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Am J Perinatol 1987; 4(2): 134-137
DOI: 10.1055/s-2007-999756
ORIGINAL ARTICLE

© 1987 by Thieme Medical Publishers, Inc.

Pregnancy Management and Successful Outcome of Ehlers-Danlos Syndrome Type IV

Paul J. Weinbaum, Suzanne B. Cassidy, Winston A. Campbell, Frederick R. Rickles, Anthony M. Vintzileos, David J. Nochimson, Petros Tsipouras
  • Departments of Obstetrics and Gynecology, Pediatrics, and Medicine, The University of Connecticut Health Center, Farmington, Connecticut; and the Department of Pediatrics, University of Medicine and Dentistry of New Jersey, Rutgers Medical School, Piscattaway, New Jersey
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Publication Date:
04 March 2008 (online)

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ABSTRACT

Ehlers-Danlos syndrome type IV (EDS IV) is a rare but serious disorder of connective tissue that is characterized by the abnormal production or secretion of type III collagen. Experience with pregnant patients exhibiting this syndrome is limited. This report describes the successful management of a pregnancy in a woman with EDS IV that included activity restriction and complete bed rest after 32 weeks, use of 1-desamino-8-D-arginine vasopressin (DDAVP) before delivery to improve coagulation, and cesarean section before the onset of labor. The possibility of prenatal diagnosis based on genetic linkage studies also is discussed.