Bladder exstrophy repair

Bladder exstrophy repair

Anesthesia type	General
Airway	ETT
Lines and access	PIV x2 Arterial line (often recommended)
Monitors	Standard ASA Temperature Arterial BP (if placed)
Primary anesthetic considerations
Preoperative	-Neonate/infant physiology -Associated congenital anomalies
Intraoperative	-Large fluid shifts and blood loss risk -Hypothermia prevention -Pelvic osteotomies need -Regional analgesia (caudal/epidural)
Postoperative	-Ventilatory support (neonate or prolonged case) -Epidural analgesia management -Wound dehiscence risk -Electrolyte abnormalities
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Bladder exstrophy is a rare congenital anomaly characterized by failure of lower abdominal wall and anterior bladder closure, resulting in exposure of posterior bladder wall through the abdominal wall. The defect results from failure of mesenchymal migration and premature rupture of cloacal membrane during embryologic development.

Surgical repair is typically performed in neonatal period (primary closure) or later in staged reconstruction.

Overview

Indications

Surgical repair indicated in all cases of classic bladder exstrophy with goals to

• Protect exposed bladder mucosa from infection and trauma
• Prevent progressive renal damage
• Restore urinary continence
• Reconstruct genital and abdominal anatomy

Timing

• Ideally within first year of life, preferably before 9 months
• Immediate closure within first 72 hours of life when pelvic bones are more malleable can help avoid osteotomies
• Delayed or staged reconstruction can be performed in older infants or failed primary closure

Surgical procedure

• Bladder plate mobilization and closure (exposed bladder plate is dissected and mobilized to allow posterior wall approximation)
• Bladder neck and urethra reconstruction (if part of staged repair)
• Abdominal wall closure (rectus muscles and fascia are mobilized and reapproximated)
• Pelvic osteotomies (commonly done to reduce tension and allow pubic approximation, which increase surgical time, blood loss, and postop pain)
• Urinary drainage (suprapubic catheter, urethral stents, urethral catheter depending on stage)
• Postoperative immobilization (e.g. Bryant's traction or Spica cast to protect repair)

Preoperative management

Patient evaluation

System	Considerations
Airway	-Neonatal airway considerations -Syndromic features possible -Prematurity possible -Appropriate ETT size
Neurologic	-Developmental abnormalities possible
Cardiovascular	-Congenital heart disease screening -Neonatal physiology (HR dependence)
Pulmonary	-Prematurity related lung disease -Postoperative apnea risk
Gastrointestinal	-NPO considerations in neonate -Aspiration risk
Hematologic	-T&C -Anticipate moderate to significant blood loss
Renal	-Urinary tract anomalies -Monitor electrolytes
Endocrine	-Neonatal glucose (hypoglycemia risk)
Other	-Hypothermia risk -Latex allergy precautions

Labs and studies

• T&C
• CBC
• BMP, electrolytes
• Echocardiogram if cardiac anomaly suspected

Operating room setup

• Blood products available
• Fluid warmer
• Forced-air warming device
• Pediatric airway equipment
• Consider arterial line setup
• Caudal/epidural kit

Patient preparation and premedication

• Multidisciplinary planning per institution (urology + orthopedic if osteotomies)

Regional and neuraxial techniques

Caudal or Lumbar Epidural strongly considered for

• Intra/postoperative opioid-sparing pain control
• Reduction in movement to protect repair
• Sometimes tunneled as epidural will be in place for days to weeks

Procedure variants

	Variant 1	Variant 2
Unique considerations
Indications
Position
Surgical time
EBL
Postoperative disposition
Pain management
Potential complications