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Extracardiac malformations in asplenia syndrome

Published online by Cambridge University Press:  19 August 2008

Steven A. Webber
Affiliation:
From the Division of Cardiology, Department of Pediatrics, and the Departments of Cardiothoracic Surgery, Medical Genetics and Pathology, University of British Columbia, Vancouver
Glenn P. Taylor
Affiliation:
From the Division of Cardiology, Department of Pediatrics, and the Departments of Cardiothoracic Surgery, Medical Genetics and Pathology, University of British Columbia, Vancouver
Kim Colwell
Affiliation:
From the Division of Cardiology, Department of Pediatrics, and the Departments of Cardiothoracic Surgery, Medical Genetics and Pathology, University of British Columbia, Vancouver
George G.S. Sandor
Affiliation:
From the Division of Cardiology, Department of Pediatrics, and the Departments of Cardiothoracic Surgery, Medical Genetics and Pathology, University of British Columbia, Vancouver
Michael W.H. Patterson
Affiliation:
From the Division of Cardiology, Department of Pediatrics, and the Departments of Cardiothoracic Surgery, Medical Genetics and Pathology, University of British Columbia, Vancouver

Summary

The incidence and spectrum of extracardiac malformations in congenital asplenia syndrome were determined by review of43 cases (40 live-births) identified in the province of British Columbia over a 21 year period. Major extracardiac anomalies (excluding those of abnormal arrangement of the organs) were identified in nine cases (21%), and minor anomalies were present in an additional twelve infants (28%). Extracardiac anomalies were associated with one spontaneous stillbirth, one termination of pregnancy, and two early neonatal deaths. In an additional four newborns, they contributed to a decision not to palliate the complex cardiac anomalies. Gastrointestinal malformations led to neonatal intestinal obstruction in four infants. Of the 14 surviving children, one is severely handicapped due to a congenital anomaly of the central nervous system. The clinical significance of extracardiac malformations and their developmental implications are discussed.

Type
Original Articles
Copyright
Copyright © Cambridge University Press 1992

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