Bladder Exstrophy and Epispadias
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Questions and Answers

What is the primary goal of the preoperative work-up for bladder exstrophy?

  • To determine the best surgical approach
  • To exclude anomalies of the upper renal tract
  • To explain the malformation to the parents
  • To identify other renal malformations (correct)

    • What is the recommended timeframe for bladder closure in bladder exstrophy correction?

  • Within the first week of life
  • Within the first 24 hours of life
  • Within the first 48 hours of life (correct)
  • Within the first month of life

    • What is the second stage of surgical intervention for bladder exstrophy correction?

  • Bladder neck reconstruction
  • Bladder augmentation
  • Ureteral stent removal
  • Penile reconstruction (Epispadias repair) (correct)

    • What is a potential long-term complication of bladder exstrophy correction?

    <p>All of the above</p> Signup and view all the answers

    What is the purpose of applying a bandage around the hips with inner rotation in postoperative care?

    <p>To immobilize the hips and promote healing</p> Signup and view all the answers

    What is a potential complication of bladder exstrophy correction?

    <p>All of the above</p> Signup and view all the answers

    What is epispadias?

    <p>A congenital anomaly involving the development of the urethra</p> Signup and view all the answers

    Why is it recommended that children with bladder exstrophy be managed at specialized centers?

    <p>To ensure access to multidisciplinary teams</p> Signup and view all the answers

    In girls with epispadias, where is the urethral opening located?

    <p>In the belly area</p> Signup and view all the answers

    What is a characteristic of boys with distal epispadias?

    <p>They are usually continent</p> Signup and view all the answers

    What is the incidence of cloacal exstrophy?

    <p>1 in 200,000</p> Signup and view all the answers

    What is a common associated anomaly with cloacal exstrophy?

    <p>Spinal dysraphism and short bowel syndrome</p> Signup and view all the answers

    What is the characteristic post-natal appearance of cloacal exstrophy?

    <p>Bladder separated into two halves by an intestinal plate</p> Signup and view all the answers

    What is the significance of having an intact bladder at birth in epispadias patients?

    <p>It is a sign of a less severe condition</p> Signup and view all the answers

    What is the incidence of bladder exstrophy?

    <p>1 in 40,000 live births</p> Signup and view all the answers

    What is the recurrence risk of bladder exstrophy?

    <p>1 in 100</p> Signup and view all the answers

    What is the male-to-female ratio of bladder exstrophy?

    <p>2:1</p> Signup and view all the answers

    What is the embryological defect that leads to bladder exstrophy?

    <p>All of the above</p> Signup and view all the answers

    What is a common antenatal ultrasound finding of exstrophy-epispadias complex?

    <p>Failure to visualize the bladder</p> Signup and view all the answers

    What is a characteristic feature of bladder exstrophy in males?

    <p>Dorsal chordee</p> Signup and view all the answers

    What is an associated anomaly with bladder exstrophy?

    <p>Inguinal hernia</p> Signup and view all the answers

    What is an objective of management of bladder exstrophy?

    <p>All of the above</p> Signup and view all the answers

    Study Notes

    Exstrophy-Epispadias Complex

    • The exstrophy-epispadias complex is a spectrum of midline defects that includes classic bladder exstrophy, epispadias, and cloacal exstrophy.
    • Incidence: 1 in 40,000 live births, with a male to female ratio of 2:1.
    • Recurrence risk is 1 in 100.

    Embryology

    • Failure of mesenchyme to migrate between the ectodermal and endodermal layers of the lower abdominal wall with subsequent premature rupture of the cloacal membrane.
    • Rupture after complete separation of the GU and GI tracts results in classic bladder exstrophy, and prior to descent of the urorectal septum results in cloacal exstrophy.

    Antenatal US Findings

    • Failure to visualize the bladder.
    • Lower abdominal wall bulge and low-set umbilical cord.
    • Abnormal genitalia and abnormal widening of iliac crests.

    Post-natal Appearance

    • The bladder is open on the lower abdomen, with urothelium fully exposed.
    • In males, the penis is short and broad with dorsal chordee.
    • In females, the clitoris is bifid, anterior labia are displaced laterally, and the mons pubis is absent.
    • The pubic symphysis is widely separated in both sexes, with divergent recti muscles.
    • Inguinal hernias are frequently associated with exstrophy.

    Management

    • Objectives of management include:
      • Restoration of urinary continence.
      • Preservation of renal function.
      • Reconstruction of functionally and cosmetically acceptable external genitalia.

    Epispadias

    • Epispadias is a problem that can occur in both boys and girls.
    • In boys, the urethra does not develop into a full tube, and the urine exits the body from an abnormal location.
    • In girls, the urethral opening is in the belly area instead of between the clitoris and labia.
    • Primary epispadias can be detected in the immediate postnatal examination, but in some boys, it may only become evident later.

    Cloacal Exstrophy

    • Incidence: 1 in 200,000, with a male to female ratio of 1:1.
    • Associated with spinal dysraphism (66%) and short bowel syndrome (33%).
    • Post-natal appearance:
      • Bladder is open and separated into two halves by an intestinal plate.
      • Each hemi-bladder may have a ureteric orifice.
      • Nearly all patients have an associated exomphalos.

    Preoperative Work-up

    • Cover the defect with a moist plastic sheet to prevent epithelium swelling.
    • Ultrasonography to exclude other renal malformations.
    • Intravenous urography, if anomalies of the upper renal tract are suspected.
    • Explain the malformation to the parents and involve them in the decision-making process.

    Surgical Intervention

    • Bladder exstrophy correction usually requires staged reconstructive procedures.
    • Children born with the exstrophy-epispadias complex should only be managed within a limited number of centers with a multidisciplinary team.
    • Surgical stages:
      • Bladder closure (first stage) within the first 48 hours of life.
      • Penile reconstruction (Epispadias repair) (second stage) at 12-18 months.
      • Bladder neck reconstruction (third stage) at 4 years.

    Postoperative Care

    • Antibiotics to prevent urinary infection.
    • Bandage application around the hips with inner rotation.
    • Ureteral stents for 2 weeks.
    • Drainage of the bladder to prevent bladder outlet obstruction.

    Complications

    • Bladder dehiscence – partial/complete.
    • Bladder prolapse.
    • Upper urinary tract deterioration due to high outlet resistance and vesicoureteric reflux.
    • Incomplete bladder emptying.

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    Description

    This quiz covers the basics of bladder exstrophy and epispadias, including incidence, recurrence risk, and embryology. Learn about these complex midline defects with Dr. Ahmed Elrashidy.

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