Epispadias is a rare congenital disorder characterized by an abnormal dorsal opening of the urethra due to the failure of the urethral plate to tubularize. It is usually part of broader conditions like bladder or cloacal exstrophy and rarely occurs in isolation. We present the case of a 14-year-old girl with isolated epispadias who had lifelong urinary incontinence without urgency. Genital examination revealed a bifid clitoris, underdeveloped labia minora, and a widely open urethral meatus with continuous leakage of urine. Urethrocystoscopy showed a short, patulous urethra, a poorly coapting external sphincter, and an open bladder neck. She underwent a successful single-stage surgical repair consisting of urethroplasty, bladder neck reconstruction, and perineoplasty. Two weeks after surgery, following catheter removal, the patient achieved urinary continence. This case supports the effectiveness of single-stage reconstruction even in delayed presentations of isolated female epispadias.

Isolated male epispadias is a rare congenital condition where the urethral opening is abnormally on the upper penis side. Its severity varies, affecting continence and often requiring surgery. Outcomes depend on severity, voiding problems, and the surgeon's expertise. Two boys, aged 7 and 9, each presented with abnormal urethral opening on the dorsum of the penis, with abnormal urinary stream (bent upwards), without a history of prior surgeries, which was consistent with isolated epispadias. Clinical evaluations revealed a subcoronal meatus on the dorsum of the penis for the first case, and the coronal meatus for the second. The preoperative workups were normal, and there was no pubic symphysis diastasis for either case, and both were continent. The surgical repair involved urethral plate tubulization, neourethra ventralization, glanuloplasty, and circumcision using the modified Cantwell-Ransley technique. Postoperative outcome was successful, with good functional and cosmetic results. About 90% of congenital anomalies occur in low- and middle-income countries, including Tanzania, where urethral anomalies affect 24% of children and 0.5% present with epispadias. Diagnosis relies on clinical evaluation, while management involves surgical correction, most effectively using the modified Cantwell-Ranseley technique, which preserves urethral blood supply and achieves excellent functional outcomes. Early diagnosis and surgical repair of isolated male epispadias, even in resource-limited settings, can result in excellent functional and cosmetic outcomes using standard reconstructive techniques.

Isolated male epispadias (IME) presents as an abnormally displaced dorsal opening of the urethral meatus. It occurs in 1:120,000 live male births. Like all pathologies within the Bladder Exstrophy-Epispadias Complex (BEEC), this condition is associated with varying degrees of a widened pubic diastasis. This study sought to investigate the correlation between width of diastasis and continence outcomes. An IRB-approved, prospectively maintained, single-institutional BEEC database was utilized to identify male patients with isolated epispadias. Electronic medical records were reviewed for data pertaining to patient demographics, their original epispadias revision surgeries, and continence procedures and outcomes. Width of pubic diastasis was recorded through measurements obtained in radiographic imaging. Continence was described in terms of social continence or >3 h of daytime dry intervals between voids. These factors were assessed for their impact on continence outcomes. Of the 144 male epispadias cases reported in the database, 61 patients with complete data were identified. Of the 61 patients, 31 (51 %) achieved social continence while the remainder are incontinent. Mean width of pubic diastasis was relatively shorter in the continent group (2.00 cm, SD 1.18 cm vs 2.54 cm, SD 1.02 cm) when compared to the incontinent cohort (p = 0.0606). 15 of 31 continent patients did not require additional surgery to achieve continence while the remainder received 1-3 surgeries to achieve dryness. The 16 that required surgery had a significantly wider pubic diastasis (2.8 cm, SD 0.98 vs 1.17 cm, SD 0.69 cm) when compared to the nonsurgical group (p < 0.001). Of the 30 incontinent patients, 22 of them have not received additional continence surgery while 8 have had 1-2 surgeries. There was no significant difference (p = 0,0724) in diastasis width between those who received surgery and those who did not (2.54, SD 0.71 cm vs 2.54, SD 1.13 cm). This is the first study to investigate the relationship between pubic diastasis with continence outcomes and suggest that width of pubic diastasis can play a role in the surgical management of achieving continence in the IME population. These findings can assist in clinical decision making when considering the need for continence surgery in these patients, while also helping to manage patient and parent expectations.

Isolated female epispadias is a rare congenital anomaly. We report on sub-symphseal repair of this anomaly. Our study was an ambidirectional observational cohort one with minimum 6 months post operative evaluation. Surgical approach involved creation of paraurethral skin flap to lengthen the urethra and bladder neck (BN) plication under cystoscopic guidance. Second layer cover of the urethral tube using bilateral Maritus labial flap was performed. Of the 7 patients, 6 were fully continent. Our approach is effective for gaining both urinary continence and cosmesis issues.

Isolated male epispadias is one of the most severe congenital genital anomalies that require surgical correction. The goals of the surgery are to reach good aesthetic and functional outcomes. The aim of this retrospective study was to analyze the long-term outcomes of surgical reconstruction of male epispadias. A total of 31 patients with a mean age of 17 years, who underwent surgical repair of isolated male epispadias from January 2000 to January 2015, were involved. The main outcome measures were defined as: aesthetic outcome, continence, postoperative complications, sexual function, and quality of life. The follow-up period ranged from 8 to 23 years, with an average of 14.4 years. Each patients underwent an average of 2.2 surgical procedures in this period. The most common postoperative complications were urethral fistula and residual curvature, in 22.6% and 12.9%, respectively. Satisfactory aesthetic outcome was reported in 71.4% of cases. The repair of male epispadias usually includes more than two procedures with satisfactory aesthetic outcome. Unsolved urinary incontinence remains a significant issue and has a high impact on the quality of life. Follow-up should be extended even after complete sexual maturity. Comprehensive long-term evaluation is necessary for proper treatment of isolated epispadias. Epispadias is a rare urogenital malformation characterized by the failure of the urethral tube to form fully. Unlike hypospadias, where the urethra is complete but emerges proximal to the tip on the ventral aspect of the penis, epispadias features an open and exposed urethral plate on the penile dorsum. Epispadias is part of the exstrophy-epispadias complex (EEC), which comprises a range of genitourinary congenital deformities ranging from relatively minor epispadias to complete bladder or cloacal exstrophy. Males with epispadias have characteristic anatomic abnormalities, including a short, upward-pointing phallus with a meatus located in variable positions along the dorsal aspect of the penis and ventral hooding of the prepuce. Female epispadias is even more uncommon, with an incidence ranging from 1 in 160,000 to 1 in 480,000 live births. Young females with epispadias will have a bifid clitoris, patulous urethral meatus, an anteriorly placed vaginal opening, and an ill-formed or absent mons pubis. Isolated epispadias is a rare spontaneous urogenital defect that occurs more commonly in boys but can affect both sexes, and surgical intervention is essential to restore urinary continence and cosmesis. The diagnosis of epispadias is clinical and does not require any additional investigations. However, there are several functional and anatomical comorbidities associated with this congenital condition, including urinary incontinence, reflux, renal anomalies, and altered cosmesis. Workup of epispadias may, therefore, require imaging (ultrasound, voiding cystourethrogram [VCUG], pelvic magnetic resonance imaging [MRI], renal scintigraphy), cystoscopy, urodynamics, and laboratory tests. The most important factors determining postoperative continence and cosmesis in these patients are the extent of the initial anatomic abnormalities, the degree of voiding dysfunction, the surgeon's experience and operative skill, and the need for surgical operations. Long-term follow-up is necessary to address the psychosexual issues and monitor renal and bladder function. Patients may require lifelong intermittent imaging and laboratory evaluations. In some cases, multiple operations are required to address continence, correct complications such as postoperative fistulae, and achieve satisfactory anatomic positioning and appearance. Given that many patients will require lifelong monitoring of bladder and kidney function, education for patients and families is a crucial component of comprehensive care.