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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
- Exstrophy-epispadias complex
Definitions
- Classic bladder exstrophy (CBE): Low midline abdominal wall defect (AWD) with exposure of bladder plate & urethra + low-set umbilicus
- Split lower abdominal skin & rectus abdominis + pubic symphysis diastasis
- Bifid clitoris in girls, epispadias in boys
- Epispadias: Abnormal dorsal urethral opening
- ± focal if isolated
- In CBE, entire urethra is open with abnormal bladder sphincter
- Bladder exstrophy variants
- Duplicated: CBE + 2nd intact bladder
- Pseudoexstrophy: Typical musculoskeletal defects without genitourinary anomaly
- Superior vesical fistula/fissure: Small portion of otherwise intact bladder has minimal exstrophy
- Covered: Thin skin layer over intact urinary tract
- Classic bladder exstrophy (CBE): Low midline abdominal wall defect (AWD) with exposure of bladder plate & urethra + low-set umbilicus
IMAGING
General Features
Radiographic Findings
Fluoroscopic Findings
Ultrasonographic Findings
CT Findings
Nuclear Medicine Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Weiss DA et al: Key anatomic findings on fetal ultrasound and MRI in the prenatal diagnosis of bladder and cloacal exstrophy. J Pediatr Urol. 16(5):665-71, 2020
- Szymanski KM et al: Probability of bladder augmentation, diversion and clean intermittent catheterization in classic bladder exstrophy: a 36-year, multi-institutional, retrospective cohort study. J Urol. 202(6):1256-62, 2019
- Victoria T et al: Fetal anterior abdominal wall defects: prenatal imaging by magnetic resonance imaging. Pediatr Radiol. 48(4):499-512, 2018
- Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
- Torres US et al: When closure fails: what the radiologist needs to know about the embryology, anatomy, and prenatal imaging of ventral body wall defects. Semin Ultrasound CT MR. 36(6):522-36, 2015
- Pierre K et al: Bladder exstrophy: current management and postoperative imaging. Pediatr Radiol. 44(7):768-86; quiz 765-7, 2014
- Cervellione RM et al: Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms. J Pediatr Urol. 6(5):450-6, 2010
- Tomita SS et al: An unusual form of duplicate bladder exstrophy. J Pediatr Surg. 45(4):834-6, 2010
- Ebert AK et al: The exstrophy-epispadias complex. Orphanet J Rare Dis. 4:23, 2009
- Eeg KR et al: The exstrophy-epispadias complex. Curr Urol Rep. 9(2):158-64, 2008
- Schaeffer AJ et al: Complications of primary closure of classic bladder exstrophy. J Urol. 180(4 Suppl):1671-4; discussion 1674, 2008
- Williams AM et al: 3-dimensional magnetic resonance imaging modeling of the pelvic floor musculature in classic bladder exstrophy before pelvic osteotomy. J Urol. 172(4 Pt 2):1702-5, 2004
- Stec AA et al: Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights. J Urol. 166(4):1444-9, 2001
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
- Exstrophy-epispadias complex
Definitions
- Classic bladder exstrophy (CBE): Low midline abdominal wall defect (AWD) with exposure of bladder plate & urethra + low-set umbilicus
- Split lower abdominal skin & rectus abdominis + pubic symphysis diastasis
- Bifid clitoris in girls, epispadias in boys
- Epispadias: Abnormal dorsal urethral opening
- ± focal if isolated
- In CBE, entire urethra is open with abnormal bladder sphincter
- Bladder exstrophy variants
- Duplicated: CBE + 2nd intact bladder
- Pseudoexstrophy: Typical musculoskeletal defects without genitourinary anomaly
- Superior vesical fistula/fissure: Small portion of otherwise intact bladder has minimal exstrophy
- Covered: Thin skin layer over intact urinary tract
- Classic bladder exstrophy (CBE): Low midline abdominal wall defect (AWD) with exposure of bladder plate & urethra + low-set umbilicus
IMAGING
General Features
Radiographic Findings
Fluoroscopic Findings
Ultrasonographic Findings
CT Findings
Nuclear Medicine Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Weiss DA et al: Key anatomic findings on fetal ultrasound and MRI in the prenatal diagnosis of bladder and cloacal exstrophy. J Pediatr Urol. 16(5):665-71, 2020
- Szymanski KM et al: Probability of bladder augmentation, diversion and clean intermittent catheterization in classic bladder exstrophy: a 36-year, multi-institutional, retrospective cohort study. J Urol. 202(6):1256-62, 2019
- Victoria T et al: Fetal anterior abdominal wall defects: prenatal imaging by magnetic resonance imaging. Pediatr Radiol. 48(4):499-512, 2018
- Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
- Torres US et al: When closure fails: what the radiologist needs to know about the embryology, anatomy, and prenatal imaging of ventral body wall defects. Semin Ultrasound CT MR. 36(6):522-36, 2015
- Pierre K et al: Bladder exstrophy: current management and postoperative imaging. Pediatr Radiol. 44(7):768-86; quiz 765-7, 2014
- Cervellione RM et al: Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms. J Pediatr Urol. 6(5):450-6, 2010
- Tomita SS et al: An unusual form of duplicate bladder exstrophy. J Pediatr Surg. 45(4):834-6, 2010
- Ebert AK et al: The exstrophy-epispadias complex. Orphanet J Rare Dis. 4:23, 2009
- Eeg KR et al: The exstrophy-epispadias complex. Curr Urol Rep. 9(2):158-64, 2008
- Schaeffer AJ et al: Complications of primary closure of classic bladder exstrophy. J Urol. 180(4 Suppl):1671-4; discussion 1674, 2008
- Williams AM et al: 3-dimensional magnetic resonance imaging modeling of the pelvic floor musculature in classic bladder exstrophy before pelvic osteotomy. J Urol. 172(4 Pt 2):1702-5, 2004
- Stec AA et al: Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights. J Urol. 166(4):1444-9, 2001
