EPISPADIAS.pptx

Epispadias

1. EPISPADIAS BY RAJAH AMINA SULEIMAN

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. 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.

5. ETIOLOGY E pispadias 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.

6. CLASSIFICATION Glandular Penile C omplete ( ie , penopubic ).

7. 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.

8. 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

9. 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

10. 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

11. 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 )

12. 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

13. 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

14. 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