Isolated Male Epispadias with Continence in a 40 Year Old: A Case Report

Abstract

Epispadias is part of the bladder exstrophy-epispadias complex; it has a defective dorsal wall of the urethra with potential incompetence of the urinary continence mechanism. Isolated male epispadias is due to failure of the urethral plate to tubularize on the dorsum of the penis. It is rare with an incidence rate of 1 in 117,000 live births in males. The severity depends on the position of the urethral opening and ranges from peno-pubic to penile and glandular. Epispadias is usually repaired within the first year of life though some patients have presented in their second decade. Epispadias is classically associated with bladder exstrophy in over 90% of the cases while isolated epispadias with continence is very rare constituting less than 10% of cases. Separation of pelvic bones is seen in 70% of peno-pubic epispadias affecting the bladder neck and external sphincter leading to incontinence and stress urinary dribbling. The goals of repair include; achieving a cosmetically acceptable and functional penis, which is straight and adequate in length, enabling penetrative sexual intercourse and urinary continence. There have been reports of isolated epispadias in adults but none was as old as 40 years. This patient has been married for 20 years and has been unable to impregnate his spouse. He had modified Cantwell-Ransley procedure after a detailed clinical and psychological evaluation to achieve the goals of repair. Adults presenting with isolated continent peno-pubic epispadias are rare. Presenting at this age and after having been married for two decades could have a derogatory effect on body image, self-confidence, psycho-sexual and reproductive life. Surgical correction of this anomaly as we did has the potential to lead to acceptable outcomes as demonstrated in this index case.