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Cloacal Exstrophy/OEIS Syndrome
Anne M. Kennedy, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Cardinal findings of cloacal exstrophy (CE) are hemibladder exstrophy, hindgut extrusion, imperforate anus
                • Hemibladders contain ureter/vas deferens orifices in males, uterovaginal canal in females
                • Hemibladders flank opening of small intestine/blind-ending large intestine
                • Omphalocele present in 64% (i.e., it is not essential for diagnosis)
              • Exstrophy epispadias complex (EEC)
                • Spectrum of malformations ranging from epispadias to bladder exstrophy (BE) to CE
                • Defects involve genitourinary/musculoskeletal systems, pelvis, pelvic floor, abdominal wall, spine, anus
              • Term OEIS complex was proposed by Carey et al in 1978 as way to recall all defects present
                • Omphalocele
                • Exstrophy of bladder
                • Imperforate anus
                • Spinal deformities (includes vertebral anomalies, dysraphism, sacral defects)
              • Birth defects registries now use other terms to describe other combinations of findings with CE
                • OEI: Omphalocele, exstrophy, imperforate anus
                • EIS: Exstrophy, imperforate anus, spine abnormalities

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                    2. Prefumo F et al: Fetal abdominal wall defects. Best Pract Res Clin Obstet Gynaecol. 28(3):391-402, 2014
                                    3. Calvo-Garcia MA et al: Fetal MRI of cloacal exstrophy. Pediatr Radiol. 43(5):593-604, 2013
                                    4. Suson KD et al: Intracranial anomalies and cloacal exstrophy--is there a role for screening? J Pediatr Surg. 48(11):2256-60, 2013
                                    5. Feldkamp ML et al: Cloacal exstrophy: an epidemiologic study from the International Clearinghouse for Birth Defects Surveillance and Research. Am J Med Genet C Semin Med Genet. 157C(4):333-43, 2011
                                    6. Sawaya D et al: Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience. J Pediatr Surg. 45(1):171-5; discussion 175-6, 2010
                                    7. Wax JR et al: First-trimester prenatal sonographic diagnosis of omphalocele-exstrophy-imperforate anus-spinal defects complex. J Clin Ultrasound. 37(3):171-4, 2009
                                    8. Chen CP et al: Prenatal 3-dimensional sonographic and MRI findings in omphalocele-exstrophy-imperforate anus-spinal defects complex. J Clin Ultrasound. 36(5):308-11, 2008
                                    9. Gobbi D et al: Early prenatal diagnosis of cloacal exstrophy with fetal magnetic resonance imaging. Fetal Diagn Ther. 24(4):437-9, 2008
                                    10. Levitt MA et al: Cloacal exstrophy--pull-through or permanent stoma? A review of 53 patients. J Pediatr Surg. 43(1):164-8; discussion 168-70, 2008
                                    11. Tiblad E et al: OEIS sequence--a rare congenital anomaly with prenatal evaluation and postnatal outcome in six cases. Prenat Diagn. 28(2):141-7, 2008
                                    12. Ben-Neriah Z et al: OEIS complex: prenatal ultrasound and autopsy findings. Ultrasound Obstet Gynecol. 29(2):170-7, 2007
                                    13. Keppler-Noreuil K et al: Prenatal ascertainment of OEIS complex/cloacal exstrophy - 15 new cases and literature review. Am J Med Genet A. 143A(18):2122-8, 2007
                                    14. Mukherjee B et al: Psychopathology, psychosocial, gender and cognitive outcomes in patients with cloacal exstrophy. J Urol. 178(2):630-5; discussion 634-5, 2007
                                    15. Oshita M et al: Complete covered cloacal exstrophy. Pediatr Surg Int. 23(10):1029-31, 2007
                                    16. Carey JC: Exstrophy of the cloaca and the OEIS complex: one and the same. Am J Med Genet. 99(4):270, 2001
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Definitions

                                                • Cardinal findings of cloacal exstrophy (CE) are hemibladder exstrophy, hindgut extrusion, imperforate anus
                                                  • Hemibladders contain ureter/vas deferens orifices in males, uterovaginal canal in females
                                                  • Hemibladders flank opening of small intestine/blind-ending large intestine
                                                  • Omphalocele present in 64% (i.e., it is not essential for diagnosis)
                                                • Exstrophy epispadias complex (EEC)
                                                  • Spectrum of malformations ranging from epispadias to bladder exstrophy (BE) to CE
                                                  • Defects involve genitourinary/musculoskeletal systems, pelvis, pelvic floor, abdominal wall, spine, anus
                                                • Term OEIS complex was proposed by Carey et al in 1978 as way to recall all defects present
                                                  • Omphalocele
                                                  • Exstrophy of bladder
                                                  • Imperforate anus
                                                  • Spinal deformities (includes vertebral anomalies, dysraphism, sacral defects)
                                                • Birth defects registries now use other terms to describe other combinations of findings with CE
                                                  • OEI: Omphalocele, exstrophy, imperforate anus
                                                  • EIS: Exstrophy, imperforate anus, spine abnormalities

                                              IMAGING

                                              • General Features

                                                • Ultrasonographic Findings

                                                  • MR Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Gross Pathologic & Surgical Features

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                                                      2. Prefumo F et al: Fetal abdominal wall defects. Best Pract Res Clin Obstet Gynaecol. 28(3):391-402, 2014
                                                                      3. Calvo-Garcia MA et al: Fetal MRI of cloacal exstrophy. Pediatr Radiol. 43(5):593-604, 2013
                                                                      4. Suson KD et al: Intracranial anomalies and cloacal exstrophy--is there a role for screening? J Pediatr Surg. 48(11):2256-60, 2013
                                                                      5. Feldkamp ML et al: Cloacal exstrophy: an epidemiologic study from the International Clearinghouse for Birth Defects Surveillance and Research. Am J Med Genet C Semin Med Genet. 157C(4):333-43, 2011
                                                                      6. Sawaya D et al: Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience. J Pediatr Surg. 45(1):171-5; discussion 175-6, 2010
                                                                      7. Wax JR et al: First-trimester prenatal sonographic diagnosis of omphalocele-exstrophy-imperforate anus-spinal defects complex. J Clin Ultrasound. 37(3):171-4, 2009
                                                                      8. Chen CP et al: Prenatal 3-dimensional sonographic and MRI findings in omphalocele-exstrophy-imperforate anus-spinal defects complex. J Clin Ultrasound. 36(5):308-11, 2008
                                                                      9. Gobbi D et al: Early prenatal diagnosis of cloacal exstrophy with fetal magnetic resonance imaging. Fetal Diagn Ther. 24(4):437-9, 2008
                                                                      10. Levitt MA et al: Cloacal exstrophy--pull-through or permanent stoma? A review of 53 patients. J Pediatr Surg. 43(1):164-8; discussion 168-70, 2008
                                                                      11. Tiblad E et al: OEIS sequence--a rare congenital anomaly with prenatal evaluation and postnatal outcome in six cases. Prenat Diagn. 28(2):141-7, 2008
                                                                      12. Ben-Neriah Z et al: OEIS complex: prenatal ultrasound and autopsy findings. Ultrasound Obstet Gynecol. 29(2):170-7, 2007
                                                                      13. Keppler-Noreuil K et al: Prenatal ascertainment of OEIS complex/cloacal exstrophy - 15 new cases and literature review. Am J Med Genet A. 143A(18):2122-8, 2007
                                                                      14. Mukherjee B et al: Psychopathology, psychosocial, gender and cognitive outcomes in patients with cloacal exstrophy. J Urol. 178(2):630-5; discussion 634-5, 2007
                                                                      15. Oshita M et al: Complete covered cloacal exstrophy. Pediatr Surg Int. 23(10):1029-31, 2007
                                                                      16. Carey JC: Exstrophy of the cloaca and the OEIS complex: one and the same. Am J Med Genet. 99(4):270, 2001