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Bladder Exstrophy
Steven J. Kraus, MD
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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 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

            IMAGING

            • General Features

              • Radiographic Findings

                • Fluoroscopic Findings

                  • CT Findings

                    • Ultrasonographic 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

                                        1. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                        2. 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
                                        3. Pierre K et al: Bladder exstrophy: current management and postoperative imaging. Pediatr Radiol. 44(7):768-86; quiz 765-7, 2014
                                        4. 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
                                        5. Tomita SS et al: An unusual form of duplicate bladder exstrophy. J Pediatr Surg. 45(4):834-6, 2010
                                        6. Ebert AK et al: The exstrophy-epispadias complex. Orphanet J Rare Dis. 4:23, 2009
                                        7. Eeg KR et al: The exstrophy-epispadias complex. Curr Urol Rep. 9(2):158-64, 2008
                                        8. Schaeffer AJ et al: Complications of primary closure of classic bladder exstrophy. J Urol. 180(4 Suppl):1671-4; discussion 1674, 2008
                                        9. 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
                                        10. 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
                                        Related Anatomy
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                                        Related Differential Diagnoses
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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 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

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • Fluoroscopic Findings

                                                        • CT Findings

                                                          • Ultrasonographic 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

                                                                              1. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                                                              2. 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
                                                                              3. Pierre K et al: Bladder exstrophy: current management and postoperative imaging. Pediatr Radiol. 44(7):768-86; quiz 765-7, 2014
                                                                              4. 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
                                                                              5. Tomita SS et al: An unusual form of duplicate bladder exstrophy. J Pediatr Surg. 45(4):834-6, 2010
                                                                              6. Ebert AK et al: The exstrophy-epispadias complex. Orphanet J Rare Dis. 4:23, 2009
                                                                              7. Eeg KR et al: The exstrophy-epispadias complex. Curr Urol Rep. 9(2):158-64, 2008
                                                                              8. Schaeffer AJ et al: Complications of primary closure of classic bladder exstrophy. J Urol. 180(4 Suppl):1671-4; discussion 1674, 2008
                                                                              9. 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
                                                                              10. 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