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Congenital Diaphragmatic Hernia
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Congenital diaphragmatic hernia (CDH): Herniation of abdominal contents into chest via developmental defect in diaphragm
              • Intrapleural hernia (80%): Worse prognosis
                • Bochdalek (posterolateral) is most common subtype
              • Mediastinal hernia (20%)
                • Anteriorly involves central tendon of diaphragm
                  • Morgagni hernia: Smaller paramedian subtype
                • Posteriorly may involve hiatus of esophagus or IVC

          IMAGING

          • General Features

            • Radiographic Findings

              • Ultrasonographic Findings

                • MR Findings

                  • CT Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    Selected References

                                    1. Deprest JA et al: Randomized trial of fetal surgery for moderate left diaphragmatic hernia. N Engl J Med. 385(2):119-29, 2021
                                    2. Koh JY et al: Functional and structural evaluation in the lungs of children with repaired congenital diaphragmatic hernia. BMC Pediatr. 21(1):120, 2021
                                    3. Mukthapuram S et al: MRI assessment of pulmonary vascularity in infants with congenital diaphragmatic hernia: a novel tool for direct assessment of severity of pulmonary hypertension and hypoplasia. J Pediatr. ePub, 2021
                                    4. Beel E et al: Chest CT scoring for evaluation of lung sequelae in congenital diaphragmatic hernia survivors. Pediatr Pulmonol. 55(3):740-6, 2020
                                    5. Mehollin-Ray AR: Congenital diaphragmatic hernia. Pediatr Radiol. 50(13):1855-71, 2020
                                    6. Verla MA et al: Prenatal imaging features and postnatal factors associated with gastrointestinal morbidity in congenital diaphragmatic hernia. Fetal Diagn Ther. 47(4):252-60, 2020
                                    7. Yu PT et al: The role of ECMO in the management of congenital diaphragmatic hernia. Semin Perinatol. 44(1):151166, 2020
                                    8. Adaikalam SA et al: Neonatal lung growth in congenital diaphragmatic hernia: evaluation of lung density and mass by pulmonary MRI. Pediatr Res. 86(5):635-40, 2019
                                    9. Corsini I et al: Lung ultrasound findings in congenital diaphragmatic hernia. Eur J Pediatr. 178(4):491-5, 2019
                                    10. Hosokawa T et al: Postnatal ultrasound to determine the surgical strategy for congenital diaphragmatic hernia. J Ultrasound Med. 38(9):2347-58, 2019
                                    11. Oliver ER et al: Congenital diaphragmatic hernia sacs: prenatal imaging and associated postnatal outcomes. Pediatr Radiol. 49(5):593-9, 2019
                                    12. Victoria T et al: Right congenital diaphragmatic hernias: is there a correlation between prenatal lung volume and postnatal survival, as in isolated left diaphragmatic hernias? Fetal Diagn Ther. 43(1):12-8, 2018
                                    13. Puligandla PS et al: Management of congenital diaphragmatic hernia: a systematic review from the APSA outcomes and evidence based practice committee. J Pediatr Surg. 50(11):1958-70, 2015
                                    14. Danzer E et al: Controversies in the management of severe congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 19(6):376-84, 2014
                                    15. Harting MT et al: The congenital diaphragmatic hernia study group registry update. Semin Fetal Neonatal Med. 19(6):370-5, 2014
                                    16. Slavotinek AM: The genetics of common disorders - congenital diaphragmatic hernia. Eur J Med Genet. 57(8):418-23, 2014
                                    17. Tracy S et al: Multidisciplinary long-term follow-up of congenital diaphragmatic hernia: a growing trend. Semin Fetal Neonatal Med. 19(6):385-91, 2014
                                    18. Wynn J et al: Genetic causes of congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 19(6):324-30, 2014
                                    19. Josephsen JB et al: Strangulated bowel as an unusual complication of neonatal congenital diaphragmatic hernia: a case report and literature review. J Pediatr Surg. 48(4):866-8, 2013
                                    20. Mehta A et al: Splenic torsion after congenital diaphragmatic hernia repair: case report and review of the literature. J Pediatr Surg. 48(3):e29-31, 2013
                                    21. Victoria T et al: Use of ultrasound and MRI for evaluation of lung volumes in fetuses with isolated left congenital diaphragmatic hernia. Semin Pediatr Surg. 22(1):30-6, 2013
                                    22. Lee TC et al: Late gestation fetal magnetic resonance imaging-derived total lung volume predicts postnatal survival and need for extracorporeal membrane oxygenation support in isolated congenital diaphragmatic hernia. J Pediatr Surg. 46(6):1165-71, 2011
                                    23. Sandstrom CK et al: Diaphragmatic hernias: a spectrum of radiographic appearances. Curr Probl Diagn Radiol. 40(3):95-115, 2011
                                    24. Taylor GA et al: Imaging of congenital diaphragmatic hernias. Pediatr Radiol. 39(1):1-16, 2009
                                    25. Barnewolt CE et al: Percent predicted lung volumes as measured on fetal magnetic resonance imaging: a useful biometric parameter for risk stratification in congenital diaphragmatic hernia. J Pediatr Surg. 42(1):193-7, 2007
                                    26. Donnelly LF et al: Correlation between findings on chest radiography and survival in neonates with congenital diaphragmatic hernia. AJR Am J Roentgenol. 173(6):1589-93, 1999
                                    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

                                            TERMINOLOGY

                                            • Definitions

                                              • Congenital diaphragmatic hernia (CDH): Herniation of abdominal contents into chest via developmental defect in diaphragm
                                                • Intrapleural hernia (80%): Worse prognosis
                                                  • Bochdalek (posterolateral) is most common subtype
                                                • Mediastinal hernia (20%)
                                                  • Anteriorly involves central tendon of diaphragm
                                                    • Morgagni hernia: Smaller paramedian subtype
                                                  • Posteriorly may involve hiatus of esophagus or IVC

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • Ultrasonographic Findings

                                                  • MR Findings

                                                    • CT Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Consider

                                                                      Selected References

                                                                      1. Deprest JA et al: Randomized trial of fetal surgery for moderate left diaphragmatic hernia. N Engl J Med. 385(2):119-29, 2021
                                                                      2. Koh JY et al: Functional and structural evaluation in the lungs of children with repaired congenital diaphragmatic hernia. BMC Pediatr. 21(1):120, 2021
                                                                      3. Mukthapuram S et al: MRI assessment of pulmonary vascularity in infants with congenital diaphragmatic hernia: a novel tool for direct assessment of severity of pulmonary hypertension and hypoplasia. J Pediatr. ePub, 2021
                                                                      4. Beel E et al: Chest CT scoring for evaluation of lung sequelae in congenital diaphragmatic hernia survivors. Pediatr Pulmonol. 55(3):740-6, 2020
                                                                      5. Mehollin-Ray AR: Congenital diaphragmatic hernia. Pediatr Radiol. 50(13):1855-71, 2020
                                                                      6. Verla MA et al: Prenatal imaging features and postnatal factors associated with gastrointestinal morbidity in congenital diaphragmatic hernia. Fetal Diagn Ther. 47(4):252-60, 2020
                                                                      7. Yu PT et al: The role of ECMO in the management of congenital diaphragmatic hernia. Semin Perinatol. 44(1):151166, 2020
                                                                      8. Adaikalam SA et al: Neonatal lung growth in congenital diaphragmatic hernia: evaluation of lung density and mass by pulmonary MRI. Pediatr Res. 86(5):635-40, 2019
                                                                      9. Corsini I et al: Lung ultrasound findings in congenital diaphragmatic hernia. Eur J Pediatr. 178(4):491-5, 2019
                                                                      10. Hosokawa T et al: Postnatal ultrasound to determine the surgical strategy for congenital diaphragmatic hernia. J Ultrasound Med. 38(9):2347-58, 2019
                                                                      11. Oliver ER et al: Congenital diaphragmatic hernia sacs: prenatal imaging and associated postnatal outcomes. Pediatr Radiol. 49(5):593-9, 2019
                                                                      12. Victoria T et al: Right congenital diaphragmatic hernias: is there a correlation between prenatal lung volume and postnatal survival, as in isolated left diaphragmatic hernias? Fetal Diagn Ther. 43(1):12-8, 2018
                                                                      13. Puligandla PS et al: Management of congenital diaphragmatic hernia: a systematic review from the APSA outcomes and evidence based practice committee. J Pediatr Surg. 50(11):1958-70, 2015
                                                                      14. Danzer E et al: Controversies in the management of severe congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 19(6):376-84, 2014
                                                                      15. Harting MT et al: The congenital diaphragmatic hernia study group registry update. Semin Fetal Neonatal Med. 19(6):370-5, 2014
                                                                      16. Slavotinek AM: The genetics of common disorders - congenital diaphragmatic hernia. Eur J Med Genet. 57(8):418-23, 2014
                                                                      17. Tracy S et al: Multidisciplinary long-term follow-up of congenital diaphragmatic hernia: a growing trend. Semin Fetal Neonatal Med. 19(6):385-91, 2014
                                                                      18. Wynn J et al: Genetic causes of congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 19(6):324-30, 2014
                                                                      19. Josephsen JB et al: Strangulated bowel as an unusual complication of neonatal congenital diaphragmatic hernia: a case report and literature review. J Pediatr Surg. 48(4):866-8, 2013
                                                                      20. Mehta A et al: Splenic torsion after congenital diaphragmatic hernia repair: case report and review of the literature. J Pediatr Surg. 48(3):e29-31, 2013
                                                                      21. Victoria T et al: Use of ultrasound and MRI for evaluation of lung volumes in fetuses with isolated left congenital diaphragmatic hernia. Semin Pediatr Surg. 22(1):30-6, 2013
                                                                      22. Lee TC et al: Late gestation fetal magnetic resonance imaging-derived total lung volume predicts postnatal survival and need for extracorporeal membrane oxygenation support in isolated congenital diaphragmatic hernia. J Pediatr Surg. 46(6):1165-71, 2011
                                                                      23. Sandstrom CK et al: Diaphragmatic hernias: a spectrum of radiographic appearances. Curr Probl Diagn Radiol. 40(3):95-115, 2011
                                                                      24. Taylor GA et al: Imaging of congenital diaphragmatic hernias. Pediatr Radiol. 39(1):1-16, 2009
                                                                      25. Barnewolt CE et al: Percent predicted lung volumes as measured on fetal magnetic resonance imaging: a useful biometric parameter for risk stratification in congenital diaphragmatic hernia. J Pediatr Surg. 42(1):193-7, 2007
                                                                      26. Donnelly LF et al: Correlation between findings on chest radiography and survival in neonates with congenital diaphragmatic hernia. AJR Am J Roentgenol. 173(6):1589-93, 1999