link
Bookmarks
Cloacal Exstrophy/OEIS Syndrome
Anne Kennedy, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Subscribe
1
6
2
3

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 defect 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. Gondo K et al: Clues and pitfalls in prenatal diagnosis of classic cloacal exstrophy using ultrasonography and magnetic resonance imaging: a case with sequential observation from 17 to 30weeks' gestation and literature review. J Obstet Gynaecol Res. 46(8):1443-9, 2020
                                    2. 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
                                    3. Arteaga-Vázquez J et al: OEIS complex: prevalence, clinical, and epidemiologic findings in a multicenter Mexican birth defects surveillance program. Birth Defects Res. 111(11):666-71, 2019
                                    4. Vilanova-Sanchez A et al: Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review. J Pediatr Adolesc Gynecol. 32(1):7-14, 2019
                                    5. Coleman PW et al: Fetal MRI in the identification of a fetal ventral wall defect spectrum. AJP Rep. 8(4):e264-76, 2018
                                    6. Neel N et al: Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect complex, multiple major reconstructive surgeries needed. Urol Ann. 10(1):118-21, 2018
                                    7. Keppler-Noreuil KM et al: Clinical and risk factor analysis of cloacal defects in the National Birth Defects Prevention Study. Am J Med Genet A. 173(11):2873-85, 2017
                                    8. Mallmann MR et al: Omphalocele-exstrophy-imperforate anus-spinal defects complex: associated malformations in 12 new cases. Fetal Diagn Ther. 41(1):66-70, 2017
                                    9. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                    10. Prefumo F et al: Fetal abdominal wall defects. Best Pract Res Clin Obstet Gynaecol. 28(3):391-402, 2014
                                    11. Calvo-Garcia MA et al: Fetal MRI of cloacal exstrophy. Pediatr Radiol. 43(5):593-604, 2013
                                    12. Suson KD et al: Intracranial anomalies and cloacal exstrophy--is there a role for screening? J Pediatr Surg. 48(11):2256-60, 2013
                                    13. 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
                                    14. 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
                                    Related Anatomy
                                    Loading...
                                    Related Differential Diagnoses
                                    Loading...
                                    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 defect 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. Gondo K et al: Clues and pitfalls in prenatal diagnosis of classic cloacal exstrophy using ultrasonography and magnetic resonance imaging: a case with sequential observation from 17 to 30weeks' gestation and literature review. J Obstet Gynaecol Res. 46(8):1443-9, 2020
                                                                      2. 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
                                                                      3. Arteaga-Vázquez J et al: OEIS complex: prevalence, clinical, and epidemiologic findings in a multicenter Mexican birth defects surveillance program. Birth Defects Res. 111(11):666-71, 2019
                                                                      4. Vilanova-Sanchez A et al: Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review. J Pediatr Adolesc Gynecol. 32(1):7-14, 2019
                                                                      5. Coleman PW et al: Fetal MRI in the identification of a fetal ventral wall defect spectrum. AJP Rep. 8(4):e264-76, 2018
                                                                      6. Neel N et al: Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect complex, multiple major reconstructive surgeries needed. Urol Ann. 10(1):118-21, 2018
                                                                      7. Keppler-Noreuil KM et al: Clinical and risk factor analysis of cloacal defects in the National Birth Defects Prevention Study. Am J Med Genet A. 173(11):2873-85, 2017
                                                                      8. Mallmann MR et al: Omphalocele-exstrophy-imperforate anus-spinal defects complex: associated malformations in 12 new cases. Fetal Diagn Ther. 41(1):66-70, 2017
                                                                      9. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                                                      10. Prefumo F et al: Fetal abdominal wall defects. Best Pract Res Clin Obstet Gynaecol. 28(3):391-402, 2014
                                                                      11. Calvo-Garcia MA et al: Fetal MRI of cloacal exstrophy. Pediatr Radiol. 43(5):593-604, 2013
                                                                      12. Suson KD et al: Intracranial anomalies and cloacal exstrophy--is there a role for screening? J Pediatr Surg. 48(11):2256-60, 2013
                                                                      13. 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
                                                                      14. 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