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Extracardiac malformations in asplenia syndrome

Published online by Cambridge University Press:  19 August 2008

Steven A. Webber ,
Glenn P. Taylor ,
Kim Colwell ,
George G.S. Sandor  and
Michael W.H. Patterson
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
*
Dr.Steven A. Webber.Division of Pediatric Cardiology, Children's Hospital of Pittsburgh3705 Fifth Avenue at DeSoto Street, Pittsburgh, PA 152 13-2583, USA. Tel. 412-692-5540; Fax. 412-692-5138.
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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.

Keywords

Aspleniaextracardiac anomalies
Type
Original Articles
Information
Cardiology in the Young , Volume 2 , Issue 2 , April 1992 , pp. 136 - 140
Copyright
Copyright © Cambridge University Press 1992

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