Chapter 7.  Disorders of Male External Genitalia

69

FIGURE 7.6.  Glans dehiscence after hypospadias repair.

7.2  Epispadias

Key Points

››Epispadias is a rare condition that presents with a dorsal urethral opening below the normal meatal opening. Cosmetically the penis appears split when looking down on it and the urethra appears as an open defect like a trench.

››This is a rare condition, which presents as a spectrum ranging from very mild glanular epispadias to the most severe form, which is associated with bladder exstrophy.

››Children born with the complete exstrophyepispadias complex will need prophylactic antibiotics to prevent urinary tract infection in the face of existing vesicoureteral reflux.

70M.R. Zaontz

››In most boys with epispadias the penis is generally wider, foreshortened and is associated with dorsal chordee where the penis bends back toward the abdomen.

››The meatal location in epispadias determines whether or not urinary incontinence is an issue secondary to an incompetent bladder neck.

››Associate anomalies of other organ systems are very uncommon such that routine screening studies are not performed for isolated epispadias that does not involve the bladder neck. Epispadias-exstrophy complex requires radiographic screening to rule out vesicoureteral reflux and renal anomalies.

››Retrograde ejaculation is common in men with a penopubic epispadias and can lead to fertility problems.

››Penile size and dorsal penile curvature can adversely affect sexual intercourse.

››Correction of epispadias can be done anywhere from the first few days of life in the presence of exstrophy to 6 months post natally for the less severe cases.

››Complications are similar to hypospadias except for the issues associated with urinary continence, overall smaller penile size and difficulty correcting penile curvature.

7.2.1  Introduction

Epispadias (Fig. 7.7) is a rare condition that affects boys in about 1:100,000 live births.2 It represents a condition where the foreskin is ventrally situated and the penis is widened, foreshortened and bends upward toward the abdomen.

Because the penis is attached proximally to the pubic bones

Chapter 7.  Disorders of Male External Genitalia

71

FIGURE 7.7.  Epispadias.

that are separated in both the penopubic and the more severe cases of epispadias, the penis is pulled inward toward the abdomen. The urethral opening can be anywhere from just below the tip of the penis dorsally in the glans to the penopubic location and in the most severe cases presents as the exstrophy-epispadias complex (Fig. 7.8) where the entire bladder and the penis is “split open “ as if cut across in a complete transverse plane.

This condition is readily diagnosed at birth, although frequently incorrectly diagnosed in the nursery setting. In that scenario, the foreskin is complete and because the preputial opening turns upward, consults are inappropriately made to rule out epispadias. This is in fact a common and normal finding for normal foreskin. It is important to recognize that in epispadias the foreskin is hooded and only on the ventral surface of the penis. Hence the epispadias defect is very apparent.

72 M.R. Zaontz

FIGURE 7.8.  Exstrophy-epispadias complex. Note the filleted appearance of the penis.

7.2.2  Management Issues

Referral to a pediatric urologist should be made at the time of diagnosis, which is usually while the baby is in the nursery. While isolated epispadias is not a urologic emergency and can wait several months prior to surgical reconstruction, complete bladder exstrophy-epispadias is a more urgent matter. This condition is usually surgically managed within the first few days of life. In addition because of the presence of vesicoureteral reflux in these severe cases, prophylactic antibiotics are recommended from day one of life.

Fortunately, babies born with isolated epispadias and even the exstrophy-epispadias complex are quite healthy at birth. Isolated epispadias does not usually require any screening study, as the incidence of associated anomalies is very low. However, those boys with the exstrophy-epispadias complex require radiographic evaluation especially because of the known coexisting incidence of vesicoureteral reflux and the risk for renal anomalies such as an absent kidney.

Penopubic epispadias or exstrophy-epispadias will have problems associated with urinary incontinence as they get into toilet training even after primary surgical reconstruction because this population has an incompetent bladder neck.