Male epispadias, a rare congenital dislocation of the urethral meatus on the dorsum of the penis, is of the mildest form of a broad spectrum of urogenital anomalies known as bladder-exstrophy-epispadias complex (BEEC) [1]. Although the etiology of this entity is not completely understood, disruption of mesenchymal migration due to the early invasion of the cloacal membrane around five weeks of gestation may proceed to the development of such an anomaly [2]. The scarcity of epispadias is reported as 1 in 117,000 male live births [3]. However, given that the glandular epispadias with obscured meatus beneath the prepuce is not well diagnosed, the prevalence seems to be higher [4]. Penopubic epispadias (PPE) is the most common as well as the most severe form of epispadias. Lack of urethral tubularization and failure in urethral plate closure leads to development of a broad mucosal strip on the penile dorsum and exposure of a patulous bladder neck (BN) and posterior urethra, along with sphincter incompetence in penopubic type. This variant is also associated with variable degrees of urinary incontinence (up to 70%), penile dorsal curvature, and an unpleasant appearance [1, 4, 5].

The concerns regarding the management of isolated PPE have recently shifted to improving the health-related quality of life. Lack of urinary continence, dissatisfaction with genitalia, and impaired sexual function are essential factors in such patients, increasing the risk of psychosocial harm, sexual health disturbance, impairment of body perception, and mental disorders. Therefore, achieving volitional voiding ability, reconstructing a cosmetically acceptable penis, and maintaining sexual function are paramount [6,7,8]. Although surgical approaches have made considerable progress with promising outcomes in recent years, restoring continence has remained a major concern in these patients. Moreover, multi-stage surgeries and application of bladder neck reconstruction (BNR) are accompanied with conflicting results and high morbidity [8,9,10,11].

Herein, we represent the long-term outcomes of our single-surgeon single-center experience with modified surgical technique as a novel approach for the management of boys with isolated PPE. A technique in which we do not open the bladder neither for bladder neck plication nor for urethral reimplantation.

This study is approved by the Committee of Human Research and the Institutional Review Board at our university and all methods were carried out in accordance with the declaration of Helsinky. After explaining all the potential risks and benefits of this technique and the possible post-surgical harms, informed consent was obtained from the legal guardians or parents of the included children. Our BEEC database was reviewed for the medical records of boys with PPE. Patients who underwent our current modified surgical approach were included, and those who had other anomalies in the genitourinary system, or those with previous unsuccessful surgery, were excluded from this study. All patients underwent a thorough physical examination along with the assessment of incontinence severity. Subsequently, voiding cystourethrography (VCUG); for presence of reflux and to estimate the bladder capacity, ultrasonography, urine analysis, urine culture, and 3D-CT scan of pelvic bone were performed for each child in order to depict 3 dimensional pelvic bone anatomy and measure all bony angles and bone dimensions and bone distances of pelvis. Post-operative continence status was categorized based on a previously defined classification. Briefly, totally continent patients (Grade 0) were considered as those who were totally dry at night with dry intervals of ≥ 6 h during the day, and considered as occasionally wet (Grade 1) if they used pads for at least once a week or had infrequent episodes of incontinence. Lastly, those with dry intervals of < 3 h and those with no dry period was considered as frequently wet (Grade 2), and totally incontinent (Grade 3), respectively. Moreover, children who had dry intervals of ≥ 3 h during the day were considered socially dry patients (grade 0 and 1).

Following the general anesthesia, cystoscopy was performed in all children, evaluating the BN configuration, sphincter competency, and ureteral orifices. After that, using polypropylene 4/0, two traction sutures were placed on both sides of the glans to circumscribe the urethral plate and underlying corpus spongiosum (urethral wedge) margins. The reverse MAGPI (meatal advancement and glanuloplasty, IPGAM) incision was done at the distal glandular end of the urethral wedge. After defining the urethral wedge boundaries with a surgical marker, an inverted U-shaped incision was made along the lateral margins of the urethral wedge, starting from the distal portion, continued up to the BN. Circumcision incision was performed on the penile ventral aspect, confluence the U-shaped incision of the urethra on the dorsum. By avoiding complete penile degloving, the skin was dissected off the penis with mesothelial sparing at the median and paramedian area where the corpus spongiosum is located, and the buck's fascia has just stopped at its edges. The main reason was to preserve a greater blood supply to the corpus spongiosum and the adjacent structures, as it is the main source of blood supply to the urethra, precipitating the healing process and preventing further tissue hypoxia. The urethral wedge was proximally dissected off the corpora cavernosa up to the BN and further to the pubic bone rami. It bears mentioning that complete preservation of the corpus spongiosum should be performed carefully. Laterally dislocated neurovascular bundles (NVBs) were meticulously dissected off the corporal bodies. The corporal bodies were separated distally up to the glans, except for the distal 1 cm that was kept attached. A total of five vascular tapes were applied to isolate the structures at each separation step, two for corporal bodies, two for NVBs, and one for the urethral wedge. Skin and the underlying tissues were incised in a Z-like fashion at the suprapubic area to expose the BN and verumontanum. BN was plicated, avoiding any neck or bladder mucosa injury, following Kelly’s plication technique, using non-absorbable sutures, which led to the inversion of the mucosa back to the bladder. According to a previously published article, non-absorbable sutures are superior to absorbable ones, with seemingly better outcomes. A 10 Fr urethral catheter was placed at the BN and the posterior urethra. Tubularization started from BN down to the verumontanum. The bladder was then fulfilled with normal saline to evaluate the BN competency by manually compressing the bladder after removing the catheter. Bladder neck plication (BNP) was considered successful when the urine flowed following compression and stopped after decompression. The key point was avoiding mucosal disturbance and assessment of BN resistance following the plication. The catheter was replaced, two layers, including the urethral plate and underlying corpus spongiosum were tubularized around the 10 Fr catheter up to the distal 2 cm of the glans using 6-0 running sutures. Tubularization was continued up to the meatus. Excessive atypical skin of the glans wings was trimmed off. A normal conical glandular appearance and a ventrally located meatus were achieved following a two-layered closure of the glans wings using 7-0 PDS® (polydioxanone) sutures during the glanuloplasty procedure. Repair of the chordee was performed with interrupted suturing of the reapproximated medially rotated corporal bodies at the dorsal apex, leading to placement of the neo-urethra on the ventral aspect, chordee correction, and placing the NVBs to their original anatomic location. The appearance of the glans was assessed from now on; if it got pale, the blood supply sufficiency was examined by using a diabetic lancet device on the glans. In case of an insufficient blood supply, sutures of the corporal bodies were replaced with looser stitches. Two wide-based long fat pad pedicled flaps were taken from the subcutaneous fats that lies on the rectus muscles sheath. Each side's pedicled fat pad slings passed over the contralateral corporal body cross wisely and connected to the corpus spongiosum on the ventral aspect integrating the three main structures of the penis in a ring fashion at the penile base level with the aim of mimicking the function of an external sphincter. Besides, it is worth noting that the external sphincter plate, located at the distal end of the verumontanum, was intensely protected from invasion, traumatization, and devascularization during each step. So far, the BN is plicated, the corporal bodies are sutured together, and both are fixed in a pedicled fat pad ring along with the ventrally located neo-urethra. The 10 Fr urethral catheter was then removed; following a successful manual examination of the urine flow, neo-urethra was re-catheterized using 8 Fr. urethral catheter, fixed to the glans with two sutures. Recatheterization was performed to examine the competency of the neo-urethra as well as reducing the risk of urethral ischemia regarding the existing peritubular tissue edema. Therefore, three main factors were evaluated during the surgery, the competence of the neo-urethra that was evaluated with recatheterization, BN competency that was evaluated following manual compression after decatheterization, and the ischemic state and blood sufficiency of the penis that was evaluated by using a diabetic lancet device. Finally, the skin was reconfigured using Z-plasty (Fig. 1).

Surgical technique; a, b Z-like incision at suprapubic to expose bladder neck and verumontanum, c bladder neck plication, d isolation of corporal bodies using vascular tapes, e manual compression of bladder to evaluate bladder neck competency, f recatheterization using a smaller catheter, g skin closure and catheter fixation, h after dressing

Patients were discharged five days after surgery with antibiotic prophylaxis, and the urinary catheter was removed on the 14th post-operative day. Thereafter, a comprehensive evaluation at each visit, including continence status, development of possible complications, clinical examination, and genitalia size, were performed at intervals of quarterly in the first year and once a year in subsequent years. Moreover, uroflowmetry was performed at 1, 6, and 18 months after the surgery. Besides, genitourinary ultrasonography, VCUG, and urine analysis were performed at intervals of 6 months for the first year and annually thereafter. Patients' sexual health, including erectile function, quality of ejaculation, penile size, and dorsal curvature, were also assessed as soon as they reached puberty.

For data analysis, data with P < 0.05 was considered statistically significant. The paired t-test was used to evaluate the changes that occurred in bladder capacity during the follow-up intervals.