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Sternal defects associated with congenital pericardial and cardiac defects

Published online by Cambridge University Press:  19 August 2008

Carol M. Cottrill ,
Janet Tamaren  and
Brian Hall
Carol M. Cottrill*
Affiliation:
Department of Pediatrics, University of Kentucky, Lexington, KY, USA
Janet Tamaren
Affiliation:
Department of Pediatrics, University of Kentucky, Lexington, KY, USA
Brian Hall
Affiliation:
Department of Pediatrics, University of Kentucky, Lexington, KY, USA
*
Carol M Cottrill, M D. Department of Pediatrics, University of Kentucky Medical Center, MN - 478, Lexington, KY, 40536, USA. Tel: (606) 323-5494; Fax: (606) 323-3499
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Abstract

We describe three cases of sternal defects of varying severity associated with other congenital anomalies. In the most severe case, both anterior and posterior defects were seen, with near-absence of the sternum and pericardium continuous with a large omphalocele. This resulted in 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 anomalous. The baby had anencephaly and rachischisis. In the intermediate case, a proximal sternal cleft was associated with shortening of the ster num, and absence of the manubrium. Anterior pericardial and diaphragmatic 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 shortened sternum. A supra-umbilical omphalocele contained a left ventricular diverticulum without interposing pericardium or diaphragm. Ventricular and atrial septal defects were present. The first two cases can be considered as representing fail ure of development of both an anterior and a posterior midline field. The third case, much milder than the other two, represents failure of development of an anterior field.

Keywords

Sternal defectsdefects of pericardiumcongenital heart defectsmultiple congenital anomaly syndromespentalogy of Cantrell
Type
Original Articles
Information
Cardiology in the Young , Volume 8 , Issue 1 , January 1998 , pp. 100 - 104
Copyright
Copyright © Cambridge University Press 1998

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References

1. Ravitch, MMCongenital Deformities of the Chest Wall and Their Operative Repair, WB Saunders Co., Philadelphia, London, Toronto, 1977 p. 1–77Google Scholar
2. Hersh, JH, Walterfill, D, Rutledge, J, Harrod, MJE, óShea, SF, Verdi, G, Martinez, S and Weisskopf, BSternal malformation/Vascular dysplasia association. Amer J Med Gen 1985 21:177–186.CrossRefGoogle ScholarPubMed
3. Cantrell, JR, Haller, JA, and Ravitch, MMA syndrome of congenital defects involving the abdominal wall, stern diaphragm, pericardium, and heart. Surgery, Gynecology and Obstetrics 1958, 107:602–614.Google Scholar
4. Carmi, R, and Boughman, JAPentalogy of Cantrell and associated midline anomalies: A possible ventral midline developmental field. Amer J Med Gen 1992, 42:90–95.CrossRefGoogle ScholarPubMed
5. Anderson, RH, Macartney, FJ, Shinebourne, EA, Tynan, M Eds. Abnormal positions and relationships of the heart, in Paediatric Cardiology. Churchill Livingston, London, 1987, pp1058Google Scholar
6. Spitz, M and Gilbert, EFEditorial comment: CNS anomalies and the midline as a developmental field Amer J Med Gen, 1982, 12:443–455.Google Scholar
7. Martin, RA, Cunniff, C, Erickson, L and Jones, KLPentalogy of Cantrell and Ectopia Cordis, a familial developmental field complex. Amer J Med Gen 1992, 42:839–84.CrossRefGoogle ScholarPubMed