Pentalogy of Cantrell

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...

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Bibliographic Details
Published in:Indian journal of pediatrics 1998, Vol.65 (1), p.149-153
Main Authors: ALI OSMAN KATRANCI, SEMIH GÖRK, A, RIZALAR, R, GÜNAYDIN, M, ARITÜRK, E, BERNAY, F, GÜRSES, N
Format: Article
Language:English
Subjects:
Biological and medical sciences
Cardiology. Vascular system
Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava
Female
Heart
Hernia, Diaphragmatic - diagnosis
Hernia, Umbilical - diagnosis
Hernias, Diaphragmatic, Congenital
Humans
Infant, Newborn
Male
Medical sciences
Pregnancy
Syndrome
Tetralogy of Fallot - diagnosis
Tropical medicine
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Summary: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.
ISSN:0019-5456
0973-7693
DOI:10.1007/BF02849710