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Obstetrics
Diagnosis
Abdominal Wall and Gastrointestinal Tract
Abdominal Wall Defects
Pentalogy of Cantrell
Pentalogy of Cantrell
Paula J. Woodward, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Complex malformation with 5 classic components
                  • Midline abdominal wall defect
                  • Lower sternal defect/cleft
                  • Anterior diaphragmatic defect
                  • Defect of diaphragmatic pericardium
                  • Intracardiac defect

              IMAGING

              • General Features

                • Ultrasonographic Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            CLINICAL ISSUES

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Kaul B et al: 5, 4, 3, 2, 1: embryologic variants of pentalogy of Cantrell. J Surg Res. 199(1): 141-8, 2015
                                    2. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                    3. Türkçapar AF et al: Diagnosis of pentalogy of cantrell in the first trimester using transvaginal sonography and color Doppler. Case Rep Obstet Gynecol. 2015:179298, 2015
                                    4. Martins CF et al: Pentalogy of Cantrell: the complete spectrum. BMJ Case Rep. 2014, 2014
                                    5. Pirasteh A et al: Pentalogy of Cantrell with Ectopia Cordis: CT Findings. J Radiol Case Rep. 8(12):29-34, 2014
                                    6. Kubba T et al: Prenatal diagnosis of pentalogy of Cantrell at 11-13 weeks: Evidence for a hexalogy. J Obstet Gynaecol. 33(1):85-6, 2013
                                    7. Mallula KK et al: Spectrum of Cantrell's pentalogy: case series from a single tertiary care center and review of the literature. Pediatr Cardiol. 34(7):1703-10, 2013
                                    8. Ludwig K et al: Pentalogy of cantrell with complete ectopia cordis in a fetus with asplenia. Pediatr Dev Pathol. 15(6):495-8, 2012
                                    9. Marino AL et al: Pentalogy of Cantrell with a single-ventricle cardiac defect: collaborative management of a complex disease. Pediatr Cardiol. 32(4):498-502, 2011
                                    10. Gün I et al: Prenatal diagnosis of vertebral deformities associated with pentalogy of Cantrell: the role of three-dimensional sonography? J Clin Ultrasound. 38(8):446-9, 2010
                                    11. Rodgers EB et al: Diagnosis of pentalogy of Cantrell using 2- and 3-dimensional sonography. J Ultrasound Med. 29(12):1825-8, 2010
                                    12. Peixoto-Filho FM et al: Prenatal diagnosis of Pentalogy of Cantrell in the first trimester: is 3-dimensional sonography needed? J Clin Ultrasound. 37(2):112-4, 2009
                                    13. Pete B et al: [Pentalogy of Cantrell: six new cases.] Orv Hetil. 150(45):2068-73, 2009
                                    14. Engum SA: Embryology, sternal clefts, ectopia cordis, and Cantrell's pentalogy. Semin Pediatr Surg. 17(3):154-60, 2008
                                    15. van Hoorn JH et al: Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach. Eur J Pediatr. 167(1):29-35, 2008
                                    16. Loureiro T et al: Prenatal sonographic diagnosis of a rare Cantrell's pentalogy variant with associated open neural tube defect - a case report. Fetal Diagn Ther. 22(3):172-4, 2007
                                    17. Polat I et al: Prenatal diagnosis of pentalogy of Cantrell in three cases, two with craniorachischisis. J Clin Ultrasound. 33(6):308-11, 2005
                                    18. Carmi R et al: Pentalogy of Cantrell and associated midline anomalies: a possible ventral midline developmental field. Am J Med Genet. 42(1):90-5, 1992
                                    19. Cantrell JR et al: A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet. 107(5):602-14, 1958
                                    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

                                                  • Complex malformation with 5 classic components
                                                    • Midline abdominal wall defect
                                                    • Lower sternal defect/cleft
                                                    • Anterior diaphragmatic defect
                                                    • Defect of diaphragmatic pericardium
                                                    • Intracardiac defect

                                                IMAGING

                                                • General Features

                                                  • Ultrasonographic Findings

                                                    • MR Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Staging, Grading, & Classification

                                                              CLINICAL ISSUES

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Kaul B et al: 5, 4, 3, 2, 1: embryologic variants of pentalogy of Cantrell. J Surg Res. 199(1): 141-8, 2015
                                                                      2. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                                                      3. Türkçapar AF et al: Diagnosis of pentalogy of cantrell in the first trimester using transvaginal sonography and color Doppler. Case Rep Obstet Gynecol. 2015:179298, 2015
                                                                      4. Martins CF et al: Pentalogy of Cantrell: the complete spectrum. BMJ Case Rep. 2014, 2014
                                                                      5. Pirasteh A et al: Pentalogy of Cantrell with Ectopia Cordis: CT Findings. J Radiol Case Rep. 8(12):29-34, 2014
                                                                      6. Kubba T et al: Prenatal diagnosis of pentalogy of Cantrell at 11-13 weeks: Evidence for a hexalogy. J Obstet Gynaecol. 33(1):85-6, 2013
                                                                      7. Mallula KK et al: Spectrum of Cantrell's pentalogy: case series from a single tertiary care center and review of the literature. Pediatr Cardiol. 34(7):1703-10, 2013
                                                                      8. Ludwig K et al: Pentalogy of cantrell with complete ectopia cordis in a fetus with asplenia. Pediatr Dev Pathol. 15(6):495-8, 2012
                                                                      9. Marino AL et al: Pentalogy of Cantrell with a single-ventricle cardiac defect: collaborative management of a complex disease. Pediatr Cardiol. 32(4):498-502, 2011
                                                                      10. Gün I et al: Prenatal diagnosis of vertebral deformities associated with pentalogy of Cantrell: the role of three-dimensional sonography? J Clin Ultrasound. 38(8):446-9, 2010
                                                                      11. Rodgers EB et al: Diagnosis of pentalogy of Cantrell using 2- and 3-dimensional sonography. J Ultrasound Med. 29(12):1825-8, 2010
                                                                      12. Peixoto-Filho FM et al: Prenatal diagnosis of Pentalogy of Cantrell in the first trimester: is 3-dimensional sonography needed? J Clin Ultrasound. 37(2):112-4, 2009
                                                                      13. Pete B et al: [Pentalogy of Cantrell: six new cases.] Orv Hetil. 150(45):2068-73, 2009
                                                                      14. Engum SA: Embryology, sternal clefts, ectopia cordis, and Cantrell's pentalogy. Semin Pediatr Surg. 17(3):154-60, 2008
                                                                      15. van Hoorn JH et al: Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach. Eur J Pediatr. 167(1):29-35, 2008
                                                                      16. Loureiro T et al: Prenatal sonographic diagnosis of a rare Cantrell's pentalogy variant with associated open neural tube defect - a case report. Fetal Diagn Ther. 22(3):172-4, 2007
                                                                      17. Polat I et al: Prenatal diagnosis of pentalogy of Cantrell in three cases, two with craniorachischisis. J Clin Ultrasound. 33(6):308-11, 2005
                                                                      18. Carmi R et al: Pentalogy of Cantrell and associated midline anomalies: a possible ventral midline developmental field. Am J Med Genet. 42(1):90-5, 1992
                                                                      19. Cantrell JR et al: A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet. 107(5):602-14, 1958
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