Skip to main content
Log in

Pentalogy of cantrell

  • Clinical Brief
  • Published:
The Indian Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Omphalocele is often, associated with various abnormalities. In two patients admitted with omphalocele, we additionally found defects both in the pars stenalis of diaphragm and in the diaphragmatic surface of the pericardium, sternal defect and tetralogy of Fallot. These entities form the components of a syndrome, named pentalogy of Cantrell. It is extremely in the spectrum of midline closure defects that display wide variations and its incidence is very low. Existing cardiac defect is the most important factor influencing morbidity and mortality. Herein we report two cases of pentalogy of Cantrell with the review of the syndrome in the light of literature.

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.

References

  1. Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart.Surg Gynecol Obstet 1958; 107: 602–614.

    PubMed  CAS  Google Scholar 

  2. Milne LW, Morosin MA, Campbell JRea al. Pars sternalis diaphragmatic hernia with omphalocele. A report of two cases.J Pediatr Surg 1990; 25: 726–730.

    Article  PubMed  CAS  Google Scholar 

  3. Kamuran M, Ariturk E. Congenital bifid sternum with partial ectopia cordis.J Cardiovasc Surg 1989; 30: 288–289.

    CAS  Google Scholar 

  4. Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: A case report and review of the syndrome.Pediatrics 1972; 50: 778–792.

    PubMed  CAS  Google Scholar 

  5. Wells LJ. Development of the human diaphragm and pleural sacs.Contr Embryol, 1954; 35: 109–134.

    Google Scholar 

  6. Greenwood RD, Rosenthal A, Nadas AS. Cardiovascular abnormalities associated with congenital diaphragmatic hernia.Pediatrics 1976; 57: 92–97.

    PubMed  CAS  Google Scholar 

  7. Greenwood RD, Rosenthal A, Nadas AS. Cardiovascular abnormalities associated with omphalocele.J Pediatr 1974; 85: 818–821.

    Article  PubMed  CAS  Google Scholar 

  8. Sweed Y, Puri P. Congenital diaphragmatic hernia: Influence of associated malformations on survival.Arch Dis Child 1993; 69: 68–70.

    PubMed  CAS  Google Scholar 

  9. Gershoni-Baruch R, Machoul I, Weiss Yet al Unknown syndrome: radial ray defects, omphalocele, diaphragmatic hernia, and hepatic cyst.J Med Genetics 1990; 27: 403–404.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Katranci, A.O., Görk, A.S., Rizalar, R. et al. Pentalogy of cantrell. Indian J Pediatr 65, 149–153 (1998). https://doi.org/10.1007/BF02849710

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02849710

Key words

Navigation