Fig. 72.1
Clinical photographs showing epispadias. Note the extent of the epispadias groove in (a) (glandular) and the dorsal chordee in (b)
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The symphysis pubis is generally widened.
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The rectus muscles are divergent distally.
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In males:
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◦ The phallus is short and broad with upward chordee.
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◦ The urethral meatus is located on the dorsal penile shaft and can be limited to the glans penis or extends the whole length of the penis.
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◦ The glans penis lies open and flat .
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In females:
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◦ The clitoris is bifid with divergent labia superiorly.
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◦ The dorsal aspect of the urethra is open distally.
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◦ The urethra and bladder neck are patulous and may allow visualization of bladder.
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◦ Bladder mucosa may prolapse through the bladder neck .
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In cloacal exstrophy (covered in detail in a separate chapter; Fig. 72.2) :
Fig. 72.2
A clinical photograph showing classic cloacal exstrophy. Note the omphalocele, the open urinary bladder, and the open trunk of bowel onto the cecal plate
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Nearly all patients have an associated omphalocele.
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The bladder is open and separated into two halves, with the exposed interior of the cecum between them.
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The cecal plate contains openings to the remainder of the hindgut and to one or two appendices.
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The terminal ileum may prolapse as a “trunk” of bowel onto the cecal plate.
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The penis is generally small and bifid, with a hemi-glans located just caudal to each hemi-bladder.
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Infrequently, the phallus may be intact in the midline.
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In females, the clitoris is bifid and two vaginas are present.
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The anus is absent.
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Sixty-five percent of patients have a clubfoot or major deformity of a lower extremity.
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Eighty percent of patients have vertebral anomalies.
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Ninety-five percent of patients have myelodysplasia, which may include myelomeningocele, lipomeningocele, meningocele, or other forms of occult dysraphism.
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These patients are at risk of neurologic deterioration, and they should be observed closely .
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Early neurosurgical consultation is recommended if a radiographic abnormality of the spinal cord or canal is observed .
In exstrophy variants :
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The symphysis pubis is widely separated, and rectus muscles diverge distally.
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The umbilicus is low in position or elongated.
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A small superior bladder opening or a patch of isolated bladder mucosa may be present.
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The intact bladder may be externally covered by only a thin membrane.
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Isolated ectopic bowel segments have been reported.
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Genitalia generally are intact, though epispadias can occur.
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In the split-symphysis variants of exstrophy, the symphysis pubis is widely separated, and the rectus muscles are divergent .
Isolated Epispadias
Introduction
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Epispadias as an isolated malformation is very rare.
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It is an uncommon congenital malformation of the penis and more commonly seen as part of the epispadias–exstrophy malformation.
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Epispadias occurs more commonly in males than in females. The male to female ratio is 2.3:1.
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It occurs in around 1 in 50,000 to 1 in 120,000 male and 1 in 400,000 to 1 in 500,000 female live births.
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The extent of the defect can vary from a mild glandular defect to complete defects involving the whole length of the phallus.
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Evaluation of the bladder neck and proximal urethra is recommended in patients with epispadias in order to plan surgical management
Embryology
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Epispadias results from defective migration of the paired primordial genital tubercle that fuse on the midline to form the genital tubercle at the fifth week of embryologic development .
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Epispadias and exstrophy of the bladder are considered varying degrees of a single disorder.
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The extent of epispadias varies in severity depending on the time of the insult during embryologic development .
Classification
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Epispadias is classified into :
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Glandular (Fig. 72.3)
Fig. 72.3a and b Clinical photographs showing mild glandular epispadias and full glandular epispadias
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