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Urogenital Reconstruction, Penile Epispadias: Treatment
Updated: Jun 23, 2008
Treatment
Medical Therapy
No medical treatment corrects epispadias.
Surgical Therapy
Most of the repair techniques are based on multistaged reconstruction procedures used in hypospadias correction.
The first stage addresses correction of the eventually stenotic meatus, the second stage addresses elimination of the dorsal chordee and penile straightening, and the third stage addresses the urethroplasty.
Together with the progress in surgical treatment of hypospadias, one-stage procedures have also been used to correct epispadias.
The goals of surgical procedures are (1) correction of the curvature, (2) reconstruction of the missing portion of urethra, (3) restoration of the normal aspect of the external genitalia, and (4) reconstruction of the anterior wall of the bladder when necessary. Surgery differs according to the complexity of the malformation.
Epispadias is treated with the same technique as hypospadias located in the same anatomic position, with the exception that the repair is reversed and the epispadias reconstruction is on the dorsal surface.
Continence is a difficult goal in exstrophy-epispadias complex repair. Presumably, all anatomic components involved in the exstrophy-epispadias abnormality are present but are displaced laterally and anteriorly. A staged approach has often been used for the management of the exstrophy-epispadias complex. The Cantwell-Ransley repair and the Young procedure have also been used.1,2,3
Single-stage procedure
In glandular epispadias with a straight penis, local flaps based from the glans are often used to reconstruct the missing distal urethra (see Image 4). In penile malformation, accomplish a suprapubic urinary diversion before degloving the penile skin at the level of the Buck fascia.
Isolate the external urethral meatus, and resect it together with the most distal hypoplastic portion of the urethra. Induce an artificial erection with an intracavernous injection of saline solution to assess for the presence and the degree of curvature. Perform an extensive dorsal chordectomy and straightening of the penile shaft when needed.
According to the urethral defect and age-related urethral size, tailor a penile-preputial flap that was harvested longitudinally from the ventral penile and preputial skin (see Image 5). Stent the neourethra with a 12- or 14-Ch (Charrière) silicone catheter for 48 hours.
Apply a mild compressive bandage. When possible, the glandular urethra can be reconstructed using local flaps from the glans. At the end of the procedure, the penis has a circumcised appearance.
Intraoperative Details
After penile degloving, induce an artificial erection with an intracavernous injection of saline solution to assess for the presence and the degree of curvature. Perform cordectomy and straightening of the penile shaft if required. At this stage, the actual urethral defect shows, and the urethral reconstruction is planned.
Harvest a penile-preputial flap with the preferred technique, taking into consideration the age-related urethral size. Inset the flap to the proximal urethral stump, and tube it around a silicone catheter (see Images 6-7). Split the glans and elevate local flaps to cover the distal part of the neourethra that reaches the tip of the glans (see Image 8). Remove the redundant foreskin. Pull up the penile skin and suture it to the corona to achieve a final appearance similar to that of a circumcised penis (see Images 9-10).
Postoperative Details
Arm and leg restraints may be necessary. The dressings remain in place for 4 days if no problems occur. Remove the diverting urinary catheter after 8-10 days. Discharge the patient after removal of the urinary diversion and when spontaneous voiding occurs without difficulty.
Follow-up
Monitor patients with a flow rate study at 3 weeks and at 3 and 12 months postoperatively. If a mild urethral stenosis is noticed, dilate the urethra with probes of increasing sizes and repeat the flow rate study after 3 weeks. Question patients about persistence of the curvature 3 weeks postoperatively.
Long-term follow-up care is necessary at least through puberty to exclude late failure due to hypertrophic urethral scarring or undetected chronic inflammation.
Complications
Early complications include bleeding, infection, wound separation, flap necrosis, and edema. Urethrocutaneous fistula with urinary leakage from the new urethra and urethral stricture occur in approximately 10-19% of all epispadias repairs.
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References
Ben-Chaim J, Peppas DS, Jeffs RD, Gearhart JP. Complete male epispadias: genital reconstruction and achieving continence. J Urol. May 1995;153(5):1665-7. [Medline].
Mollard P, Basset T, Mure PY. Male epispadias: experience with 45 cases. J Urol. Jul 1998;160(1):55-9. [Medline].
Surer I, Baker LA, Jeffs RD, Gearhart JP. The modified Cantwell-Ransley repair for exstrophy and epispadias: 10- year experience. J Urol. Sep 2000;164(3 Pt 2):1040-2; discussion 1042-3. [Medline].
Kramer SA, Mesrobian HG, Kelalis PP. Long-term followup of cosmetic appearance and genital function in male epispadias: review of 70 patients. J Urol. Mar 1986;135(3):543-7. [Medline].
Caione P, Capozza N, Lais A, Matarazzo E. Periurethral muscle complex reassembly for exstrophy-epispadias repair. J Urol. Dec 2000;164(6):2062-6. [Medline].
Diamond DA, Ransley PG. Improved glanuloplasty in epispadias repair: technical aspects. J Urol. Oct 1994;152(4):1243-5. [Medline].
Gearhart JP. Re: results of complete penile disassembly for epispadias repair in 42 patients. J Urol. Jun 2004;171(6 Pt 1):2386; author reply 2386. [Medline].
Grossman JA, Caldamone A, Khouri R, Kenna DM. Cutaneous blood supply of the penis. Plast Reconstr Surg. Feb 1989;83(2):213-6. [Medline].
Horton CE, Sadove RC, Jordan GH, Sagher U. Use of the rectus abdominis muscle and fascia flap in reconstruction of epispadias/exstrophy. Clin Plast Surg. Jul 1988;15(3):393-7. [Medline].
Patten BM, Barry A. The genesis of extrophy of the bladder and epispadias. Am J Anat. 1952;90:35.
Santanelli F, Fogdestam I. Use of a vertical island flap for correction of epispadias. Ann Plast Surg. Oct 1991;27(4):345-50. [Medline].
Further Reading
Keywords
congenital malformation, genitalia malformation, genital malformation, penis malformation, deformed penis, penis repair, penis reconstruction, penile repair, penile surgery, penis, epispadias, epispadias-exstrophy of the bladder, bladder epispadias-exstrophy, bladder epispadias, bladder exstrophy, penile exstrophy, penis epispadias, penis exstrophy, impotentia coeundi, impotentia generandi, penis curvature, penile curvature, glanuloplasty, epispadiac penis, chordee
Treatment: Urogenital Reconstruction, Penile Epispadias