Penile Epispadias Reconstruction 

Updated: Feb 19, 2020
Author: Fabio Santanelli di Pompeo, MD, PhD; Chief Editor: Jorge I de la Torre, MD, FACS 

Overview

Background

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.[1, 2, 3]

Problem

Epispadias 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 first image below) to complete defects as are observed in bladder exstrophy, diastasis of the pubic bones, or both (see second image below). Simple epispadias occurs less commonly than the more severe form associated with exstrophy of the bladder.

Distal penile epispadias. Distal penile epispadias.
Wide diastasis of the pubic bone and external disp Wide diastasis of the pubic bone and external displacement of the hips in epispadias.

Epidemiology

Frequency

Epispadias 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.

Etiology

Unlike 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.

Pathophysiology

In 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.

Presentation

The 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 following image).

Distal epispadias. Outlining of local flaps from t Distal epispadias. Outlining of local flaps from the glans to reconstruct the distal urethra.

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.

Indications

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.

The complete (penopubic) form of the malformation is treated early in childhood to close the abdominal wall and the bladder exstrophy.

Relevant Anatomy

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.

The vascular axial pattern of the skin-dartos complex originating from the external pudendal vessels is present, but it is dislocated on the ventral aspect of the penile shaft. Additional vascular supply to the prepuce comes from collateral vessels of the terminal glanular branches of the dorsal penile artery. The Buck fascia surrounds the corpora cavernosa and splits to encircle the neurovascular bundle.[4]

Contraindications

Urethroplasty and restoring the normal appearance of the genitalia are contraindicated in infancy because of the small size of the structures.

 

Workup

Laboratory Studies

See the list below:

  • Obtain a complete blood cell count.

Imaging Studies

See the list below:

  • Obtain a chest radiograph if indicated based on examination findings or the patient's history.

  • Radiologic (eg, intravenous pyelogram) and ultrasound evaluations of the urogenital system (eg, kidneys, ureters, bladder; urethra, genitalia) are usually necessary to evaluate the extent of the defect. (For more information on bedside ultrasonography of the kidneys, see Medscape Reference article Obstructive Uropathy Assessment using Bedside Ultrasonography.)

Other Tests

See the list below:

  • Obtain an ECG for operating room guidelines.

 

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.[5, 6, 7] Partial penile disassembly of the corporal bodies, leaving the urethral plate attached to preserve the blood supply, with their attachments extended down to the horizontal branches of the pubic bones, has been successfully used for primary or secondary repairs.[8, 9, 10]

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 following image). In penile malformation, accomplish a suprapubic urinary diversion before degloving the penile skin at the level of the Buck fascia.

Reconstruction of distal penile urethra using loca Reconstruction of distal penile urethra using local flaps (arrows).

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 following image). Stent the neourethra with a 12- or 14-Ch (Charrière) silicone catheter for 48 hours.

Vertical island flap drawn on the ventral aspect o Vertical island flap drawn on the ventral aspect of the penis.

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 following images).

Island flap transferred dorsally and anastomosed t Island flap transferred dorsally and anastomosed to the urethra.
Island flap sutured into a tube to reconstruct the Island flap sutured into a tube to reconstruct the missing portion of the urethra.

Split the glans and elevate local flaps to cover the distal part of the neourethra that reaches the tip of the glans (see following image).

Urethral reconstruction is completed. Urethral reconstruction is completed.

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 following images).

Drawing of the final appearance at the end of the Drawing of the final appearance at the end of the operation.
Photograph of the final appearance at the end of t Photograph of the final appearance at the end of the operation.

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.[11]

Outcome and Prognosis

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.[5, 12] Successful reconstruction of epispadias improves the body image and enables the patient to perform sexually.[13]

A study by Kertai et al indicated that in patients with bony pelvic abnormalities associated with exstrophy-epispadias complex, those who as newborns undergo symphyseal approximation without osteotomy do not suffer impairment of long-term hip function. The study included 17 postpubescent patients (14 male; 3 female) who had undergone the procedure in early childhood. The great majority of patients demonstrated no dysplastic or degenerative hip disease on follow-up.[14]

In a study of male patients who underwent reconstruction for epispadias, Reddy et al reported that these individuals were able to engage in postoperative intercourse and impregnate a sexual partner. Twelve of the study’s 15 patients stated that the quality of sexual intercourse was satisfactory overall, although 11 patients experienced at least one problem with sexual function, such as abnormal ejaculation, reduced sensation, or difficulty maintaining an erection. Five patients reported that they had impregnated a partner.[15]

Future and Controversies

Recent great interest is in developing engineered tissues from autologous materials, such as mature bladder cells, bone marrow, or adipocytes derived stem cells.[16] Ideally, regenerative medicine has the potential to preserve the normal function of the organ it is replacing or augmenting. These studies are still in the early stages with regard to urologic tract malformations.[17]