Abstract
Over the last two decades there has been a constant improvement in the understanding of the pathophysiology of Congenital Diaphragmatic Hernia (CDH) and its management. However, the ideal treatment remains elusive. The earlier management strategy of immediate surgery is replaced by the principle of physiological stabilisation and delayed surgery. Conventional mechanical ventilatory techniques, with high pressures and hyperventilation to reverse ductal shunting and cause alkalinization, are being questioned because of the risks of barotrauma and consequent broncho-pulmonary dysplasia. It has also been shown that paralysis with pancuronium bromide for patients on conventional mechanical ventilation results in increased incidence of sensorineural hearing loss in childhood survivors of CDH. With the introduction of the concept of permissive hypercapnia and high frequency oscillation ventilation, the complications of pulmonary barotrauma are circumvented. Although ECMO therapy is invasive, yet has improved survival by about 15% independently, especially in critically ill infants who have the predictive mortality rate of more than 80%. Further insights into the pathophysiology of CDH and the introduction of less invasive therapeutic techniques in the form of high frequency oscillation ventilation, inhalation nitric oxide, surfactant, and perfluorocarbon liquid ventilation may even make the need for ECMO redundant.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Ramadwar RH, Carachi R, Young DG. Collagen coated vicryl mesh is not a suitable material for repair of diaphragmatic defects.J Pediatr Surg 1997; 32 (12): 1708–1710.
Whetzel TP, Stockes RB, Greenholz SKet al. Reconstruction of the toddler diaphragm in severe anterolateral congenital diaphragmatic hernia with the reverse latissimus dorsi flap.Ann Plast Surg 1997; 39 (6): 615–619.
Clark RH, Hardin WD, Hirschl RBet al. Current surgical management of congenital diaphragmatic hernia: A report from the congenital diaphragmatic hernia study group.J Pediatr Surg 1998; 33 (7): 1004–1009.
Harrison MRet al. Fetal diaphragmatic hernia: Fatal but fixable.Semin Perinatal 1985; 9: 103.
Sharland GKet al. Prognosis in fetal diaphragmatic hernia.Am J Obstet Gynecol 1992; 166: 9.
Bohn Det al. The relationship between PaCO2 and ventilation parameters in predicting survival in CDH.J Pediatr Surg 1984; 19: 666.
Antunes MJet al. Prognosis with preoperative pulmonary function and lung volume assessment in infants with congenital diaphragmatic hernia.Pediatrics 1995; 96: 1117.
Kavvadia V, Greenough A, Laubscher Bet al. Perioperative assessment of respiratory compliance and lung volume in infants with congenital diaphragmatic hernia: predictor of outcome.J Pediatr Surg 1997; 32 (12): 1665–1669.
Hatch EL Jr, Kendall J, Blumenhagen J. Stomach position asin utero predictor of neonatal outcome in left sided diaphragmatic hernia.J Pediatr Surg 1992; 27: 778.
Thorpe-Beeston JG, Dosden CM, Nicolaides KH. Prenatal diagnosis of congenital diaphragmatic hernia: Associated malformations and chromosomal defects.Fetal Ther 1989; 4: 21.
Towokian RJ, Markowitz RI. A preoperative scoring system for risk assessment of newborns with congenital diaphragmatic hernia.J Pediatr 1984; 19: 252.
Thebaud B, Azancot A, de-Lagausie Pet al. Congenital diaphragmatic hernia: Antenatal prognostic factors. Does cardiac ventricular disproportionin utero predict outcome and pulmonary hypoplasia?Intensive Care Med 1997; 32: 1637–1642.
Lipshutz GS, Albanese CT, Feldstein VAet al. Prospective analysis of lung to head ratio predicts survival for patients with prenatally diagnosed congenital diaphragmatic hernia.J Pediatr Surg 1997; 32 (11): 1634–1636.
Keshen TH, Gursoy M, Shew SBet al. Does extracorporeal membrane oxygenation benefit neonates with congential diaphragmatic hernia? Application of predictive equation.J Pediatr Surg 1997; 32 (6): 818–822.
Extra-corporeal Life Support Organization: 1996 ECMO registry data, Ann Arbor, 1996, Extra-corporeal Life Support Organization.
Connors RHet al. Congenital diaphragmatic hernia repair on ECMO.J Pediatr Surg 1990; 25: 1043.
Thibeault DW, Haney B. Lung volume, pulmonary vasculature, and factors affecting survival in congenital diaphragmatic hernia.Pediatr 1998; 10 (2): 289–295.
Harrison MR, Adzick NS, Bullard KMet al. Correction of congenital diaphragmatic herniain utero VII: a propective trial.J Pediatr Surg 1997; 32: 1637–1642.
Reyes C, Chang LK, Waffarnet al. Delayed repair of congenital diaphragmatic hernia with early high frequency oscillatory ventilation during preoperative stabilization.J Pediatr Surg 1998; 33 (7): 1010–1014.
Weber TR, Kountzman B, Billon PAet al. Improved survival in congenital diaphragmatic hernia with evolving therapeutic strategies.Arch Surg 1998; 133 (5) 498–502.
Seemakula N, Stewart DL, Goldsmith LJet al. Survival of patients with congenital diaphragmatic hernia during the ECMO era: An eleven year experience.
Frenckner B, Ehren H, Granholmet al. Improved results in patients who have congenital diaphragmatic hernia using preoperative stabiliation, extracorporeal membrane oxygenation, and delayed surgery.J Pediatr Surg 1997; 32 (8): 1185–1189.
Kinsella Jet al. Low dose inhational nitric oxide in persistent pulmonary hypertension of the newborn.Lancet 1992; 340: 819.
Karamanoukian HLet al. Inhalet nitric oxide in congenital hypoplasia of lungs due to diaphragmatic hernia of oligohydramnios.Pediatrics 1994; 94: 1.
Harrison MRet al. Corrections of congenital diaphragmatic herniain utero: II. Summarized correction permits fetal lung growth with survival at birth.Surgery 1980; 88: 260.
Hedrick MHet al. Plug the lung until it grows (PLUG): A new method to treat congenital diaphragmatic herniain utero.J Pediatr Surg 1994; 29: 612.
Sylvester KG, Rasanen J, Kitano Yet al. Tracheal occlusion reverses the high impedence to flow in the fetal pulmonary circulation and normalized its physiological response to oxygen at term.J Pediatr Surg 1998; 33 (7): 1071–1074.
Flageole H, Evrard VA, Piedboeuf Bet al. The plug inplug sequence: An important step to achieve type II pneumoycte maturation in the fetal lamb model.J Pediatr Surg 1998; 33 (2): 299–303.
O’Toole SJ, Karamanoukian HL, Irish MSet al. Tracheal ligation: The dark side ofin utero congenital diaphragmatic hernia treatment.J Pediatr Surg 1997; 32 (3) 407–410.
Pranikoff T, Gauger PG, Hirschl RB. Partial liquid ventilation in newborn patients with congenital diaphragmatic hernia.J Pediatr Surg 1996; 31: 613.
Nobunara KK, Ferretti ML, Siddiqui AMet al. Long term effect of perflurocarbon distension on the lung.J Pediatr Surg 1998; 33 (7): 1024–1029.
Wilcox DT, Glick PL, Karamanoukranet al. Partial liquid ventilation and nitric oxide in congenital diaphragmatic hernia.J Pediatr Surg 1997; 32 (8): 1211–1215.
Tannuri U, Maksoud Filho JG, Santos MMet al. The effects of prenatal intra-amniotic surfactant or dexamethasone administration on lung development are comparable to changes induced by tracheal ligation in an animal model of congenital diaphragmatic hernia.J Pediatr Surg 1998; 33 (8): 1198–1205.
Taira Y, Miyazaki E, Oshiro Ket al. Administration of antenatal glucocorticoids prevents pulmonary artery structural changes in nitrofen induced congenital diaphragmatic hernia in rats.J Pediatr Surg 1998; 33 (7): 1052–1056.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Arora, M., Bajpai, M., Soni, T.R. et al. Congenital Diaphragmatic Hernia. Indian J Pediatr 67, 665–670 (2000). https://doi.org/10.1007/BF02762181
Issue Date:
DOI: https://doi.org/10.1007/BF02762181