The Indian Journal of Pediatrics

, Volume 77, Issue 6, pp 673–678 | Cite as

Recent advances in the management of congenital diaphragmatic hernia

  • V. Jain
  • S. Agarwala
  • V. Bhatnagar
Special Article


The management of congenital diaphragmatic hernia (CDH) is undergoing continual change and refinement, fuelled by recent advances in this field. Although many studies have documented the benefits of these recent advances but definite recommendations are lacking. Also, injudicious use of some of these strategies may be counterproductive, underscoring the importance of evidence based treatment strategy. This article discusses the utility of the recent advances in the management of CDH.

Key words

Congenital diaphragmatic hernia Surgical repair Recent advances Respiratory support Extra corporeal membrane oxygenation High frequency ventilation Intra uterine management 


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  1. 1.
    Swenson O. Chalasia, achalasia, hiatus hernia, and diaphragmatic hernia. In Swenson O, ed. Pediatr Surg, 2nd ed. New York; Appleton-Century-Crofts Inc, 1962;189–201.Google Scholar
  2. 2.
    Harrison MR, Bjordal RI, Langmark F, Knutrud O. Congenital diaphragmatic hernia: The hidden mortality. J Pediatr Surg 1978;13:227–230.CrossRefPubMedGoogle Scholar
  3. 3.
    Drummond W, Gregory G, Heyman M. The effect of alkalosis on pulmonary artery pressure, systemic artery pressure and PaO2 in infants with persistent pulmonary hypertension. Pediatr Res 1979;13:493–495.Google Scholar
  4. 4.
    Wung JT, James LS, Kilchevsky E, James E. Management of infants with severe respiratory failure and persistence of the fetal circulation, without hyperventilation. Pediatrics 1985;76:488–494.PubMedGoogle Scholar
  5. 5.
    Wung JT, Sahni R, Moffitt ST, Lipsitz E, Stolar CJ. Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration, and no chest tube. J Pediatr Surg 1995;30:406–409.CrossRefPubMedGoogle Scholar
  6. 6.
    Shaffer TH, Wolfson MR, Clark Jr L. State of the art review: liquid ventilation. Pediatr Pulmonol 1992;14:102–109.CrossRefPubMedGoogle Scholar
  7. 7.
    Hirschl RB. Current experience with Liquid Ventilation. Pediatr Respir Rev 2004;5:S339–S345.CrossRefGoogle Scholar
  8. 8.
    Mohseni-Bod H, Bohn D. Pulmonary hypertension in congenital diaphragmatic hernia. Semin Pediatr Surg 2007;16:126–133.CrossRefPubMedGoogle Scholar
  9. 9.
    Neonatal Inhaled Nitric Oxide Study Group. (No authors listed). Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic respiratory failure. Pediatrics 1997;99:838–845.CrossRefGoogle Scholar
  10. 10.
    Kinsella JP, Ivy DD, Abman SH. Pulmonary vasodilator therapy in congenital diaphragmatic hernia: acute, late, and chronic pulmonary hypertension. Semin Perinatol 2005;29:123–128.CrossRefPubMedGoogle Scholar
  11. 11.
    Noori S, Friedlich P, Wong P, Garingo A, Seri I. Cardiovascular effects of sildenafil in neonates and infants with congenital diaphragmatic hernia and pulmonary hypertension. Neonatology 2007;91:92–100.CrossRefPubMedGoogle Scholar
  12. 12.
    Shiyanagi S, Okazaki T, Shoji H et al. Management of pulmonary hypertension in congenital diaphragmatic hernia: nitric oxide with prostaglandin-E1 vs nitric oxide alone. Pediatr Surg Int 2008;24:1101–1104.CrossRefPubMedGoogle Scholar
  13. 13.
    van den Hout L, Sluiter I, Gischler S et al. Can we improve outcome of congenital diaphragmatic hernia? Pediatr Surg Int 2009;25:733–743.CrossRefPubMedGoogle Scholar
  14. 14.
    Abe K, Shimokawa H, Morikawa K et al. Long-term treatment with a Rho-kinase inhibitor improves monocrotaline-induced fatal pulmonary hypertension in rats. Circ Res 2004;94:385–393.CrossRefPubMedGoogle Scholar
  15. 15.
    Khan AM, Lally KP. The role of extracorporeal membrane oxygenation in the management of infants with congenital diaphragmatic hernia. Semin Perinatol 2005;29:118–122.CrossRefPubMedGoogle Scholar
  16. 16.
    Lally KP. Extracorporeal membrane oxygenation in patients with congenital diaphragmatic hernia. Semin Pediatr Surg 1996;5:249–255.PubMedGoogle Scholar
  17. 17.
    Bahrami KR, Van Meurs KP. ECMO for neonatal respiratory failure. Semin Perinatol 2005;29:15–23.CrossRefPubMedGoogle Scholar
  18. 18.
    Kugelman A, Gangitano E, Pincros J, Tantivit P, Taschuk R, Durand M. Venovenous versus venoarterial extracorporeal membrane oxygenation in congenital diaphragmatic hernia. J Pediatr Surg 2003;38:1131–1136.CrossRefPubMedGoogle Scholar
  19. 19.
    Elbourne D, Field D, Mugford M. Extracorporeal membrane oxygenation for severe respiratory failure in newborn infants. Cochrane Database Syst Rev 2002:CD001340.Google Scholar
  20. 20.
    The Congenital Diaphragmatic Hernia Study Group. (No authors listed). Does extracorporeal membrane oxygenation improve survival in neonates with congenital diaphragmatic hernia? J Pediatr Surg 1999;34:720–724.CrossRefGoogle Scholar
  21. 21.
    Cogo PE, Zimmermann LJ, Meneghini L et al. Pulmonary surfactant disaturated-phosphatidylcholine (DSPC) turnover and pool size in newborn infants with congenital diaphragmatic hernia (CDH). Pediatr Res 2003;54:653–658.CrossRefPubMedGoogle Scholar
  22. 22.
    Lotze A, Knight GR, Anderson KD et al. Surfactant (beractant) therapy for infants with congenital diaphragmatic hernia on ECMO: evidence of persistent surfactant deficiency. J Pediatr Surg 1994;29:407–412.CrossRefPubMedGoogle Scholar
  23. 23.
    Van Meurs K. Congenital Diaphragmatic Hernia Study Group. Is surfactant therapy beneficial in the treatment of the term newborn infant with congenital diaphragmatic hernia? J Pediatr 2004;145:312–316.CrossRefPubMedGoogle Scholar
  24. 24.
    Moya FR, Lally KP. Evidence-based management of infants with congenital diaphragmatic hernia. Semin Perinatol 2005;29:112–117.CrossRefPubMedGoogle Scholar
  25. 25.
    Sakai H, Tamura M, Hosokawa Y, Bryan AC, Barker GA, Bohn DJ. Effect of surgical repair on respiratory mechanics in congenital diaphragmatic hernia. J Pediatr 1987;111:432–438.CrossRefPubMedGoogle Scholar
  26. 26.
    de la Hunt MN, Madden N, Scott JE et al. Is delayed surgery really better for congenital diaphragmatic hernia?: a prospective randomized clinical trial. J Pediatr Surg 1996;31:1554–1556.CrossRefPubMedGoogle Scholar
  27. 27.
    Nio M, Haase G, Kennaugh J, Bui K, Atkinson JB. A prospective randomized trial of delayed versus immediate repair of congenital diaphragmatic hernia. J Pediatr Surg 1994;29:618–621.CrossRefPubMedGoogle Scholar
  28. 28.
    Moyer V, Moya F, Tibboel R, Losty P, Nagaya M, Lally KP. Late versus early surgical correction for congenital diaphragmatic hernia in newborn infants. Cochrane Database Syst Rev 2002;CD001695.Google Scholar
  29. 29.
    de Buys Roessingh AS, Dinh-Xuan AT. Congenital diaphragmatic hernia: current status and review of the literature. Eur J Pediatr 2009;168:393–406.CrossRefPubMedGoogle Scholar
  30. 30.
    Kilian AK, Büsing KA, Schuetz EM, Schaible T, Neff KW et al. Fetal MR lung volumetry in congenital diaphragmatic hernia (CDH): prediction of clinical outcome and the need for extracorporeal membrane oxygenation (ECMO). Klin Padiatr 2009;221:295–301.CrossRefPubMedGoogle Scholar
  31. 31.
    Takahashi S, Oishi Y, Ito N et al. Evaluating mortality and disease severity in congenital diaphragmatic hernia using the McGoon and pulmonary artery indices. J Pediatr Surg 2009;44:2101–2106.CrossRefPubMedGoogle Scholar
  32. 32.
    Sinha CK, Islam S, Patel S, Nicolaides K, Greenough A, Davenport M. Congenital diaphragmatic hernia: prognostic indices in the fetal endoluminal tracheal occlusion era. J Pediatr Surg 2009;44:312–316.CrossRefPubMedGoogle Scholar
  33. 33.
    Harrison MR, Adzick NS, Bullard KM et al. Correction of congenital diaphragmatic hernia in utero VII: a prospective trial. J Pediatr Surg 1997;32:1637–1642.CrossRefPubMedGoogle Scholar
  34. 34.
    Kitano Y. Prenatal intervention for congenital diaphragmatic hernia. Semin Pediatr Surg 2007;16:101–108.CrossRefPubMedGoogle Scholar
  35. 35.
    Cass DL. Fetal surgery for congenital diaphragmatic hernia: the North American experience. Semin Perinatol 2005;29:104–111.CrossRefPubMedGoogle Scholar
  36. 36.
    Flake AW, Crombleholme TM, Johnson MP, Howell LJ, Adzick NS. Treatment of severe congenital diaphragmatic hernia by fetal tracheal occlusion: clinical experience with fifteen cases. Am J Obstet Gynecol 2000;183:1059–1066.CrossRefPubMedGoogle Scholar
  37. 37.
    Harrison MR, Keller RL, Hawgood SB et al. A randomized trial of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia. N Engl J Med 2003;349:1916–1924.CrossRefPubMedGoogle Scholar
  38. 38.
    Deprest J, Jani J, Van Schoubroeck D et al. Current consequences of prenatal diagnosis of congenital diaphragmatic hernia. J Pediatr Surg 2006;41:423–430.CrossRefPubMedGoogle Scholar
  39. 39.
    Jani JC, Nicolaides KH, Gratacós E et al. Severe diaphragmatic hernia treated by fetal endoscopic tracheal occlusion. Ultrasound Obstet Gynecol 2009;34:304–310.CrossRefPubMedGoogle Scholar
  40. 40.
    van der Zee DC, Bax NM. Laparoscopic repair of congenital diaphragmatic hernia in a 6-month-old child. Surg Endosc 1995;9:1001–1003.PubMedGoogle Scholar
  41. 41.
    Arca MJ, Barnhart DC, Lelli JL Jr et al. Early experience with minimally invasive repair of congenital diaphragmatic hernias: results and lessons learned. J Pediatr Surg 2003;38:1563–1568.CrossRefPubMedGoogle Scholar
  42. 42.
    Van Meurs KP, Rhine WD, Benitz WE et al. Lobar lung transplantation as a treatment for congenital diaphragmatic hernia. J Pediatr Surg 1994;29:1557–1560.CrossRefPubMedGoogle Scholar
  43. 43.
    Bhat YR, Kumar V, Rao A. Congenital diaphragmatic hernia in a developing country. Singapore Med J 2008;49:715–718.PubMedGoogle Scholar
  44. 44.
    Jain A, Singh V, Sharma M. Congenital diaphragmatic hernia: our experience - a brief review. Indian J Anaesth 2002;46:426–429.Google Scholar
  45. 45.
    Pandey A, Tandon RK, Kureel SN, Wakhlu A, Rawat J. Evaluation of congenital diaphragmatic hernia in a tertiary health center of a developing country: management and survival. Hernia 2008;12:189–192.CrossRefPubMedGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2010

Authors and Affiliations

  1. 1.Department of Pediatric SurgeryAll India Institute of Medical SciencesNew DelhiIndia
  2. 2.Department of Pediatric SurgeryAll India Institute of Medical SciencesNew DelhiIndia

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