The Indian Journal of Pediatrics

, Volume 84, Issue 8, pp 629–635

Management of Congenital Diaphragmatic Hernia in Newborn — Paradigm Shift and Ethical Issues

Review Article

Abstract

Management of congenital diaphragmatic hernia (CDH) begins soon after it is detected, whether antenatally or postnatally. Assessment of the severity of the condition, associated congenital anomalies, maternal health and related issues, weight of the fetus/baby, mode of delivery, timing of delivery, immediate appropriate management of the baby with CDH at birth, appropriate utilization of available treatment modalities as well as infrastructure of the treating institute have an impact on the outcome of the neonate. Survival without significant long-term/permanent morbidity is considered as good outcome. With advances in antenatal diagnosis, several legal and ethical considerations have cropped up. While on one hand there are proponents of early antenatal diagnosis and medical termination of pregnancy (MTP), on the other hand there are several socio-cultural groups who look upon human life as precious and argue against MTP. There is an ongoing ethical battle between maternal vs. fetal rights; there is no way to put a lid on the controversy whether the mother be allowed to choose in favor of MTP after being aware of the anomalous fetus or, we must attempt to save every fetus irrespective of the antenatal diagnosis of life-threatening anomalies. Notwithstanding, appropriate assessment of the condition, thorough counseling and sound evidence-based decisions could avert ethical dilemma in most cases. This review article provides information about the various choices available in the diagnostic and treatment armamentarium, though it should be kept in mind that the entire spectrum of management strategies may not be universally available.

Keywords

Congenital diaphragmatic hernia Paradigm shift Antenatal diagnosis Intervention Postnatal management 

References

  1. 1.
    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;3:CD001695.Google Scholar
  2. 2.
    Rizzo G, Pietrolucci M, Aiello E, Mammarella S, Bosi C, Arduini D. The role of three-dimensional ultrasound in the diagnosis of fetal congenital anomalies: a review. Minerva Ginecol. 2011;63:401–10.PubMedGoogle Scholar
  3. 3.
    Liu X, Ashtari M, Leonidas JC, Chan Y. Magnetic resonance imaging of the fetus in congenital intrathoracic disorders: preliminary observations. Pediatr Radiol. 2001;31:435–9.CrossRefPubMedGoogle Scholar
  4. 4.
    Phatak SV, Ghodke K. Antenatal diagnosis of congenital diaphragmatic hernia: a case report. Indian J Radiol Imag. 2006;16:793–5.CrossRefGoogle Scholar
  5. 5.
    Stiller RJ, Roberts NS, Weiner S. Congenital diaphragmatic hernia: antenatal diagnosis and obstetrical management. J Clin Ultrasound. 1985;13:212–5.CrossRefPubMedGoogle Scholar
  6. 6.
    Metkus AP, Filly RA, Stringer MD, Harrison MR, Adzick NS. Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg. 1996;31:148–51.CrossRefPubMedGoogle Scholar
  7. 7.
    Fisher JC, Jefferson RA, Arkovitz MS, Stolar CJ. Redefining outcomes in right congenital diaphragmatic hernia. J Pediatr Surg. 2008;43:373–9.CrossRefPubMedGoogle Scholar
  8. 8.
    Usui N, Kitano Y, Okuyama H, et al. Reliability of the lung to thorax transverse area ratio as a predictive parameter in fetuses with congenital diaphragmatic hernia. Pediatr Surg Int. 2011;27:39–45.CrossRefPubMedGoogle Scholar
  9. 9.
    Walleyo A, Debus A, Kehl S, et al. Periodic MRI lung volume assessment in fetuses with congenital diaphragmatic hernia: prediction of survival, need for ECMO, and development of chronic lung disease. AJR Am J Roentgenol. 2013;201:419–26.CrossRefPubMedGoogle Scholar
  10. 10.
    Kastenholz KE, Weis M, Hagelstein C, et al. Correlation of observed-to-expected MRI fetal lung volume and ultrasound lung-to-head ratio at different gestational times in fetuses with congenital diaphragmatic hernia. Am J Roentgenol. 2016;206:856–66.CrossRefGoogle Scholar
  11. 11.
    Harrison MR, Adzick NS, Longaker MT, et al. Successful repair in utero of a fetal diaphragmatic hernia after removal of herniated viscera from the left thorax. N Engl J Med. 1990;322:1582–4.CrossRefPubMedGoogle Scholar
  12. 12.
    VanderWall KJ, Bruch SW, Meuli M, et al. Fetal endoscopic ('Fetendo') tracheal clip. J Pediatr Surg. 1996;31:1101–3.CrossRefPubMedGoogle Scholar
  13. 13.
    Jani JC, Nicolaides KH. Fetal surgery for severe congenital diaphragmatic hernia? Ultrasound Obstet Gynecol. 2012;39:7–9.CrossRefPubMedGoogle Scholar
  14. 14.
    Hedrick HL. Ex utero intrapartum therapy. Semin Pediatr Surg. 2003;12:190–5.CrossRefPubMedGoogle Scholar
  15. 15.
    Kunisaki SM, Barnewolt CE, Estroff JA, et al. Ex utero intrapartum treatment with extracorporeal membrane oxygenation for severe congenital diaphragmatic hernia. J Pediatr Surg. 2007;42:98–104.CrossRefPubMedGoogle Scholar
  16. 16.
    Shieh HF, Wilson JM, Sheils CA, et al. Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change morbidity outcomes for high-risk congenital diaphragmatic hernia survivors? J Pediatr Surg. 2017;52:22–5.CrossRefPubMedGoogle Scholar
  17. 17.
    Terui K, Nagata K, Kanamori Y, et al. Risk stratification for congenital diaphragmatic hernia by factors within 24 h after birth. J Perinatol. 2017 Feb 23; doi:10.1038/jp.2017.11.
  18. 18.
    Snoek KG, Reiss IK, Greenough A, et al. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO consortium consensus - 2015 update. Neonatology. 2016;110:66–74.CrossRefPubMedGoogle Scholar
  19. 19.
    Ingimarsson J, Björklund LJ, Curstedt T, et al. Incomplete protection by prophylactic surfactant against the adverse effects of large lung inflations at birth in immature lambs. Intensive Care Med. 2004;30:1446–53.CrossRefPubMedGoogle Scholar
  20. 20.
    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;76:488–94.PubMedGoogle Scholar
  21. 21.
    Cacciari A, Ruggeri G, Mordenti M, et al. High-frequency oscillatory ventilation versus conventional mechanical ventilation in congenital diaphragmatic hernia. Eur J Pediatr Surg. 2001;11:3–7.CrossRefPubMedGoogle Scholar
  22. 22.
    Ng GY, Derry C, Marston L, Choudhury M, Holmes K, Calvert SA. Reduction in ventilator-induced lung injury improves outcome in congenital diaphragmatic hernia? Pediatr Surg Int. 2008;24:145–50.CrossRefPubMedGoogle Scholar
  23. 23.
    Chou HC, Tang JR, Lai HS, Tsao PN, Yau KI. Prognostic indicators of survival in infants with congenital diaphragmatic hernia. J Formos Med Assoc. 2001;100:173–5.PubMedGoogle Scholar
  24. 24.
    Weis CM, Wolfson MR, Shaffer TH. Liquid-assisted ventilation: physiology and clinical application. Ann Med. 1997;29:509–17.CrossRefPubMedGoogle Scholar
  25. 25.
    Gauger PG, Pranikoff T, Schreiner RJ, Moler FW, Hirschl RB. Initial experience with partial liquid ventilation in pediatric patients with the acute respiratory distress syndrome. Crit Care Med. 1996;24:16–22.CrossRefPubMedGoogle Scholar
  26. 26.
    VallsiSoler A, Wauer RR. 2nd European symposium on liquid ventilation. Eur J Med Res. 2001;6:115–38.Google Scholar
  27. 27.
    Al-Hathlol K, Elmahdy H, Nawaz S, et al. Perioperative course of pulmonary hypertension in infants with congenital diaphragmatic hernia: impact on outcome following successful repair. J Pediatr Surg. 2011;46:625–9.CrossRefPubMedGoogle Scholar
  28. 28.
    Sarin YK. Paradigm shift in management of congenital diaphragmatic hernia in newborn. Pediatr Clin India. 2008;43:364–84.Google Scholar
  29. 29.
    Putnam LR, Tsao K, Morini F, et al; Congenital Diaphragmatic Hernia Study Group. Evaluation of variability in inhaled nitric oxide use and pulmonary hypertension in patients with congenital diaphragmatic hernia. JAMA Pediatr. 2016;170:1188–94.Google Scholar
  30. 30.
    Steinhorn RH. Diagnosis and treatment of pulmonary hypertension in infancy. Early Hum Dev. 2013;89:865–74.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Porta NF, Steinhorn RH. Pulmonary vasodilator therapy in the NICU: inhaled nitric oxide, sildenafil, and other pulmonary vasodilating agents. Clin Perinatol. 2012;39:149–64.CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    McNamara PJ, Shivananda SP, Sahni M, Freeman D, Taddio A. Pharmacology of milrinone in neonates with persistent pulmonary hypertension of the newborn and suboptimal response to inhaled nitric oxide. Pediatr Crit Care Med. 2013;14:74–84.CrossRefPubMedGoogle Scholar
  33. 33.
    Mohamed WA, Ismail M. A randomized, double-blind, placebo-controlled, prospective study of bosentan for the treatment of persistent pulmonary hypertension of the newborn. J Perinatol. 2012;32:608–13.CrossRefPubMedGoogle Scholar
  34. 34.
    Morini F, Goldman A, Pierro A. Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia: a systematic review of the evidence. Eur J Pediatr Surg. 2006;16:385–91.CrossRefPubMedGoogle Scholar
  35. 35.
    Sluiter I, van de Ven CP, Wijnen RM, Tibboel D. Congenital diaphragmatic hernia: still a moving target. Semin Fetal Neonatal Med. 2011;16:139–44.CrossRefPubMedGoogle Scholar
  36. 36.
    Bojanić K, Woodbury JM, Cavalcante AN, et al. Congenital diaphragmatic hernia: outcomes of neonates treated at Mayo Clinic with and without extracorporeal membrane oxygenation. Paediatr Anaesth. 2017;27:314–21.CrossRefPubMedGoogle Scholar
  37. 37.
    Neerhof MG, MacGregor SN, Kilner JF. Congenital diaphragmatic hernia: in utero therapy and ethical considerations. Clin Perinatol. 1996;23:465–72.PubMedGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2017

Authors and Affiliations

  • Sushmita Nitin Bhatnagar
    • 1
  • Yogesh Kumar Sarin
    • 2
  1. 1.Department of Pediatric SurgeryB.J.Wadia Hospital for ChildrenMumbaiIndia
  2. 2.Department of Pediatric SurgeryMaulana Azad Medical CollegeNew DelhiIndia

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