PEDIATRICS Vol. 84 No. 1 July 1989, pp. 83-89
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Severe Perinatal Marfan Syndrome

Donald M. Gross MD, CM1, Luther K. Robinson MD1, Lynne T. Smith PhD1, Nancy Glass MD1, Harvey Rosenberg MD1, and Madeleine Duvic MD1

1 The University of Texas Medical School at Houston, Departments of Pediatrics, Pathology, and Internal Medicine/Dermatology, Houston, and the University of Washington, Department of Biologic Structure, Seattle

The cardiovascular manifestations of the Marfan syndrome in older children and adults have been well described. Clinical, radiographic, and echocardiographic data regarding three patients with severe perinatal Marfan syndrome are described. Two of these patients had the syndrome at birth and died in infancy. The syndrome was diagnosed in the third patient at 6 months of age and the child is still alive at 3 years of age. The possible relationship among the Marfan syndrome, Ehlers-Danlos syndrome, and osteogenesis imperfecta is considered. Patients with Marfan syndrome and severe cardiorespiratory problems early in life tend to have a limited life expectancy.

Key Words: Marfan syndrome • cardiorespiratory problem • dermatologic findings

Submitted on February 16, 1988
Accepted on April 26, 1988




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