Skip to main content

Advertisement

SpringerLink
Log in

Current Concepts in the Management of Congenital Diaphragmatic Hernia in Infants

  • REVIEW ARTICLE
  • Published:
Indian Journal of Surgery Aims and scope Submit manuscript

Abstract

The therapeutic approach to congenital diaphragmatic hernia (CDH) has shifted from one of immediate repair to management of pulmonary hypertension, physiologic stabilization, and delayed surgical repair. Lung hypoplasia, remodeled pulmonary vasculature, and ventricular dysfunction all contribute to the high morbidity and mortality associated with CDH. In addition, genetic syndromes associated with CDH can increase the incidence of serious anomalies and hence impact survival. Prenatal and postnatal management of infants with CDH is challenging in the best of circumstances and need multidisciplinary teams for optimal outcomes. However, advances using ultrasound and fetal MRI can predict prognosis and survival and plan for postnatal management. Survival rates for patients with CDH have increased for the past decade with better management at resuscitation; implementation of gentle ventilation strategies; and medical management of pulmonary hypertension, physiologic stabilization, and extracorporeal membrane oxygenation. However, follow-up of these infants for long-term morbidities is essential for optimal outcomes after discharge.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C et al (2012) Congenital diaphragmatic hernia. Eur Respir J 39:820–9

    Article  PubMed  CAS  Google Scholar 

  2. Wynn J, Yu L, Chung WK (2014) Genetic causes of congenital diaphragmatic hernia. Semin Fetal Neonatal Med 19:324–30

    Article  PubMed  PubMed Central  Google Scholar 

  3. Gallot D, Marceau G, Coste K, Hadden H, Robert-Gnansia E, Laurichesse H et al (2005) Congenital diaphragmatic hernia: a retinoid-signaling pathway disruption during lung development? Birth Defects Res Part A Clin Mol Teratol 73:523–31

    Article  PubMed  CAS  Google Scholar 

  4. Beurskens LW, Tibboel D, Lindemans J, Duvekot JJ, Cohen-Overbeek TE, Veenma DC et al (2010) Retinol status of newborn infants is associated with congenital diaphragmatic hernia. Pediatrics 126:712–20

    Article  PubMed  Google Scholar 

  5. Major D, Cadenas M, Fournier L, Leclerc S, Lefebvre M, Cloutier R (1998) Retinol status of newborn infants with congenital diaphragmatic hernia. Pediatr Surg Int 13:547–9

    Article  PubMed  CAS  Google Scholar 

  6. Keijzer R, Liu J, Deimling J, Tibboel D, Post M (2000) Dual-hit hypothesis explains pulmonary hypoplasia in the nitrofen model of congenital diaphragmatic hernia. Am J Pathol 156:1299–306

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  7. Karamanoukian HL, Glick PL, Wilcox DT, O’Toole SJ, Rossman JE, Azizkhan RG (1995) Pathophysiology of congenital diaphragmatic hernia. XI: anatomic and biochemical characterization of the heart in the fetal lamb CDH model. J Pediatr Surg 30:925–8, discussion 9

    Article  PubMed  CAS  Google Scholar 

  8. Gallot D, Coste K, Francannet C, Laurichesse H, Boda C, Ughetto S et al (2006) Antenatal detection and impact on outcome of congenital diaphragmatic hernia: a 12-year experience in Auvergne, France. Eur J Obstet Gynecol Reprod Biol 125:202–5

    Article  PubMed  Google Scholar 

  9. Mullassery D, Ba’ath ME, Jesudason EC, Losty PD (2010) Value of liver herniation in prediction of outcome in fetal congenital diaphragmatic hernia: a systematic review and meta-analysis. Ultrasound Obstet Gynecol: Off J Int Soc Ultrasound Obstet Gynecol 35:609–14

    Article  CAS  Google Scholar 

  10. Hedrick HL, Danzer E, Merchant A, Bebbington MW, Zhao H, Flake AW 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 e1–4

    Article  PubMed  Google Scholar 

  11. Metkus AP, Filly RA, Stringer MD, Harrison MR, Adzick NS (1996) Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg 31:148–51, discussion 51-2

    Article  PubMed  CAS  Google Scholar 

  12. Waag KL, Loff S, Zahn K, Ali M, Hien S, Kratz M et al (2008) Congenital diaphragmatic hernia: a modern day approach. Semin Pediatr Surg 17:244–54

    Article  PubMed  Google Scholar 

  13. Busing KA, Kilian AK, Schaible T, Dinter DJ, Neff KW (2008) MR lung volume in fetal congenital diaphragmatic hernia: logistic regression analysis—mortality and extracorporeal membrane oxygenation. Radiology 248:233–9

    Article  PubMed  Google Scholar 

  14. Jani JC, Nicolaides KH, Gratacos E, Valencia CM, Done E, Martinez JM et al (2009) Severe diaphragmatic hernia treated by fetal endoscopic tracheal occlusion. Ultrasound Obstet Gynecol: Off J Int Soc Ultrasound Obstet Gynecol 34:304–10

    Article  CAS  Google Scholar 

  15. Effect of corticosteroids for fetal maturation on perinatal outcomes. NIH consensus statement. 1994;12:1-24

  16. Davey M, Shegu S, Danzer E, Ruchelli E, Adzick S, Flake A et al (2007) Pulmonary arteriole muscularization in lambs with diaphragmatic hernia after combined tracheal occlusion/glucocorticoid therapy. Am J Obstet Gynecol 197:381 e1–7

    Article  PubMed  Google Scholar 

  17. Stevens TP, Chess PR, McConnochie KM, Sinkin RA, Guillet R, Maniscalco WM et al (2002) Survival in early- and late-term infants with congenital diaphragmatic hernia treated with extracorporeal membrane oxygenation. Pediatrics 110:590–6

    Article  PubMed  Google Scholar 

  18. Stevens TP, van Wijngaarden E, Ackerman KG, Lally PA, Lally KP, Congenital Diaphragmatic Hernia Study G (2009) Timing of delivery and survival rates for infants with prenatal diagnoses of congenital diaphragmatic hernia. Pediatrics 123:494–502

    Article  PubMed  Google Scholar 

  19. Frenckner BP, Lally PA, Hintz SR, Lally KP, Congenital Diaphragmatic Hernia Study G (2007) Prenatal diagnosis of congenital diaphragmatic hernia: how should the babies be delivered? J Pediatr Surg 42:1533–8

    Article  PubMed  Google Scholar 

  20. Reiss I, Schaible T, van den Hout L, Capolupo I, Allegaert K, van Heijst A et al (2010) Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium consensus. Neonatology 98:354–64

    Article  PubMed  CAS  Google Scholar 

  21. Van Meurs K, Congenital Diaphragmatic Hernia Study G (2004) Is surfactant therapy beneficial in the treatment of the term newborn infant with congenital diaphragmatic hernia? J Pediatr 145:312–6

    Article  PubMed  Google Scholar 

  22. van den Hout L, Tibboel D, Vijfhuize S, te Beest H, Hop W, Reiss I et al (2011) The VICI-trial: high frequency oscillation versus conventional mechanical ventilation in newborns with congenital diaphragmatic hernia: an international multicentre randomized controlled trial. BMC Pediatr 11:98

    Article  PubMed  PubMed Central  Google Scholar 

  23. Moenkemeyer F, Patel N (2014) Right ventricular diastolic function measured by tissue Doppler imaging predicts early outcome in congenital diaphragmatic hernia. Pediatr Crit Care Med: J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc 15:49–55

    Article  Google Scholar 

  24. Aggarwal S, Stockmann P, Klein MD, Natarajan G (2011) Echocardiographic measures of ventricular function and pulmonary artery size: prognostic markers of congenital diaphragmatic hernia? J Perinatol: Off J Calif Perinatal Assoc 31:561–6

    Article  CAS  Google Scholar 

  25. The Neonatal Inhaled Nitric Oxide Study Group (NINOS) (1997) Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. Pediatrics 99:838–45

    Article  Google Scholar 

  26. Kinsella JP (2008) Inhaled nitric oxide in the term newborn. Early Hum Dev 84:709–16

    Article  PubMed  CAS  Google Scholar 

  27. Buss M, Williams G, Dilley A, Jones O (2006) Prevention of heart failure in the management of congenital diaphragmatic hernia by maintaining ductal patency. A case report. J Pediatr Surg 41:e9–11

    Article  PubMed  Google Scholar 

  28. Shiyanagi S, Okazaki T, Shoji H, Shimizu T, Tanaka T, Takeda S et al (2008) Management of pulmonary hypertension in congenital diaphragmatic hernia: nitric oxide with prostaglandin-E1 versus nitric oxide alone. Pediatr Surg Int 24:1101–4

    Article  PubMed  Google Scholar 

  29. Hoffman TM, Wernovsky G, Atz AM, Kulik TJ, Nelson DP, Chang AC et al (2003) Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 107:996–1002

    Article  PubMed  CAS  Google Scholar 

  30. Patel N (2012) Use of milrinone to treat cardiac dysfunction in infants with pulmonary hypertension secondary to congenital diaphragmatic hernia: a review of six patients. Neonatology 102:130–6

    Article  PubMed  Google Scholar 

  31. Noori S, Friedlich P, Wong P, Garingo A, Seri I (2007) Cardiovascular effects of sildenafil in neonates and infants with congenital diaphragmatic hernia and pulmonary hypertension. Neonatology 91:92–100

    Article  PubMed  CAS  Google Scholar 

  32. Atz AM, Wessel DL (1999) Sildenafil ameliorates effects of inhaled nitric oxide withdrawal. Anesthesiology 91:307–10

    Article  PubMed  CAS  Google Scholar 

  33. De Luca D, Zecca E, Vento G, De Carolis MP, Romagnoli C (2006) Transient effect of epoprostenol and sildenafil combined with iNO for pulmonary hypertension in congenital diaphragmatic hernia. Paediatr Anaesth 16:597–8

    Article  PubMed  Google Scholar 

  34. Goissen C, Ghyselen L, Tourneux P, Krim G, Storme L, Bou P et al (2008) Persistent pulmonary hypertension of the newborn with transposition of the great arteries: successful treatment with bosentan. Eur J Pediatr 167:437–40

    Article  PubMed  CAS  Google Scholar 

  35. Guner YS, Khemani RG, Qureshi FG, Wee CP, Austin MT, Dorey F et al (2009) Outcome analysis of neonates with congenital diaphragmatic hernia treated with venovenous vs venoarterial extracorporeal membrane oxygenation. J Pediatr Surg 44:1691–701

    Article  PubMed  Google Scholar 

  36. Bryner BS, Kim AC, Khouri JS, Drongowski RA, Bruch SW, Hirschl RB et al (2009) Right-sided congenital diaphragmatic hernia: high utilization of extracorporeal membrane oxygenation and high survival. J Pediatr Surg 44:883–7

    Article  PubMed  Google Scholar 

  37. Morini F, Goldman A, Pierro A (2006) Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia: a systematic review of the evidence. Eur J Pediatr Surg: Off J Aust Assoc Pediatr Surg [et al] = Zeitschrift fur Kinderchirurgie 16:385–91

    Article  CAS  Google Scholar 

  38. Sluiter I, van de Ven CP, Wijnen RM, Tibboel D (2011) Congenital diaphragmatic hernia: still a moving target. Semin Fetal Neonatal Med 16:139–44

    Article  PubMed  CAS  Google Scholar 

  39. Zani A, Zani-Ruttenstock E, Pierro A (2014) Advances in the surgical approach to congenital diaphragmatic hernia. Semin Fetal Neonatal Med 19:364–9

    Article  PubMed  Google Scholar 

  40. Romao RL, Nasr A, Chiu PP, Langer JC (2012) What is the best prosthetic material for patch repair of congenital diaphragmatic hernia? Comparison and meta-analysis of porcine small intestinal submucosa and polytetrafluoroethylene. J Pediatr Surg 47:1496–500

    Article  PubMed  Google Scholar 

  41. Lansdale N, Alam S, Losty PD, Jesudason EC (2010) Neonatal endosurgical congenital diaphragmatic hernia repair: a systematic review and meta-analysis. Ann Surg 252:20–6

    Article  PubMed  Google Scholar 

  42. Dassinger MS, Copeland DR, Gossett J, Little DC, Jackson RJ, Smith SD et al (2010) Early repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation. J Pediatr Surg 45:693–7

    Article  PubMed  Google Scholar 

  43. Congenital Diaphragmatic Hernia Study G, Bryner BS, West BT, Hirschl RB, Drongowski RA, Lally KP et al (2009) Congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: does timing of repair matter? J Pediatr Surg 44:1165–71, discussion 71-2

    Article  Google Scholar 

  44. American Academy of Pediatrics Section on S, American Academy of Pediatrics Committee on F, Newborn, Lally KP, Engle W (2008) Postdischarge follow-up of infants with congenital diaphragmatic hernia. Pediatrics 121:627–32

    Article  Google Scholar 

Download references

Conflict of Interest

The author declares that he has no competing interests.

Author information

Authors and Affiliations

  1. Department of Pediatrics, University at Buffalo, State University of New York, Buffalo, NY, USA

    Vasanth H. S. Kumar

Authors
  1. Vasanth H. S. Kumar
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Vasanth H. S. Kumar.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kumar, V.H.S. Current Concepts in the Management of Congenital Diaphragmatic Hernia in Infants. Indian J Surg 77, 313–321 (2015). https://doi.org/10.1007/s12262-015-1286-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12262-015-1286-8

Keywords

Search

Navigation