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Imaging of congenital diaphragmatic hernias

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Abstract

Congenital diaphragmatic hernias are complex and life-threatening lesions that are not just anatomic defects of the diaphragm, but represent a complex set of physiologic derangements of the lung, the pulmonary vasculature, and related structures. Imaging plays an increasingly important role in the care of these infants. Prenatal sonography and MRI have allowed early and accurate identification of the defect and associated anomalies. These tools have also been the key to defining the degree of pulmonary hypoplasia and to predicting neonatal survival and need for aggressive respiratory rescue strategies. In the postnatal period, conventional radiography supplemented by cross-sectional imaging in selected cases can be very useful in sorting out the differential diagnosis of intrathoracic masses, in the detection of associated anomalies, and in the management of complications. Understanding the pathogenesis of diaphragmatic defects, the underlying physiologic disturbances, and the strengths and limitations of current imaging protocols is essential to the effective and accurate management of these complex patients.

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References

  1. Cohen MS, Rychik J, Bush DM et al (2002) Influence of congenital heart disease on survival in children with congenital diaphragmatic hernia. J Pediatr 141:25–30

    Article  PubMed  Google Scholar 

  2. Stege G, Fenton A, Jaffray B (2003) Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia. Pediatrics 112:532–535

    Article  PubMed  Google Scholar 

  3. Skandalakis JE, Gray SW, Ricketts RR (1994) The diaphragm. In: Skandalakis JE, Gray SW (eds) Embryology for surgeons, 2nd edn. Williams & Wilkins, Baltimore, pp 491–539

    Google Scholar 

  4. Stolar CJH (1997) Congenital diaphragmatic hernia. In: Oldham KT, Colombani PM, Foglia RP (eds) Surgery of infants and children: scientific principles and practice. Lippincott-Raven, Philadelphia, pp 883–895

    Google Scholar 

  5. Pringle KC, Turner JW, Schofield JC et al (1984) Creation and repair of diaphragmatic hernia: lung development and morphology. J Pediatr Surg 19:131–140

    Article  PubMed  CAS  Google Scholar 

  6. Clugston RD, Klattig J, Englert C et al (2006) Teratogen-induced, dietary and genetic models of congenital diaphragmatic hernia share a common mechanism of pathogenesis. Am J Pathol 169:1541–1549

    Article  PubMed  CAS  Google Scholar 

  7. Bielinska M, Jay PY, Erlich JM et al (2007) Molecular genetics of congenital diaphragmatic defects. Ann Med 39:261–274

    Article  PubMed  CAS  Google Scholar 

  8. Sharma S, Jain R, Singh MK et al (2007) A case of congenital diaphragmatic hernia with a hernia sac attached to the liver: hints for an early embryological insult. J Pediatr Surg 42:1761–1763

    Article  PubMed  Google Scholar 

  9. Greer JJ, Cote D, Allan DW et al (2000) Structure of the primordial diaphragm and defects associated with nitrofen-induced CDH. J Appl Physiol 89:2123–2129

    PubMed  CAS  Google Scholar 

  10. Babiuk RP, Greer JJ (2002) Diaphragm defects occur in a CDH hernia model independently of myogenesis and lung formation. Am J Physiol Lung Cell Mol Physiol 283:L1310–L1314

    PubMed  CAS  Google Scholar 

  11. Garne E, Haeusler M, Barisic I et al (2002) Congenital diaphragmatic hernia: evaluation of prenatal diagnosis in 20 European regions. Ultrasound Obstet Gynecol 19:329–333

    Article  PubMed  CAS  Google Scholar 

  12. Seaward GR (2005) The fetal chest. In: Rumack CM, Wilson SR, Charboneau JW (eds) Diagnostic ultrasound, 3rd edn. Elsevier Mosby, St. Louis, pp 1303–1321

    Google Scholar 

  13. Bootstaylor BS, Filly RA, Harrison MR et al (1995) Prenatal sonographic predictors of liver herniation in congenital diaphragmatic hernia. J Ultrasound Med 14:515–520

    PubMed  CAS  Google Scholar 

  14. Ruano R, Benachi A, Joubin L et al (2004) Three-dimensional ultrasonographic assessment of fetal lung volume as prognostic factor in isolated congenital diaphragmatic hernia. BJOG 111:423–429

    Article  PubMed  Google Scholar 

  15. Jani JC, Cannie M, Peralta CFA et al (2007) Congenital diaphragmatic hernia: comparison of 3D US and MR imaging assessments. Radiology 244:575–582

    Article  PubMed  Google Scholar 

  16. Levine D, Barnewolt CE, Mehta TS et al (2003) Fetal thoracic abnormalities: MR imaging. Radiology 228:379–388

    Article  PubMed  Google Scholar 

  17. Hubbard AM, Adzick NS, Crombleholme TM et al (1999) Congenital chest lesions: diagnosis and characterization with prenatal MR imaging. Radiology 212:43–48

    PubMed  CAS  Google Scholar 

  18. Barnewolt CE, Kunisaki SM, Fauza DO et al (2007) 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 42:193–197

    Article  PubMed  Google Scholar 

  19. Bonfils M, Emeriaud G, Durand C (2006) Fetal lung volume in congenital diaphragmatic hernia. Arch Dis Child Fetal Neonatal Ed 91:F363–F364

    Article  PubMed  CAS  Google Scholar 

  20. Coakley FY, Hricak H, Filly RA et al (1999) Complex fetal disorders: effect of MR imaging on management – preliminary clinical experience. Radiology 213:691–696

    PubMed  CAS  Google Scholar 

  21. Rypens F, Metens T, Rocourt N et al (2001) Fetal lung volume: estimation at MR imaging – initial results. Radiology 219:236–241

    PubMed  CAS  Google Scholar 

  22. Büsing KA, Kilian K, Schaible T et al (2007) Reliability and validity of MR image lung volume measurement in fetuses with congenital diaphragmatic hernia and in vitro lung models. Radiology 246:553–561

    Article  PubMed  Google Scholar 

  23. Enns GM, Cox VA, Goldstein RB et al (1998) Congenital diaphragmatic defects and associated syndromes, malformations, and chromosome anomalies: a retrospective study of 60 patients and literature review. Am J Med Genet 79:215–225

    Article  PubMed  CAS  Google Scholar 

  24. Migliazza L, Otten C, Xia H et al (1999) Cardiovascular malformations in congenital diaphragmatic hernia: human and experimental studies. J Pediatr Surg 34:1352–1358

    Article  PubMed  CAS  Google Scholar 

  25. Fauza DO, Wilson JM (1994) Congenital diaphragmatic hernia and associated anomalies: their incidence, identification, and impact on prognosis. J Pediatr Surg 29:1113–1117

    Article  PubMed  CAS  Google Scholar 

  26. Graziano JN (2005) Cardiac anomalies in patients with congenital diaphragmatic hernia and their prognosis: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg 40:1045–1049

    Article  PubMed  Google Scholar 

  27. Grethel EJ, Farrell J, Ball R et al (2007) Congenital diaphragmatic hernia associated with spinal anomalies. Obstet Gynecol 209:485–488

    Google Scholar 

  28. Slavotinek AM, Warmerdam B, Lin AE et al (2007) Population-based analysis of left- and right-sided diaphragmatic hernias demonstrates different frequencies of selected additional anomalies. Am J Med Genet 143A:3127–3136

    Article  PubMed  Google Scholar 

  29. Witters I, Legius E, Moerman P et al (2001) Associated malformations and chromosomal anomalies in 42 cases of prenatally diagnosed diaphragmatic hernia. Am J Med Genet 103:278–282

    Article  PubMed  CAS  Google Scholar 

  30. Metkus AP, Filly RA, Stringer MD et al (1996) Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg 31:148–151

    Article  PubMed  CAS  Google Scholar 

  31. Walsh DS, Hubbard AM, Olutoye OO et al (2000) Assessment of fetal lung volumes and liver herniation with magnetic resonance imaging in congenital diaphragmatic hernia. Am J Obstet Gynecol 183:1067–1069

    Article  PubMed  CAS  Google Scholar 

  32. Laudy JAM, Van Gucht M, van Dooren MF et al (2003) Congenital diaphragmatic hernia: an evaluation of the prognostic value of the lung-to-head ratio and other prenatal parameters. Prenat Diagn 23:634–639

    Article  PubMed  CAS  Google Scholar 

  33. Datin-Dorriere V, Rouzies S, Taupin P et al (2008) Prenatal prognosis in isolated congenital diaphragmatic hernia. Am J Obstet Gynecol 198:80.e1–80.e5

    Article  Google Scholar 

  34. Sbragia L, Paek BW, Filly RA et al (2000) Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival? J Ultrasound Med 19:845–848

    PubMed  CAS  Google Scholar 

  35. O’Rourke PP (1993) Congenital diaphragmatic hernia: are there reliable clinical predictors? Crit Care Med 21:S380–S381

    Article  PubMed  CAS  Google Scholar 

  36. Hedrick H, Danzer E, Merchant A et al (2007) Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia. Am J Obstet Gynecol 197:422.e1–422.e4

    Article  Google Scholar 

  37. Ruano R, Benachi A, Aubry M-C et al (2003) Prenatal sonographic diagnosis of congenital hiatal hernia. Prenat Diagn 24:26–30

    Article  Google Scholar 

  38. Gorincour G, Bouvenot J, Mourot MG et al (2005) Prenatal prognosis of congenital diaphragmatic hernia using magnetic resonance imaging measurement of fetal lung volume. Ultrasound Obstet Gynecol 26:738–744

    Article  PubMed  CAS  Google Scholar 

  39. Wilson J, DiFiore JW, Peters CA (1993) Experimental fetal tracheal ligation prevents the pulmonary hypoplasia associated with fetal nephrectomy: possible application for congenital diaphragmatic hernia. J Pediatr Surg 28:1433–1439

    Article  PubMed  CAS  Google Scholar 

  40. DiFiore JW, Fauza DO, Slavin R et al (1994) Experimental fetal tracheal ligation reverses the structural and physiological effects of pulmonary hypoplasia in congenital diaphragmatic hernia. J Pediatr Surg 29:248–256

    Article  PubMed  CAS  Google Scholar 

  41. Bohn D (2006) Diaphragmatic hernia – pre and postnatal. Paediatr Respir Rev 7 [Suppl 1]:S249–S250

    Article  PubMed  Google Scholar 

  42. Sakurai M, Donnelly LF, Klosterman LA et al (2000) Congenital diaphragmatic hernia in neonates: variations in umbilical catheter and enteric tube position. Radiology 216:112–116

    PubMed  CAS  Google Scholar 

  43. Holt PD, Arkovitz MS, Berdon WE et al (2004) Newborns with diaphragmatic hernia: initial chest radiography does not have a role in predicting clinical outcome. Pediatr Radiol 34:462–464

    Article  PubMed  Google Scholar 

  44. Treves ST, Packard AB (2007) Lungs. In: Treves ST (ed) Pediatric nuclear medicine, 3rd edn. Springer, New York, pp 87–127

    Google Scholar 

  45. Wohl ME, Griscom NT, Strieder DJ et al (1977) The lung following repair of congenital diaphragmatic hernia. J Pediatr 90:405–414

    Article  PubMed  CAS  Google Scholar 

  46. Falconer AR, Brown RA, Helms P et al (1990) Pulmonary sequelae in survivors of congenital diaphragmatic hernia. Thorax 45:126–129

    Article  PubMed  CAS  Google Scholar 

  47. Van Meurs KP, Newman KD, Anderson KD et al (1990) Effect of extracorporeal membrane oxygenation on survival of infants with congenital diaphragmatic hernia. J Pediatr 117:954–960

    Article  PubMed  Google Scholar 

  48. Trachsel D, Selvadurai H, Bohn D et al (2005) Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia. Pediatr Pulmonol 39:433–439

    Article  PubMed  Google Scholar 

  49. Rais-Bahrami K, Robbins ST, Reed VL et al (1995) Congenital diaphragmatic hernia. Outcome of preoperative extracorporeal membrane oxygenation. Clin Pediatr (Phila) 34:471–474

    Article  CAS  Google Scholar 

  50. Downard CD, Jaksic T, Garza JJ et al (2003) Analysis of an improved survival rate for congenital diaphragmatic hernia. J Pediatr Surg 38:729–732

    Article  PubMed  Google Scholar 

  51. Frenckner B, Ehren H, Granholm T et al (1997) Improved results in patients who have congenital diaphragmatic hernia using preoperative stabilization, extracorporeal membrane oxygenation, and delayed surgery. J Pediatr Surg 32:1185–1189

    Article  PubMed  CAS  Google Scholar 

  52. Boloker J, Bateman DA, Wung JT et al (2002) Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous respiration/elective repair. J Pediatr Surg 37:357–366

    Article  PubMed  Google Scholar 

  53. Brown RA, Bösenberg AT (2007) Evolving management of congenital diaphragmatic hernia. Paediatr Anaesth 17:713–719

    Article  PubMed  Google Scholar 

  54. Brant-Zawadzki PB, Fenton SJ, Nichol PF et al (2007) The split abdominal wall muscle flap repair for large congenital diaphragmatic hernias on extracorporeal membrane oxygenation. J Pediatr Surg 42:1047–1050

    Article  PubMed  Google Scholar 

  55. Skari H, Bjornland K, Egeland T et al (2000) Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 35:1187–1197

    Article  PubMed  CAS  Google Scholar 

  56. Lund DP, Mitchell J, Kharasch V et al (1994) Congenital diaphragmatic hernia: the hidden morbidity. J Pediatr Surg 29:258–262

    Article  PubMed  CAS  Google Scholar 

  57. Arensman RM, Bambini DA (2000) Congenital diaphragmatic hernia and eventration. In: Ashcraft KW (ed) Pediatric surgery, 3rd edn. Saunders, Philadelphia, pp 300–317

    Google Scholar 

  58. Robnett-Filly B, Goldstein RB, Sampior D et al (2003) Morgagni hernia. A rare form of congenital diaphragmatic hernia. J Ultrasound Med 22:537–539

    PubMed  Google Scholar 

  59. Karpelowsky JS, Wieselthaler N, Rode H (2006) Primary paraesophageal hernia. J Pediatr Surg 41:1588–1593

    Article  PubMed  Google Scholar 

  60. Pokorny W, McGill C, Halberg F (1984) Morgagni hernia during infancy: presentation and associated anomalies. J Pediatr Surg 19:394–397

    Article  PubMed  CAS  Google Scholar 

  61. de Fonseca J, Davies M, Bolton K (1987) Congenital hydropericardium associated with the herniation of part of the liver into the pericardial sac. J Pediatr Surg 22:851–853

    Article  PubMed  Google Scholar 

  62. Cantrell J, Haller J, Ravitch M (1958) A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 107:602–614

    PubMed  CAS  Google Scholar 

  63. Imamoglu M, Çay A, Kosucu P et al (2005) Congenital paraesophageal hiatal hernia: pitfalls in the diagnosis and treatment. J Pediatr Surg 40:112–113

    Google Scholar 

  64. Al-Salem AH (2000) Congenital paraesophageal hernia in infancy and childhood. Saudi Med J 21:164–167

    PubMed  CAS  Google Scholar 

  65. Mei-Zahav M, Solomon M, Trachsel D et al (2003) Bochdalek diaphragmatic hernia: not only a neonatal disease. Arch Dis Child 88:532–535

    Article  PubMed  CAS  Google Scholar 

  66. Ramos CT, Koplewits BZ, Babyn PS et al (2000) What have we learned about traumatic diaphragmatic hernias in children? J Pediatr Surg 35:601–604

    Article  PubMed  CAS  Google Scholar 

  67. Cigdem MK, Onen A, Otcu S et al (2007) Late presentation of Bochdalek-type congenital diaphragmatic hernia in children: a 23-year experience at a single center. Surg Today 37:642–645

    Article  PubMed  Google Scholar 

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Author notes

  1. Omolola M. Atalabi

    Present address: The College of Medicine/University College Hospital, Ibadan, Nigeria

Authors and Affiliations

  1. Department of Radiology, Children’s Hospital Boston, Harvard Medical School, 300 Longwood Ave., Boston, MA, 02115, USA

    George A. Taylor, Omolola M. Atalabi & Judy A. Estroff

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  1. George A. Taylor
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Correspondence to George A. Taylor.

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Taylor, G.A., Atalabi, O.M. & Estroff, J.A. Imaging of congenital diaphragmatic hernias. Pediatr Radiol 39, 1–16 (2009). https://doi.org/10.1007/s00247-008-0917-7

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