Bladder exstrophy or ectopia vescica

Bladder exstrophy or ectopia vescica BY MR. DINABANDHU BARAD

1. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

2. CONGENITAL ANOMALY OF GENITOURINARY SYSTEM Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU BY: Dinabandhu Barad MSC TUTOR, SNC,SOA,DTU

3. BLADDER EXSTROPHY OR ECTOPIA VESCICA Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

4. Exstrophy of the bladder or ectopia vesicae is a congenital malformation o f t h e urin ar y bl a dd er i n w h i ch a p art of the wall of the bladder in front is absent and the inner part of the bladder is exposed. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU BLADDER EXSTROPHY

5. INCIDENCE The incidence of bladder exstrophy is approximately 2.07 per 100,000 live births and is slightly more common in males than females. The risk of a family having more than one child with this condition is approximately one in 100 and children born to a parent with exstrophy have a risk of approximately one in 70 of having the condition Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

6. ETIOLOGY There is no known cause for this condition but there are many theories. Some experts believe during the 11 th week of pregnancy the embryo undergoes structural changes including growths of tissue in the lower abdominal wall, which stimulates development of muscles and pelvic bones. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

7. RISK FACTORS The disorder has no known risk factors, although it is more common in boys and in infants of mothers who had tobacco exposure or were young when giving birth. Children whose parents were also born with bladder exstrophy have a 1 in 70 chance of having it themselves. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

8. ASSOCIATED PROBLEMS Bladder : The bladder is turned inside out and exposed on the abdominal wall. The bladder neck has not developed properly and the bladder itself is usually small. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

9. Epispadias : The urethral opening, which is the hollow tube that drains urine from the bladder to the outside of the body, is in an abnormal location. In males, the urethral opening is usually on the topside of the penis and not the tip. In girls, the urethral opening may be positioned further up between the divided clitoris and labia minora. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU ASSOCIATED PROBLEMS

10. Widening of the pubic bones: The pubic bones usually join to protect and support the bladder, urethra and abdominal muscles. In children with exstrophy, the pubic bones do not join, leaving a wide opening. This causes the hips to be outwardly rotated. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU ASSOCIATED PROBLEMS

11. Vesicoureteral reflux (VUR) : Normally the kidneys make urine and drain down the ureters (drainage tubes) into the bladder. VUR is a condition where urine travels back up into the kidneys. This may develop after the bladder is reconstructed. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU ASSOCIATED PROBLEMS

12. ABNORMAL DEVELOPMENT OF GENITALIA Boys : The penis may appear shorter and curved in an upward direction. The testicles may not be in a normal position in the scrotum and a hernia may be seen. Girls : The clitoris and labia minora are separated and spread apart; the vagina and urethra are shorter. The uterus, fallopian tubes and ovaries are generally normal. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

13. CLINICAL FEATURES Constant urinary dribbling through the defect Skin excoriation Infection Ulceration of bladder mucosa Ambiguous genitalia Waddling unsteady gait Urinary tract infections Growth failure Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

14. DIAGNOSTIC MEASURES Fetal ultrasound cystoscopic examination X ray U S G IVP Urodynamic studies All these will help to determine the extent of anomaly and other associated problems Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

15. TREATMENT Management of bladder exstrophy should start at birth. Cover the bladder with plastic wrap to keep the bladder mucosa moist. Avoid application of gauze or petroleum-gauze to the bladder mucosa. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

16. SURGICAL MANAGEMENT Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

17. PROMPT BLADDER CLOSURE During this procedure the abdominal wall is mobilized and the pubic rami are brought together in the midline. (If the bladder closure is performed during the first 48 hr of life, often there is sufficient mobility of the pubic rami to allow approximation of the pubic symphysis. If the procedure is delayed, the pelvic bones must be broken (pelvic osteotomy) to allow the pubic rami to be brought together and create a pubic symphysis to support the bladder closure.) Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

18. TOTAL RECONSTRUCTION It includes closure of the bladder, closure of the abdominal wall, and, in boys, correction of epispadias . Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

19. Creation of a sphincter muscle for bladder control and correction of the vesicoureteral reflux The final stage of reconstruction involves creation of a sphincter muscle for bladder control and correction of the vesicoureteral reflux. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

20. POST OPERATIVE CARE Bladder capacity is monitored every 12–24 months using cystoscopy under anesthesia. Monitor for the development of hydronephrosis and infection. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

21. POST OPERATIVE CARE Most infants with bladder exstrophy have vesicoureteral reflux and should receive antibiotic prophylaxis. In boys, if the epispadias is not corrected at birth, epispadias repair usually is performed between 1–2 yr of age Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

22. NURSING CARE ACUTE PAIN NURSING DIAGNOSIS Acute pain related to surgery as evidienced by verbalization of pain crying irritability, restlessness ,distractive behaviour, changes in vital signs GOAL Child will experience decreased pain as evidenced by infrequent crying episodes and exhibit normal sleeping pattern. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

23. NURSING CARE ACUTE PAIN Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU Assess location, characteristics, onset, duration, frequency, location, and severity of pain; Observe for verbal and nonverbal cues. Provides data about the description of pain which can be used as a guideline for analgesic therapy. Maintain a position of comfort; Properly set the catheter to avoid tension and kinking. Promotes comfort and avoids pain due to pulling on or manipulating catheter. Encourage use of  relaxation  techniques. Promotes rest and refocus attention thus decreases discomfort. RATIONALE INTERVENTION

24. NURSING CARE ACUTE PAIN Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU RATIONALE INTERVENTION Administer analgesic (e.g., Tylenol) as ordered. Lessens pain and promotes rest which reduces stimuli and pain. Educate parents that medications will prevent pain and restlessness and allow for healing. Provides information about the need for pain medications for child’s comfort.

25. Impaired Urinary Elimination Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU Nursing Interventions Rationale Record input and output; Assess voiding stream, color and amount of urine on first flow of urine and each succeeding void. Provides data on voiding pattern after clamping or removal of the catheter. Assess for pain, abdominal distention, inability to void for 8 hours after catheter. Indicates urinary dysfunction and possible obstruction or continuing edema of the meatus. Encourage high fluid intake after catheter removed, offer favored choice of liquids hourly. Maintain hydration and free flowing of urine.

26. Impaired Urinary Elimination Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU Nursing Interventions Rationale Support child after the catheter is removed and provide privacy for voiding. Avoids embarrassment on the part of an older child. Instruct parents to notify the physician of changes in the urinary pattern or inability to void. Allows for early intervention to avoid further complications.

27. RISK FOR INFECTION Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU Risk for Infection  related to inadequate primary defenses (surgical incision) invasive procedure (catheter)as evidenced by [not applicable] Desired Outcomes Child will remain free from infection as evidenced by clean and intact wound without redness, edema, odor or drainage and negative urine culture.

28. Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU Nursing Interventions Rationale Assess wound for redness, swelling, drainage on dressing. Provides information on the presence of infection or impaired healing. Observe catheter  insertion  site for redness, irritation, swelling; Monitor urine in the catheter bag for cloudiness, foul odor, sediment. Indicates infectious process at catheter site or in the urinary bladder. Obtain urine specimen for culture and sensitivities as indicated. Determines the specific organism and sensitivity to the  antibiotic . RISK FOR INFECTION

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