Skip to main content
Log in

Bilateral congenital diaphragmatic hernia and gastroschisis in a newborn: can low intrathoracic pressure prevent the pulmonary hypoplasia?

  • Case Report
  • Published:
Pediatric Surgery International Aims and scope Submit manuscript

Abstract

Congenital diaphragmatic hernia (CDH) is associated with high mortality and morbidity due to pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Bilateral CDH is extremely rare with poor prognosis. It is usually accepted that PH in CDH is due to the herniation of abdominal viscera in the thorax leading to compression of the lung and preventing the normal lung development. On the other hand, some authors suggest that the PH occurs independently from the intrathoracic pressure in foetuses with CDH because of embryologic and genetic factors. We report a case of a newborn with bilateral CDH and gastroschisis born without PH, with favourable outcome. We support the hypothesis that a low intrathoracic pressure in patients with CDH allows an improved lung development.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. The Congenital Diaphragmatic Hernia Study Group (2003) Bilateral congenital diaphragmatic hernia. J Pediatr Surg 38(3):522–524

    Article  Google Scholar 

  2. Bennet AJ, Driver CP, Munro M (2005) Bilateral congenital diaphragmatic hernia. Pediatr Surg Int 21:739–741

    Article  Google Scholar 

  3. Kufeji DI, Crabbe DCG (1999) Familial bilateral congenital diaphragmatic hernia. Pediatr Surg Int 15:58–60

    Article  PubMed  CAS  Google Scholar 

  4. Tenbrinck R, Gaillard JLJ, Tibboel D, Kluth D, Lachmann B, Molenaar JC (1992) Pulmonary vascular abnormalities in experimentally induced congenital diaphragmatic hernia in rats. J Pediatr Surg 27:862–865

    Article  PubMed  CAS  Google Scholar 

  5. Geggel RL, Murphy JD, Langleben D, Crone RK, Vacanti JP, Reid LM (1985) Congenital diaphragmatic hernia: arterial structural changes and persistent pulmonary hypertension after surgical repair. J Pediatr 107(3):457–464

    Article  PubMed  CAS  Google Scholar 

  6. Mychaliska GB, Officer SM, Heintz CK, Starcher BC, Pierce RA (2004) Pulmonary elastin expression is decreased in the nitrofen-induced rat model of congenital diaphragmatic hernia. J Pediatr Surg 39(5):666–671

    Article  PubMed  Google Scholar 

  7. Glick PL, Stannard VA, Leach CL et al (1992) Pathophysiology of congenital diaphragmatic hernia II. The fetal lamb model is surfactant deficient. J Pediatr Surg 27(3):382–388

    Article  PubMed  CAS  Google Scholar 

  8. Jesudason EC, Connel MG, Fernig DG, Lloyd DA, Losty PD (2000) Early lung malformations in congenital diaphragmatic hernia. J Pediatr Surg 35(1):124–127

    PubMed  CAS  Google Scholar 

  9. Dave S, Gupta DK (2000) Congenital diaphragmatic hernia. In: Gupta (ed) Textbook of neonatal surgery, 11th edn, chap 60:374–393

  10. Kluth D, Tenbrinck R, von Ekesparre M, Kangah R, Reich P, Brandsma A, Tibboel D, Lambrecht W (1993) The natural history of congenital diaphragmatic hernia and pulmonary hypoplasia in the embryo. J Pediatr Surg 28:456–463

    Article  PubMed  CAS  Google Scholar 

  11. Iritani I (1984) Experimental study on embryogenesis of congenital diaphragmatic hernia. Anat Embryol 169:133–139

    Article  PubMed  CAS  Google Scholar 

  12. Ackerman KG, Herron BJ, Vargas SO, Huang H, Tevosian SG, Kochilas L, Rao C, Pober BR, Babiuk RP, Epstein JA, Greer JJ, Beier DR (2005) Fog2 is required for normal diaphragm and lung development in mice and humans. PloS Genet 1(1):58–65

    Article  PubMed  CAS  Google Scholar 

  13. Bargy F, Beaudoin S, Barbet P (2006) Fetal lung growth in congenital diaphragmatic hernia. Fetal Diagn Ther 21:39–44

    Article  PubMed  CAS  Google Scholar 

  14. Chun YS, Kim WK, Jung SJ (2004) The effect of gastroschisis on experimental diaphragmatic hernia in fetal rabbits. J Pediatr Surg 39:1863–1866

    Article  PubMed  Google Scholar 

  15. Montgomery LD, Belfort MA, Saade GR, Baker BW, Pokorny W, Minifee P, Langston C, Jevon G, Van den Veyver I, Robie D et al (1995) Iatrogenic gastroschisis decreases pulmonary hypoplasia in an ovine congenital diaphragmatic hernia model. Fetal Diagn Ther 10(2):119–126

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Paola Zaupa.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Zaupa, P., Kleinlein, B. & Höllwarth, M.E. Bilateral congenital diaphragmatic hernia and gastroschisis in a newborn: can low intrathoracic pressure prevent the pulmonary hypoplasia?. Pediatr Surg Int 23, 711–713 (2007). https://doi.org/10.1007/s00383-006-1873-7

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00383-006-1873-7

Keywords

Navigation