenting in adulthood: A single-center review of presentation, management, and outcomes. Urology 2014;84:1243-7. |
| 21. | Hafez AT, Helmy T. Complete penile disassembly for epispadias repair in postpubertal patients. Urology 2011;78:1407-10. |
| 22. | Devine CJ Jr, Horton CE, Scarff JE Jr. Epispadias. Urol Clin North Am 1980;7:465-76. [ PUBMED] |
| 23. | Wittmeyer V, Aubry E, Liard-Zmuda A, Grise P, Ravasse P, Ricard J, et al. Quality of life in adults with bladder exstrophy-epispadias complex. J Urol 2010;184:2389-94. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
|
Search Pubmed for