Epispadias: a urethra that is open on the top side

What is epispadias?

In epispadias, the top wall of the urinary channel (urethra) does not fully form in the womb, and the channel stays open on the top side. In boys, the urinary opening is on the top surface of the penis; in girls, it is higher than usual, between the two halves of a split clitoris.

Epispadias is the opposite of hypospadias, in which the urinary opening is on the underside of the penis. Hypospadias is common, while epispadias is very rare.

Isolated epispadias and the exstrophy-epispadias complex

Epispadias is the mildest form of the exstrophy-epispadias complex. This spectrum also includes bladder exstrophy and cloacal exstrophy.

• Isolated epispadias: the bladder is closed; only the urethra is open.
• Epispadias with exstrophy: all boys with bladder exstrophy also have epispadias, and its repair is one of the stages of exstrophy treatment.

Types

In boys

• Glanular epispadias: the urinary opening is on the top of the head of the penis. This is the mildest form, and urinary control is usually normal.
• Penile epispadias: the opening is on the top side of the shaft of the penis.
• Penopubic epispadias: the opening is at the base of the penis, where it meets the belly wall. Because the bladder neck is also not fully formed, these children often leak urine.

In all forms the penis is usually short, broad and curved upward, and the foreskin lies mainly on the underside. In severe forms the pubic bones may be spread apart.

In girls

In girls, epispadias shows as a split clitoris, separated labia minora, a flattened mons and a short, wide urethra. Urinary leakage is common. Because the visible changes are subtle, the diagnosis is sometimes made late, only when the cause of constant wetness is being looked for.

How common is it?

Isolated epispadias is very rare: in boys about one in 100,000 births or fewer, and even rarer in girls.

Signs and how it is found

• in boys, a urinary opening on the top surface of the penis and upward curvature,
• a urine stream that points up or to the side,
• in girls, a split clitoris and separated labia minora,
• constant dribbling of urine with no dry intervals, or leakage with effort (laughing, coughing, running).

In boys, epispadias is usually visible at birth. In girls, it is often found at toilet training age or later, when the child is never dry. In these girls, other causes of daytime wetting are also considered, especially an ectopic ureter in a duplex kidney.

Tests

• Physical exam: the diagnosis is made mainly by examination.
• Ultrasound: the kidneys and bladder.
• Some children have vesicoureteral reflux; a voiding cystourethrogram is done when needed.
• When there is leakage, bladder size and function are assessed, sometimes with urodynamics.

Treatment

Treatment is surgical and should be done by a team experienced in the exstrophy-epispadias complex.

Repair of the penis or external genitalia

In boys, the goals of surgery are to straighten the penis, rebuild the urethra and bring its opening to the head of the penis, and give the penis an acceptable appearance. This operation is usually done in infancy or early childhood. Commonly used techniques include the Cantwell-Ransley repair and complete penile disassembly (Mitchell-Bagli). A short course of hormone treatment before surgery may be discussed.

In girls, the two halves of the clitoris, the labia minora and the mons are reconstructed. This operation is often combined with bladder neck surgery.

Surgery for urinary control

Children who leak urine have bladder neck reconstruction. It is done when the bladder is large enough and the child is old enough to take part in the treatment. In boys, bladder size often increases after the penile repair.

Achieving urinary control sometimes takes several operations. If the bladder stays small, enlarging it and emptying it with a catheter may be discussed. More about intermittent catheterization: neurogenic bladder.

Long-term outlook and follow-up

• In glanular and many penile forms, urinary control is usually normal.
• In penopubic forms and in girls, additional operations may be needed to achieve urinary control.
• In adolescence, penile size and curvature, sexual function and fertility are discussed. Most men are able to have a sexual life.
• Emotional support for the child and family is important.

Follow-up is long-term and continues after adolescence with a planned move to an adult urologist.

When to see a doctor

• If a boy’s urinary opening is on the top side of the penis or the penis curves upward, have him seen by a pediatric urologist before any circumcision.
• If a girl is never dry and leaks urine constantly day and night, see a pediatric urologist.
• After surgery, seek care urgently for fever, being unable to urinate or a catheter problem.