Skip to main content
NCBI home page
Annals of Surgery logoLink to Annals of Surgery
. 1979 Sep;190(3):332–341. doi: 10.1097/00000658-197909000-00008

Congenital diaphragmatic hernia. New concept in management.

S J Shochat, R L Naeye, W D Ford, V Whitman, M J Maisels
PMCID: PMC1344664  PMID: 485607

Abstract

The newborn infant with a congenital diaphragmatic hernia (CDH) who develops severe respiratory distress requiring operative repair within the first 24 hours of life represents one of the most challenging problems in pediatric surgery. The mortality in these infants still exceeds 50% and has historically been attributed to ventilatory insufficiency secondary to pulmonary hypoplasia. However, the primary abnormality in these neonates seems to be an increase in pulmonary vascular resistance with an elevation of pulmonary artery pressure, right-left ductal shunting, preductal shunting and progressive hypoxemia. Eighteen neonates with a CDH were operated upon within the first 24 hours of life with a mortality of 38%. In no instance did ventilatory insufficiency seem to be a major factor in the death of the patient. Seven infants with progressive hypoxemia were treated with a vasodilator, tolazoline. Six of the seven infants showed an initial response to treatment, with a rise in preductal PaO2 and a decrease in ductal shunting. Four of these seven desperately ill neonates survived. Pathologic examination of the pulmonary vasculature in the non-survivors revealed an increase in muscle mass within the pulmonary arterioles. An exaggerated vasoconstrictive response of an abnormally hypertrophied pulmonary vascular bed leading to an elevation of pulmonary vascular resistance, rather than abnormalities of ventilation, appears to be the important mechanism leading to the often fatal hypoxemia observed in the neonate with a CDH. Improved survival will depend upon the successful management of the deranged pulmonary vascular hemodynamics seen in these infants.

Full text

PDF
332

Images in this article

335Fig. 2.
on p.335

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Benumof J. L., Wahrenbrock E. A. Blunted hypoxic pulmonary vasoconstriction by increased lung vascular pressures. J Appl Physiol. 1975 May;38(5):846–850. doi: 10.1152/jappl.1975.38.5.846. [DOI] [PubMed] [Google Scholar]
  2. Collins D. L., Pomerance J. J., Travis K. W., Turner S. W., Pappelbaum S. J. A new approach to congenital posterolateral diaphragmatic hernia. J Pediatr Surg. 1977 Apr;12(2):149–156. doi: 10.1016/s0022-3468(77)80001-8. [DOI] [PubMed] [Google Scholar]
  3. Dibbins A. W. Neonatal diaphragmatic hernia: a physiologic challenge. Am J Surg. 1976 Apr;131(4):408–410. doi: 10.1016/0002-9610(76)90147-1. [DOI] [PubMed] [Google Scholar]
  4. Dibbins A. W., Wiener E. S. Mortality from neonatal diaphragmatic hernia. J Pediatr Surg. 1974 Oct;9(5):653–662. doi: 10.1016/0022-3468(74)90102-x. [DOI] [PubMed] [Google Scholar]
  5. Ehrlich F. E., Salzberg A. M. Pathophysiology and management of congenital posterolateral diaphragmatic hernias. Am Surg. 1978 Jan;44(1):26–30. [PubMed] [Google Scholar]
  6. Fishman A. P. Hypoxia on the pulmonary circulation. How and where it acts. Circ Res. 1976 Apr;38(4):221–231. doi: 10.1161/01.res.38.4.221. [DOI] [PubMed] [Google Scholar]
  7. Fitzgerald R. J. Congenital diaphragmatic hernia: a study of mortality factors. Ir J Med Sci. 1977 Sep;146(9):280–284. doi: 10.1007/BF03030975. [DOI] [PubMed] [Google Scholar]
  8. Goetzman B. W., Sunshine P., Johnson J. D., Wennberg R. P., Hackel A., Merten D. F., Bartoletti A. L., Silverman N. H. Neonatal hypoxia and pulmonary vasospasm: response to tolazoline. J Pediatr. 1976 Oct;89(4):617–621. doi: 10.1016/s0022-3476(76)80403-9. [DOI] [PubMed] [Google Scholar]
  9. Graham T. P., Jr, Atwood G. F., Boucek R. J., Jr Pharmacologic dilatation of the ductus arteriosus with prostaglandin E1 in infants with congenital heart disease. South Med J. 1978 Oct;71(10):1238-41, 1246. doi: 10.1097/00007611-197810000-00015. [DOI] [PubMed] [Google Scholar]
  10. Haller J. A., Jr, Signer R. D., Golladay E. S., Inon A. E., Harrington D. P., Shermeta D. W. Pulmonary and ductal hemodynamics in studies of simulated diaphragmatic hernia of fetal and newborn lambs. J Pediatr Surg. 1976 Oct;11(5):675–680. doi: 10.1016/0022-3468(76)90089-0. [DOI] [PubMed] [Google Scholar]
  11. Hislop A., Reid L. Persistent hypoplasia of the lung after repair of congenital diaphragmatic hernia. Thorax. 1976 Aug;31(4):450–455. doi: 10.1136/thx.31.4.450. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Levin D. L., Cates L., Newfeld E. A., Muster A. J., Paul M. H. Persistence of the fetal cardiopulmonary circulatory pathway: survival of an infant after a prolonged course;. Pediatrics. 1975 Jul;56(1):58–64. [PubMed] [Google Scholar]
  13. Levin D. L., Heymann M. A., Kitterman J. A., Gregory G. A., Phibbs R. H., Rudolph A. M. Persistent pulmonary hypertension of the newborn infant. J Pediatr. 1976 Oct;89(4):626–630. doi: 10.1016/s0022-3476(76)80405-2. [DOI] [PubMed] [Google Scholar]
  14. Levin D. L. Morphologic analysis of the pulmonary vascular bed in congenital left-sided diaphragmatic hernia. J Pediatr. 1978 May;92(5):805–809. doi: 10.1016/s0022-3476(78)80162-0. [DOI] [PubMed] [Google Scholar]
  15. Levy R. J., Rosenthal A., Freed M. D., Smith C. D., Eraklis A., Nadas A. S. Persistent pulmonary hypertension in a newborn with congenital diaphragmeatic hernia: successful management with tolazoline. Pediatrics. 1977 Nov;60(5):740–742. [PubMed] [Google Scholar]
  16. Moodie D. S., Telander R. L., Kleinberg F., Feldt R. H. Use of tolazoline in newborn infants with diaphragmatic hernia and severe cardiopulmonary disease. A preliminary report. J Thorac Cardiovasc Surg. 1978 May;75(5):725–729. [PubMed] [Google Scholar]
  17. Murdock A. I., Burrington J. B., Swyer P. R. Alveolar to arterial oxygen tension difference and venous admixture in newly born infants with congenital diaphragmatic herniation through the foramen of Bochdalek. Biol Neonate. 1971;17(3):161–172. doi: 10.1159/000240311. [DOI] [PubMed] [Google Scholar]
  18. Naeye R. L., Shochat S. J., Whitman V., Maisels M. J. Unsuspected pulmonary vascular abnormalities associated with diaphragmatic hernia. Pediatrics. 1976 Dec;58(6):902–906. [PubMed] [Google Scholar]
  19. Peckham G. J., Fox W. W. Physiologic factors affecting pulmonary artery pressure in infants with persistent pulmonary hypertension. J Pediatr. 1978 Dec;93(6):1005–1010. doi: 10.1016/s0022-3476(78)81239-6. [DOI] [PubMed] [Google Scholar]
  20. Raphaely R. C., Downes J. J., Jr Congenital diaphragmatic hernia: prediction of survival. J Pediatr Surg. 1973 Oct;8(5):815–823. doi: 10.1016/0022-3468(73)90426-0. [DOI] [PubMed] [Google Scholar]
  21. Rowe M. I., Uribe F. L. Diaphragmatic hernia in the newborn infant: blood gas and pH considerations. Surgery. 1971 Nov;70(5):758–761. [PubMed] [Google Scholar]
  22. Starrett R. W., de Lorimier A. A. Congenital diaphragmatic hernia in lambs: hemodynamic and ventilatory changes with breathing. J Pediatr Surg. 1975 Oct;10(5):575–582. doi: 10.1016/0022-3468(75)90359-0. [DOI] [PubMed] [Google Scholar]
  23. Wohl M. E., Griscom N. T., Strieder D. J., Schuster S. R., Treves S., Zwerdling R. G. The lung following repair of congenital diaphragmatic hernia. J Pediatr. 1977 Mar;90(3):405–414. doi: 10.1016/s0022-3476(77)80702-6. [DOI] [PubMed] [Google Scholar]

Articles from Annals of Surgery are provided here courtesy of Lippincott, Williams, and Wilkins

RESOURCES