STERNAL DEFECTS ASSOCIATED WITH CONGENITAL PERICARDIAL AND CARDIAC DEFECTS

We describe three cases of sternal defects of varying severity associa ted with other congenital anomalies. In the most severe case, both ant erior and posterior defects were seen, with near-absence of the sternu m and pericardium continuous with a large omphalocele. This resulted i n external location of organs usually confined within the thoracic and abdominal cavities. A ventricular septal defect was present, and the arterial duct was absent. The course of the ascending aorta was anomal ous. The baby had anencephaly and rachischisis. In the intermediate ca se, a proximal sternal cleft was associated with shortening of the ste rnum, and absence of the manubrium. Anterior pericardial and diaphragm atic defects were seen, while a scalp defect and an encephalocele were present on the posterior aspect of the head. This baby had tricuspid atresia. The qremaining case had only an anterior defect with a shorte ned sternum. A supra-umbilical omphalocele contained a left ventricula r diverticulum without interposing pericardium or diaphragm. Ventricul ar and atrial septal defects were present. The first two cases can be considered as representing failure of development of both an anterior and a posterior midline field. The third case, much milder than the ot her two, represents failure of development of an anterior field.