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(Chest. 1969;55:301-305.)
© 1969 American College of Chest Physicians

Congenital Herniation of the Large Bowel Into the Pericardial Cavity

Survival into Adulthood Despite a Generalized Connective Tissue Disorder

Arthur J. Moss M.D.1 and David J. Harris M.D.2

1 Assistant Professor of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York
2 Resident in Pediatrics, Stanford University School of Medicine, Palo Alto, California

An adult with an unusual combination of mesodermal defects is presented. A striking finding was the herniation of a major portion of the large bowel into the pericardial cavity through a foramen of Morgagni defect. In addition, the patient exhibited a variety of muscular skeletal anomalies, Monckeberg sclerosis of the muscular arteries, and complete heart block. Urinary mucopolysaccharide excretion and a chromosome count were normal. The most likely explanation of the observed findings is a biochemical defect in connective tissue.






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Copyright © 1969 by the American College of Chest Physicians.