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.
Subject(s)
Female , Hernia, Diaphragmatic/congenital , Hernia, Umbilical/diagnosis , Humans , Infant, Newborn , Male , Pregnancy , Syndrome , Tetralogy of Fallot/diagnosisABSTRACT
Intestinal atresia is the most frequently encountered cause of ileus in the neonate. Of all atresias combination of small and large bowel atresias is extremely rare. In 1973, Guttman presented a case with multiple, widespread atresias of small and large bowel, intraluminal calcifications and a hereditary nature. This paper describes the detailed pathological findings of a similar case of multiple intestinal atresias and discusses for the pathogenesis of this rare condition.