You are in: eMedicine Specialties > Plastic Surgery > TRUNK Urogenital Reconstruction, Penile EpispadiasArticle Last Updated: Jun 23, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 11
  Author: Fabio Santanelli, MD, PhD, Associate Professor of Plastic Surgery, University of Rome; Chief of Unita Operativa Dipartimentale di Chirurgia Plastica, Azienda Ospedaliera, Sant'Andrea, Rome Fabio Santanelli is a member of the following medical societies: American Society of Plastic and Reconstructive Surgery, European Association of Plastic Surgeons, and International Confederation for Plastic and Reconstructive Surgery Coauthor(s): Francesca Romana Grippaudo, MD, Assistant Professor, Unit of Plastic Surgery, 2nd Medical Faculty, Sapienza University of Rome, Italy Editors: Dennis P Orgill, MD, PhD, Associate Professor, Harvard Medical School; Director, Burn Center, Brigham and Women's Hospital; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Jorge I de la Torre, MD, FACS, Professor of Surgery and Physical Medicine and Rehabilitation, Residency Program Director, Division of Plastic Surgery, University of Alabama at Birmingham; Director, Center for Advanced Surgical Aesthetics; Nicolas (Nick) G Slenkovich, MD, Practice Director, Colorado Plastic Surgery Center at Swedish Medical Center; Jorge I de la Torre, MD, FACS, Professor of Surgery and Physical Medicine and Rehabilitation, Residency Program Director, Division of Plastic Surgery, University of Alabama at Birmingham; Director, Center for Advanced Surgical Aesthetics Author and Editor Disclosure Synonyms and related 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 INTRODUCTIONSection 2 of 11
  Congenital malformations of the male genitalia include a wide range of clinical conditions such as hypospadias, epispadias (in females, bifid clitoris) with bladder exstrophy, and hidden penis. Epispadias is an uncommon congenital malformation of the penis and is often part of the condition termed epispadias-exstrophy of the bladder. ProblemEpispadias is a rare congenital malformation of the male or female urogenital apparatus that consists of a defect of the dorsal wall of the urethra. The extent of the defect can vary from a mild glandular defect (see Image 1) to complete defects as are observed in bladder exstrophy, diastasis of the pubic bones, or both (see Image 2). Simple epispadias occurs less commonly than the more severe form associated with exstrophy of the bladder. FrequencyEpispadias occurs more commonly in males than in females, with a prevalence of 1 case in 10,000-50,000 persons. The male-to-female ratio is 2.3:1. EtiologyUnlike hypospadias, epispadias can be explained by defective migration of the paired primordia of the genital tubercle that fuse on the midline to form the genital tubercle at the fifth week of embryologic development. Epispadias and exstrophy of the bladder are considered varying degrees of a single disorder. Another hypothesis relates the defect to the abnormal development of the cloacal membrane. Epispadias is rarely observed in 2 members of the same family. PathophysiologyIn males, epispadias causes impotentia coeundi, which results from the dorsal curvature of the penile shaft, and impotentia generandi, which results from the incomplete urethra. Also reported are frequent ascending infections to the prostate or bladder and kidneys and psychological problems related to the deformity. If epispadias is distal to the bladder neck, urinary continence may not be present. ClinicalThe deformity manifests in males. The normal urethra is replaced by a broad mucosal strip lying on the dorsum of the corpora cavernosa; the meatus is divided dorsally between the tip of the glans and the pubis, the penile shaft is curved dorsally with the absence of the preputial apron, and a cleft is present on the upper surface of the penis (see Image 3). Epispadias vary in severity according to the time of the pathognomic insult during embryologic development and can be classified as glandular, penile, or complete (ie, penopubic). With the glandular type, the malformation affects the distal part of the urethra. With the penile type, the entire penile urethra is affected, with an external meatus on the dorsal shaft of the penis. With the complete or penopubic type, a total deficiency of the dorsal wall of the urethra and the anterior wall of the bladder is present. The glans is often spatulated, and the prepuce is clefted dorsally with ventral transposition. All forms of epispadias are associated with chordee. The extent of chordee varies. In females, epispadias consists of bifid clitoris with diastases of the corpora cavernosa, flattening of the mons, and separation of the labia. Associated defects are usually limited to the genital tract and diastases of the pubic bones. In exstrophy of the bladder, the lower abdominal wall is absent, with diastasis of the rectus abdominis muscle. Reflux develops in approximately 40% of patients. INDICATIONSSection 3 of 11
Correction of glandular epispadias with reposition of the distal urethra and creation of a symmetric glans (glanuloplasty) is indicated in childhood or adolescence at the patient's request for cosmetic or psychological reasons. Penile epispadias is corrected in childhood with penile straightening by resection of the chordee and creation of a new urethra of adequate caliber and length (urethroplasty). In females, bifid clitoris and normal genitalia appearance can be restored during adolescence. The aim is to permit normal voiding and erection and to avoid urinary tract infections. Click here to complete a Medscape activity on approaches to urinary tract infection and voiding dysfunction in children. The complete (penopubic) form of the malformation is treated early in childhood to close the abdominal wall and the bladder exstrophy. RELEVANT ANATOMYSection 4 of 11
Normally, the male urethra runs through the penile shaft, ventrally to the corpora cavernosa, and meets with the meatus at the tip of the glans. The penis is formed by the corpus spongiosum surrounding the urethra and by 2 corpora cavernosa; these structures are composed of erectile tissue surrounded by the tunica albuginea (Buck fascia) and the dartos fascia more superficially, which contains terminal branches of external pudendal arteries and veins and the superficial lymphatics. CONTRAINDICATIONSSection 5 of 11
Urethroplasty and restoring the normal appearance of the genitalia are contraindicated in infancy because of the small size of the structures. WORKUPSection 6 of 11
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TREATMENTSection 7 of 11
Medical TherapyNo medical treatment corrects epispadias. Surgical TherapyMost 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 DetailsAfter 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 DetailsArm 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-upMonitor 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. COMPLICATIONSSection 8 of 11
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. OUTCOME AND PROGNOSISSection 9 of 11
In terms of overall success rates, the incidence of fistulas or stenosis, and the mean hospitalization time, functional results obtained with one-stage epispadias correction have proved to be superior to those obtained with multistage procedures; the prognosis is good. For one-stage reconstruction, the use of a well-nourished and innervated flap with a long and pliable pedicle is necessary to achieve the high success rate reported in the literature. Surgical repair generally results in both continence (ability to control the flow of urine) and a good cosmetic outcome.1 Successful reconstruction of epispadias improves the body image and enables the patient to perform sexually.4 MULTIMEDIASection 10 of 11
REFERENCESSection 11 of 11
Urogenital Reconstruction, Penile Epispadias excerpt Article Last Updated: Jun 23, 2008 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
