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Bladder Exstrophy
Anne M. Kennedy, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Bladder exstrophy (BE)
                  • Failure of closure of lower abdominal wall resulting in exposed bladder
                • Exstrophy epispadias complex (EEC)
                  • Spectrum of malformations ranging from epispadias to BE to cloacal exstrophy
                  • Defects involve genitourinary/musculoskeletal systems, pelvis, pelvic floor, abdominal wall, spine, and anus

              IMAGING

              • Ultrasonographic Findings

                • MR Findings

                  • Radiographic Findings

                    • CT Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Draaken M et al: Genome-wide association study and meta-analysis identify ISL1 as genome-wide significant susceptibility gene for bladder exstrophy. PLoS Genet. 11(3):e1005024, 2015
                                      2. Hurrell RA et al: Psychosocial screening at paediatric BEEC clinics: A pilot evaluation study. J Pediatr Urol. 11(2):79.e1-6, 2015
                                      3. Park W et al: Sexual function in adult patients with classic bladder exstrophy: A multicenter study. J Pediatr Urol. 11(3):125 2015
                                      4. Pierre K et al: Bladder exstrophy: current management and postoperative imaging. Pediatr Radiol. 44(7):768-86; quiz 765-7, 2014
                                      5. Tekes A et al: 2D and 3D MRI features of classic bladder exstrophy. Clin Radiol. 69(5):e223-9, 2014
                                      6. Castagnetti M et al: Issues with the external and internal genitalia in postpubertal females born with classic bladder exstrophy: a surgical series. J Pediatr Adolesc Gynecol. 24(1):48-52, 2010
                                      7. Jochault-Ritz S et al: Short and long-term quality of life after reconstruction of bladder exstrophy in infancy: preliminary results of the QUALEX (QUAlity of Life of bladder EXstrophy) study. J Pediatr Surg. 45(8):1693-700, 2010
                                      8. Wittmeyer V et al: Quality of life in adults with bladder exstrophy-epispadias complex. J Urol. 184(6):2389-94, 2010
                                      9. Ebert AK et al: The exstrophy-epispadias complex. Orphanet J Rare Dis. 4:23, 2009
                                      10. Gambhir L et al: Epidemiological survey of 214 families with bladder exstrophy-epispadias complex. J Urol. 179(4):1539-43, 2008
                                      11. Lumen N et al: Phalloplasty: a valuable treatment for males with penile insufficiency. Urology. 71(2):272-6; discussion 276-7, 2008
                                      12. Cadeddu JA et al: Spinal abnormalities in classic bladder exstrophy. Br J Urol. 79(6):975-8, 1997
                                      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

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Definitions

                                                    • Bladder exstrophy (BE)
                                                      • Failure of closure of lower abdominal wall resulting in exposed bladder
                                                    • Exstrophy epispadias complex (EEC)
                                                      • Spectrum of malformations ranging from epispadias to BE to cloacal exstrophy
                                                      • Defects involve genitourinary/musculoskeletal systems, pelvis, pelvic floor, abdominal wall, spine, and anus

                                                  IMAGING

                                                  • Ultrasonographic Findings

                                                    • MR Findings

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Draaken M et al: Genome-wide association study and meta-analysis identify ISL1 as genome-wide significant susceptibility gene for bladder exstrophy. PLoS Genet. 11(3):e1005024, 2015
                                                                          2. Hurrell RA et al: Psychosocial screening at paediatric BEEC clinics: A pilot evaluation study. J Pediatr Urol. 11(2):79.e1-6, 2015
                                                                          3. Park W et al: Sexual function in adult patients with classic bladder exstrophy: A multicenter study. J Pediatr Urol. 11(3):125 2015
                                                                          4. Pierre K et al: Bladder exstrophy: current management and postoperative imaging. Pediatr Radiol. 44(7):768-86; quiz 765-7, 2014
                                                                          5. Tekes A et al: 2D and 3D MRI features of classic bladder exstrophy. Clin Radiol. 69(5):e223-9, 2014
                                                                          6. Castagnetti M et al: Issues with the external and internal genitalia in postpubertal females born with classic bladder exstrophy: a surgical series. J Pediatr Adolesc Gynecol. 24(1):48-52, 2010
                                                                          7. Jochault-Ritz S et al: Short and long-term quality of life after reconstruction of bladder exstrophy in infancy: preliminary results of the QUALEX (QUAlity of Life of bladder EXstrophy) study. J Pediatr Surg. 45(8):1693-700, 2010
                                                                          8. Wittmeyer V et al: Quality of life in adults with bladder exstrophy-epispadias complex. J Urol. 184(6):2389-94, 2010
                                                                          9. Ebert AK et al: The exstrophy-epispadias complex. Orphanet J Rare Dis. 4:23, 2009
                                                                          10. Gambhir L et al: Epidemiological survey of 214 families with bladder exstrophy-epispadias complex. J Urol. 179(4):1539-43, 2008
                                                                          11. Lumen N et al: Phalloplasty: a valuable treatment for males with penile insufficiency. Urology. 71(2):272-6; discussion 276-7, 2008
                                                                          12. Cadeddu JA et al: Spinal abnormalities in classic bladder exstrophy. Br J Urol. 79(6):975-8, 1997