Isolated male epispadias is a rare congenital malformation in which the urethral opening is situated on the dorsal side of the phallus with an open distal urethral plate and deficiency of dorsal penile skin. ⁽¹⁾ It is seen as a part of the bladder-exstrophy-epispadias complex more frequently. The phallus is short and wide, with an abnormal dorsal curvature. The urethra most often opens on the dorsal aspect the penis instead of the tip. The female has depressed mons, a bifid clitoris, and labia minora. The urethra is lying above vagina, short and widely open dorsally, communicating with an open bladder neck. Vagina and hymen are normal. The incidence of isolated male epispadias is approximately 1 in 1,17,000 males. Female epispadias occurs in 1 in 4,84,000 females. ^(2,3) Male to female ratio is 4:1. ^(3,4)

Epispadias in males is classified according to the location of the urethral meatus on the penis, which includes glanular epispadias where the meatus is located on the glans, penile epispadias where it is situated along the shaft, and penopubic epispadias where it is positioned near the pubic bone. ⁽⁴⁾

The bladder neck is also frequently involved. It is often wide and incompetent. This directly affects continence mechanism, bladder cycling and growth, and the ability to achieve urinary continence. In boys with epispadias, continence is possible if the epispadias is located distally and the bladder neck is usually formed. In girls, continence is affected to some degree because of the associated urethral and bladder neck ectasia. The primary treatment for isolated epispadias is a comprehensive surgical repair of the Genitourinary area, usually performed during the first 6 to 12 months of life. The most popular and successful technique is known as the modified Cantwell-Ransley approach. ⁽⁵⁾

Even with successful surgical repair, patients may have long-term problems like incontinence, cosmetic appearance of external genitalia, small phallus, sexual dysfunction, upper tract changes, depression, and psycho-social issues. The study aimed to evaluate the incidence and clinical profile of isolated epispadias, with particular focus on postoperative complications, cosmetic outcomes, continence status, upper urinary tract involvement, and the need for further surgical interventions.

Isolated male epispadias is a rare congenital malformation in which the urethral opening is situated on the dorsal side of the phallus with an open distal urethral plate and deficiency of dorsal penile skin. ⁽¹⁾ It is seen as a part of the bladder-exstrophy-epispadias complex more frequently. The phallus is short and wide, with an abnormal dorsal curvature. The urethra most often opens on the dorsal aspect the penis instead of the tip. The female has depressed mons, a bifid clitoris, and labia minora. The urethra is lying above vagina, short and widely open dorsally, communicating with an open bladder neck. Vagina and hymen are normal. The incidence of isolated male epispadias is approximately 1 in 1,17,000 males. Female epispadias occurs in 1 in 4,84,000 females. ^(2,3) Male to female ratio is 4:1. ^(3,4)

Epispadias in males is classified according to the location of the urethral meatus on the penis, which includes glanular epispadias where the meatus is located on the glans, penile epispadias where it is situated along the shaft, and penopubic epispadias where it is positioned near the pubic bone. ⁽⁴⁾

The bladder neck is also frequently involved. It is often wide and incompetent. This directly affects continence mechanism, bladder cycling and growth, and the ability to achieve urinary continence. In boys with epispadias, continence is possible if the epispadias is located distally and the bladder neck is usually formed. In girls, continence is affected to some degree because of the associated urethral and bladder neck ectasia. The primary treatment for isolated epispadias is a comprehensive surgical repair of the Genitourinary area, usually performed during the first 6 to 12 months of life. The most popular and successful technique is known as the modified Cantwell-Ransley approach. ⁽⁵⁾

Even with successful surgical repair, patients may have long-term problems like incontinence, cosmetic appearance of external genitalia, small phallus, sexual dysfunction, upper tract changes, depression, and psycho-social issues. The study aimed to evaluate the incidence and clinical profile of isolated epispadias, with particular focus on postoperative complications, cosmetic outcomes, continence status, upper urinary tract involvement, and the need for further surgical interventions.

This single-centre cohort comprised 22 individuals with isolated epispadias managed between October 2017 and February 2019, integrating both prospectively enrolled cases (n = 7) and retrospectively reviewed records (n = 15). Patients presented late—reflecting low socio-economic status—with a median age at surgery of 4.2 years; 36.4 % were operated on before three years, 27.3 % between three and five years, 31.8 % between five and ten years, and one child (4.5 %) after ten years (Table 1). Males predominated (19/22, 86.4 %), and overall follow-up averaged 18 months (range 1 month–2.5 years).

Table 1. Age at Surgery (n=22)

As shown in Table 2, seventeen children (77.3 %) exhibited peno-pubic epispadias, two (9.1 %) had penile lesions, and three (13.6 %) were female. Sixteen were primary repairs, whereas five had undergone prior surgery—three at this institution and two elsewhere—and one required staged bladder-neck reconstruction (BNR) for persistent incontinence. Bladder-neck repair was done in 17 patients (77%), and pubic-symphysis approximation with bilateral anterior iliac osteotomy was necessary in seven (32%) due to diastasis.

Table 2. Type of epispadias

Postoperative morbidity was acceptable: superficial suture-line infection developed in two children (9.1 %) and resolved with dressings, urethrocutaneous fistula occurred in five (22.7 %) and closed conservatively, two produced hypertrophic scars necessitating revision, and one formed an intravesical calculus removed cystoscopically. Continence outcomes were encouraging: seven patients (31.8 %) achieved dry intervals > 120 min, another seven (31.8 %) remained dry for 90–120 min, four (18.2 %) for 30–60 min, while four (18.2 %) dribbled continuously or < 30 min (Table 3).

Table 3: Continence outcomes (n=22)

As shown in Table 4, the functional bladder capacity exceeded 100 mL in two-thirds of the cohort, including five children (22.7%) who reached a capacity of more than 200 mL, with a maximum recorded capacity of 450 mL. Cosmetically, 14 children displayed inconspicuous scars; four had acceptable and four poor scars (two revised surgically, two keloids treated with intralesional steroid). Post-repair meatal position was orthotopic in 84.2 % of boys, sub-coronal in the remainder; significant chordee persisted in two, and torsion > 20° in three. Stretched penile length was ≤ 1.5 cm in three, 1.5–3 cm in fourteen, and > 3 cm in two; the four patients now older than 15 years reported satisfactory erections without deformity.

Table 4. Bladder capacity

Renal scintigraphy revealed upper-tract cortical defects in three children, and voiding cystourethrography showed unilateral grade II vesicoureteric reflux in six, all of whom were managed with chemoprophylaxis and surveillance. During the study period, four patients required redo urethroplasty, two underwent scar revision, and one had cystoscopic stone extraction, underscoring the necessity of ongoing multidisciplinary follow-up to optimise functional and cosmetic outcomes in this complex cohort.

In our study, the majority of patients were male, with 19 (86.36%) males and 3 (13.63%) females. Similarly, a prospective study conducted by Cervellione et al. (6) in 2015 on behalf of the European Society of Pediatric Urology (ESPU) investigated the incidence of the exstrophy-epispadias complex across Europe over 12 months. Among the 71 identified cases of primary epispadias, 66 patients (92.95%) were male and 5 (7.04%) were female, reflecting a comparable male predominance as observed in our cohort.

Cendron M et al.(7) conducted a study at the Department of Urology, Boston Children's Hospital, to evaluate the anatomical distribution of epispadias in boys, identifying 26 patients who were categorized into three types: peno-pubic in 14 cases (53%), penile in 8 (30%), and glanular in 4 (15%). In comparison, our study demonstrated a higher proportion of peno-pubic epispadias with 17 patients (77.27%), followed by 2 (9.09%) with penile epispadias, and none with the glanular form. Additionally, our series included three female patients (13.63%), who were not included in Cendron's study, indicating a broader inclusion in our cohort.

In our study, postoperative complications included suture line infection in 2 patients (9.09%), urethrocutaneous fistula in 5 patients (22%), poor scarring in 2 patients (9.09%), and bladder stone formation in 1 patient. In comparison, Braga LH et al.(8) retrospectively reviewed 33 male children who underwent primary epispadias repair and reported urethrocutaneous fistula in 5 patients (15.15%), meatal stenosis in 1 patient (3.03%), poor scar formation in 1 patient (3.03%), and residual dorsal chordee in 4 patients (11.12%). While the rate of fistula was comparable, our study revealed a slightly higher incidence of poor scars and an additional complication, namely bladder stone, which was not observed in Braga's cohort.

Continence remains the most critical parameter in assessing postoperative functional outcomes. In our study, seven patients (31%) achieved dry intervals exceeding 2 hours, and another 7 (31%) had dry intervals between 1.5 and 2 hours. Six patients (27.27%) had dry intervals of less than 1 hour, and two patients (9%) exhibited continuous dribbling. In comparison, Mouriquand PDE et al. (9) reported 13 patients (52%) with dry intervals greater than 2 hours, four patients (16%) with dry intervals between 1.5 and 2 hours, and only two patients (8%) with dry intervals of less than 1 hour. Continuous dribbling was observed in one patient (4%) in their cohort. Notably, four patients (16%) in their study underwent a Mitrofanoff procedure with clean intermittent catheterization (CIC), achieving dry intervals of more than 3 hours. Additionally, one patient was lost to follow-up. In the study by Baka-Jakubiak et al. (10), 33 patients (89%) achieved complete daytime dryness, although 15 reported occasional nocturnal incontinence. Partial daytime continence lasting approximately one hour was documented in 4 patients. These findings highlight the variability in continence outcomes across studies, which is likely influenced by surgical technique, bladder neck management, and patient-specific factors.

Braga L.H.P et al. study (n = 21) included only penopubic epispadias for calculating bladder capacity. (8) They reported bladder capacity between 50-100 ml in 6 (28.57%) patients, 100-200 ml in 8 (38.09%) patients, and more than 200 ml in 7 (33.33%) patients. We found(n=22) bladder capacity less than 50 ml in 3 (13%) patients, between 50-100 ml in 5 (22%) patients, 100-200 ml in 9 (40%) patients, and more than 200 ml in 5 (22%) patients.

In terms of cosmetic outcomes, four patients (18%) in our study developed poor postoperative scars, while the remaining 18 patients (82%) had either good or acceptable scar appearance. Among male patients, the urethral meatus was located at the tip of the penis in 16 cases (84.21%), whereas three patients (15.78%) had a subcoronal meatus. A total of 15 patients had no evidence of chordee or torsion postoperatively; however, four exhibited mild to severe chordee, and three had significant penile torsion exceeding 20 degrees. Overall, 70–80% of patients achieved an excellent cosmetic result. In comparison, Bhat A et al.(11) conducted a retrospective analysis of 43 primary epispadias cases operated between July 1998 and March 2013, in which two patients (8%) with penopubic epispadias had residual chordee, and only one patient (4%) developed mild torsion. Notably, 21 of 24 patients (88%) in their cohort with penopubic epispadias achieved excellent cosmetic results. Unlike our study, penile length was not assessed in their series, and none of their patients experienced postoperative wound infection, fistula, scar complications, or bladder stone formation.

On postoperative follow-up, six patients (28.57%) in our cohort demonstrated low-grade unilateral vesicoureteric reflux (VUR) on micturating cystourethrogram, and three patients (14.28%) exhibited unilateral cortical scarring on DMSA renal scintigraphy. All affected individuals were managed conservatively with continuous urinary prophylaxis and regular monitoring. Overall, upper tract abnormalities were observed in 14.28% of patients; however, none developed recurrent urinary tract infections during the follow-up period. In contrast, the study by Ben-Chaim et al. (12) reported no evidence of upper urinary tract damage or renal impairment following epispadias repair, highlighting the variability in long-term renal outcomes across different cohorts.

Epispadias is commonly associated with exstrophy of the bladder, forming the exstrophy-epispadias complex. Isolated epispadias is a very rare congenital anomaly. Surgeons need to demonstrate a long-term commitment to these patients. There are many methods of epispadias repair, out of which the modified Cantwell-Ransley repair and Mitchell’s repair are widely used and accepted worldwide. Based on our past experiences and outcomes, we have made modifications to the Modified Cantwell-Ransley technique for the repair of epispadias. In older children with wide pubic diastasis (greater than 4 cm), osteotomy is required. We have found that, with our modification of the standardized technique, the continent rate is improved, yielding a good cosmetic outcome, while preserving the upper tract and having a lower complication rate.

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