Epispadias is a rare birth defect related to the opening of the urethra(the tube that drains urine from the bladder). It is characterised by poorly formed urethral tube resulting in abnormal opening on top or side of the penis instead of at the tip in males, urethral opening is usually between clitoris and labia in females, but can be higher up in the abdominal area.
More common in boys than girls, with incidence of one in 100,000 males and about one in 480,000 females.
Epispadias can occur alone but can be associated with other abnormalities like urinary tract developmental problems, bladder exstrophy, pelvic floor abnormalities or abdominal wall defect. This wide range of epispadias-related conditions is referred to as the exstrophy-epispadias complex.
CAUSES
It is caused by an alteration in development during a specific stage of pregnancy when the urinary tract is forming. The exact cause of this abnormal development is unknown
SYMPTOMS
In males;

• Urethral opening in abnormal location over the top or side of penis, anywhere between pubic bone to penile tip
• Backward flow of urine into the kidney (reflux nephropathy)
• Curve penis which is broad and short
• Pubic bone widening
• Urinary tract infections
• Urinary incontinence

In females;

• 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(occasional or constant dribbling of urine)
• Urinary tract infections

DIAGNOSIS
If the defect is mild, it might not be noticed at first especially in girls. Condition is diagnosed at birth by presence of abnormal genitalia or later on by urinary incontinence or leak. In case of other associated abnormalities, blood tests, imaging studies to see internal organs and bones are performed
TREATMENT
Surgical repair of epispadias is recommended.
Surgery aims to provide control of urine flow and good cosmetic appearance. It varies widely depending upon the severity of disease, but the general steps are the same.
In males it involves disassembling of penis and urethral reconstruction(urethroplasty) to move the meatus into the correct position along with the correction of chordee. A urinary catheter is usually placed after assembling back the penile tissues.
In females, the procedure involves freeing the urethra from the clitoris area, allowing the opening to move forward to a more normal location. A catheter(urine tube) is placed to allow tissues to heal.

 

FAQs
1. What is the outlook of surgery?
Most patients achieve normal urinary function and good cosmetic outcome but may require more than one procedure to achieve continence depending on the severity of the issue and if the bladder is affected.
2. What is the expected duration of surgery ?
It depends on the procedure and severity of the disease, but usually takes a couple of hours.
3. Does it need hospital stay?
Yes, the procedure is performed under general anaesthesia and requires hospital admission for a couple of days. The hospital stay can be longer if epispadias is associated with other urogenital abnormalities.
4. Are There complications?
Risk varies with the severity of disease, the most common is persistent leakage of urine(incontinence). Infection, urinary tract infections, fistulas, hypospadias and penis injury are other risks. In cases where there is bladder involvement, bladder stones and bladder perforations are additional risks.
5. What is the best time for surgery?
It is recommended that repair is best performed as early as possible, especially for boys with penopubic or penile epispadias and all girls with the condition.
6. What is the long term outcome?
Although most patients achieve good urinary control and cosmesis, in the long term they may have problems of incontinence, sexual dysfunction and psychosocial problems.

 

Written by : Dr. Sobia Yasmeen
Copyrights: Pakistan association of Plastic Surgeons