Epispadias.

Abstract

Diastasis of the symphysis pubis has been commonly associated with exstrophy of the bladder but can occur without this anomaly. The most common anomaly, excluding bladder exstrophy, is epispadias. This defect of the urethra is the subject of this report, and the material consists of a review of the cases of epispadias seen at the Columbia-Presbyterian Medical Center in the past fifteen years. The investigation was undertaken primarily to seek a correlation between the radiographic and clinical findings. Epispadias occurs once in 30,000 births and afflicts males three times more frequently than females (1). This condition may be considered a precursor to vesical exstrophy. There are varying degrees of absence of the anterior portion of the urethra, the severity of which determines the clinical findings and symptoms. In females clitoric, subsymphyseal, and complete epispadias are described. However, since sphincteric involvement is present in 90 per cent of the females afflicted, incontinence is prevalent. Wide separation of the labia, bifid clitoris, flattened mons pubis, and diastasis of the pubic bones vary in this maldevelopment of the urethra. In males glandular, penile, and complete forms exist. The most common type is glandular with the urethra opening on the dorsum of the phallus behind the glans. In the penile form the meatus may be seen at any point along the shaft; most frequently it occurs at the base of the penis. A dorsal sulcus lined by urethral mucosa is present, associated with a spade-like glans which is uncovered (1, 3–6). In the complete form, incontinence is the rule, owing to the inadequate development of the sphincteric mechanism. The external sphincteric structures form an open arc whose ends are attached to the separated pubic bones. The rami are connected by an interpubic band anterior to the urethra. As in the female, the urethra is open to the bladder neck and the bladder itself is thin-walled with small capacity. Due to the incontinence there is a small bladder capacity since the bladder has never had to stretch to accommodate a large volume of urine. Material Fourteen cases of epispadias were reviewed, consisting of 3 females and 11 males. The females had complete epispadias with incontinence, and after their operative procedure, 2 girls showed vesicoureteral reflux, one with incontinence. Five of the 11 males had complete epispadias, with preoperative incontinence in 4 cases. In this group surgery was followed by only slight improvement to a state of stress incontinence in one who was previously totally incontinent, total incontinence in another who had been incontinent prior to surgery, and continence in 3, one of whom now showed vesicoureteral reflux. During a two-year follow-up, no evidence of clinical pyelonephritis was found in the patient who showed vesicoureteral reflux. Long-term follow-up results are not available.