Abstract
Background. Isolated male epispadias (IME) is a rare congenital penile malformation, as often part of bladder-exstrophy-epispadias complex (BEEC). In its isolated presentation, it consists in a defect of the dorsal aspect of the penis, leaving the urethral plate open. Occurrence of urinary incontinence is related to the degree of dorsal displacement of the meatus and the underlying underdevelopment of the urethral sphincter. The technique for primary IME reconstruction, based on anatomic restoration of the urethra and bladder neck, is here illustrated. Patients and Methods. A retrospective database was created with patients who underwent primary IME repair between June 1998 and February 2014. Intraoperative variables, postoperative complications, and outcomes were assessed. A descriptive statistical analysis was performed. Results and Limitations. Eight patients underwent primary repair, with penopubic epispadias (PPE) in 3, penile epispadias (PE) in 2, and glandular epispadias (GE) in 3. Median age at surgery was 13.0 months [7–47]; median follow-up was 52 months [9–120]. Complications requiring further surgery were reported in two patients, while further esthetic surgeries were required in 4 patients. Conclusion. Anatomical restoration in primary IME is safe and effective, with acceptable results given the initial pathology.
Highlights
Isolated male epispadias (IME) in the absence of bladderexstrophy-epispadias complex (BEEC) is a rare malformation, with an estimated incidence in Europe around 0.6 per 100 000 live male births [1,2,3]
The severity of the condition is related to the meatal position and to the degree of incontinence associated with the meatal position, as the bladder neck might be involved in more proximal variants of IME [3, 4]
Even if sphincter insufficiency is often observed in the most proximal forms of IME, certain children possess some degree of outlet competence, in contrast to those with a bladder-exstrophy-epispadias complex (BEEC) condition [7]
Summary
Isolated male epispadias (IME) in the absence of bladderexstrophy-epispadias complex (BEEC) is a rare malformation, with an estimated incidence in Europe around 0.6 per 100 000 live male births [1,2,3]. Like hypospadias, it covers a wide spectrum in which the meatal orifice can be located anywhere from the distal penile shaft to the pubic area. The technique for primary IME reconstruction, based on anatomic restoration of the urethra and bladder neck, is here illustrated. Anatomical restoration in primary IME is safe and effective, with acceptable results given the initial pathology
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