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Omphalocele
Roya Sohaey, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Exomphalos
            • Definitions

              • Membrane-covered midline abdominal wall defect with herniation of abdominal contents into base of cord

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Oquendo M et al: Silver-impregnated hydrofiber dressing followed by delayed surgical closure for management of infants born with giant omphaloceles. J Pediatr Surg. 50(10):1668-72, 2015
                                      2. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                      3. 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
                                      4. Barseghyan K et al: Progression of a giant omphalocele in utero: ultrasound and fetal magnetic resonance imaging findings. Fetal Diagn Ther. 28(4):233-5, 2010
                                      5. Cohen-Overbeek TE et al: Omphalocele: comparison of outcome following prenatal or postnatal diagnosis. Ultrasound Obstet Gynecol. 36(6):687-92, 2010
                                      6. Danzer E et al: Prospective, interdisciplinary follow-up of children with prenatally diagnosed giant omphalocele: short-term neurodevelopmental outcome. J Pediatr Surg. 45(4):718-23, 2010
                                      7. Doi T et al: HoxB2, HoxB4 and Alx4 genes are downregulated in the cadmium-induced omphalocele in the chick model. Pediatr Surg Int. 26(10):1017-23, 2010
                                      8. Doi T et al: The role of transforming growth factor-beta 2 and 3 in formation of ventral body wall in the cadmium-induced omphalocele chick model. J Pediatr Surg. 45(5):898-902, 2010
                                      9. 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
                                      10. Joó JG et al: Abdominal wall malformations in a 15-year fetopathological study: accuracy of prenatal ultrasonography diagnosis. Prenat Diagn. 30(11):1015-8, 2010
                                      11. Mitanchez D et al: Neonatal care in patients with giant ompholocele: arduous management but favorable outcomes. J Pediatr Surg. 45(8):1727-33, 2010
                                      12. Nicholas S et al: Estimation of fetal weight in fetuses with abdominal wall defects: comparison of 2 recent sonographic formulas to the Hadlock formula. J Ultrasound Med. 29(7):1069-74, 2010
                                      13. Sadler TW: The embryologic origin of ventral body wall defects. Semin Pediatr Surg. 19(3):209-14, 2010
                                      14. Sheth NP: "The use of intraabdominal tissue expanders as a primary strategy for closure of giant omphalocele" by Drs Martin, Khan, Kim et al. J Pediatr Surg. 45(6):1384-5; author reply 1385, 2010
                                      15. Whitehouse JS et al: Conservative management of giant omphalocele with topical povidone-iodine and its effect on thyroid function. J Pediatr Surg. 45(6):1192-7, 2010
                                      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

                                                  • Exomphalos
                                                • Definitions

                                                  • Membrane-covered midline abdominal wall defect with herniation of abdominal contents into base of cord

                                                IMAGING

                                                • General Features

                                                  • Ultrasonographic Findings

                                                    • MR Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Staging, Grading, & Classification

                                                              • Gross Pathologic & Surgical Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Oquendo M et al: Silver-impregnated hydrofiber dressing followed by delayed surgical closure for management of infants born with giant omphaloceles. J Pediatr Surg. 50(10):1668-72, 2015
                                                                          2. Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
                                                                          3. 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
                                                                          4. Barseghyan K et al: Progression of a giant omphalocele in utero: ultrasound and fetal magnetic resonance imaging findings. Fetal Diagn Ther. 28(4):233-5, 2010
                                                                          5. Cohen-Overbeek TE et al: Omphalocele: comparison of outcome following prenatal or postnatal diagnosis. Ultrasound Obstet Gynecol. 36(6):687-92, 2010
                                                                          6. Danzer E et al: Prospective, interdisciplinary follow-up of children with prenatally diagnosed giant omphalocele: short-term neurodevelopmental outcome. J Pediatr Surg. 45(4):718-23, 2010
                                                                          7. Doi T et al: HoxB2, HoxB4 and Alx4 genes are downregulated in the cadmium-induced omphalocele in the chick model. Pediatr Surg Int. 26(10):1017-23, 2010
                                                                          8. Doi T et al: The role of transforming growth factor-beta 2 and 3 in formation of ventral body wall in the cadmium-induced omphalocele chick model. J Pediatr Surg. 45(5):898-902, 2010
                                                                          9. 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
                                                                          10. Joó JG et al: Abdominal wall malformations in a 15-year fetopathological study: accuracy of prenatal ultrasonography diagnosis. Prenat Diagn. 30(11):1015-8, 2010
                                                                          11. Mitanchez D et al: Neonatal care in patients with giant ompholocele: arduous management but favorable outcomes. J Pediatr Surg. 45(8):1727-33, 2010
                                                                          12. Nicholas S et al: Estimation of fetal weight in fetuses with abdominal wall defects: comparison of 2 recent sonographic formulas to the Hadlock formula. J Ultrasound Med. 29(7):1069-74, 2010
                                                                          13. Sadler TW: The embryologic origin of ventral body wall defects. Semin Pediatr Surg. 19(3):209-14, 2010
                                                                          14. Sheth NP: "The use of intraabdominal tissue expanders as a primary strategy for closure of giant omphalocele" by Drs Martin, Khan, Kim et al. J Pediatr Surg. 45(6):1384-5; author reply 1385, 2010
                                                                          15. Whitehouse JS et al: Conservative management of giant omphalocele with topical povidone-iodine and its effect on thyroid function. J Pediatr Surg. 45(6):1192-7, 2010