2. INTRODUCTION
Epispadias is a rare congenital malformation of the male or female urogenital organs that
consists of a defect of the dorsal wall of the urethra.
In boys with epispadias, the urethra generally opens on the top or side of the penis rather
than the tip.
However, it is possible for the urethra to be open along the entire length of the penis. In
girls, the opening is usually between the clitoris and the labia but may be in the abdominal
area.
Epispadias can be associated with bladder exstrophy.
3. DEFINITION
Epispadias is a rare urogenital malformation characterized by the failure of
the urethral tube to tubularize on the dorsal aspect.
Epispadias is an abnormality of the urethra in which the urethral opening is
ectopically located on the dorsal aspect of the penis.
4.
5. INCIDENCE
Epispadias occurs in
one in 117,000 newborn boys
one in 484,000 newborn girls.
It is commonly seen as a component in the spectrum of bladder exstrophyepispadias-
complex (BEEC).
Isolated epispadias constitutes less than 10 percent of the total cases of epispadias.
Isolated male epispadias is rare.
6. ETIOLOGY
Epispadias can be explained by defective migration of the paired primordia of the genital
tubercle that fuse on the midline to form the genital tubercle at the fifth week of
embryologic development.
Epispadias is rarely observed in 2 members of the same family.
8. FEMALE EPISPADIAS
Isolated female epispadias without bladder exstrophy is an extremely rare congenital
anomaly.
The symptoms of female epispadias are primary urinary incontinence and abnormal
anatomical features.
In females, epispadias consists of bifid clitoris with diastases of the corpora cavernosa,
flattening of the mons, and separation of the labia.
9. CLINICAL MANIFESTATION (MALE)
Abnormal opening from the joint between the pubic bones to the area above the tip of the
penis
Backward flow of urine into the kidney (reflux nephropathy)
Short, widened penis with an abnormal curvature
Urinary tract infections
Widened pubic bone
10.
11. CLINICAL MANIFESTATION (FEMALE)
Abnormal clitoris and labia
Abnormal opening from the bladder neck to the area above the normal urethral opening
Backward flow of urine into the kidney (reflux nephropathy)
Widened pubic bone
Urinary incontinence
Urinary tract infections
12. DIAGNOSIS
Physical Examination
Blood test to check electrolyte levels
Intravenous pyelogram (IVP), a special X-ray of the kidneys, bladder and ureters
MRI and CT scans, depending on the condition
Pelvic X-ray
Ultrasound of the urogenital system
13. SURGICAL MANAGEMENT
Surgical management of epispadias is challenging and requires technical expertise.
The surgery aims to reconstruct the genitalia and urethra, providing optimal functional and
cosmetic outcomes.
At present, surgeons across the world have adopted either of the two following approaches:
Modified Cantwell-Ransley repair
Complete penile disassembly technique (Mitchell and Bagli)
14. NURSING MANAGEMENT
Assess the newborns flow of urine, exit site, and angle of urination
Infant/child returns from surgery w/ penis wrapped in a simple dressing and a stent or
catheter for
urinary drainage
Fresh blood may be seen on the dressing and in the stent or catheter in the immediate
postop period, but the urine should become less bloody over the next few hours
Encourage fluid intake to maintain adequate urinary output and patency of the stent
15. NURSING MANAGEMENT
Accurate hourly Input & output is essential
Notify HCP if no urine drainage for 1 hour
Once caudal block wears off, acetaminophen or ibuprofen should be administered for pain
as ordered
Antibiotics are usually prescribed until the urinary stent falls out or is removed
Double diapering protects the stent
16. COMPLICATIONS
Wound infection
Dysuria (due to bladder spasms)
Urethral stricture and obstruction
Persistent chordee
Persistent short length
Urethral retraction and hypospadias
Partial or complete penile loss
