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Report of One Case with Autopsy, Special Histological Study, and Review of the Literature

JOHN W. ROARK, M.D.

AMA Arch Intern Med. 1959;103(1):123-132.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

The Marfan syndrome, or arachnodactyly, is a relatively rare familial disorder of unknown etiology characterized by widespread abnormalities of the skeletal, cardiovascular, and ocular systems in which a generalized elastic tissue disorder has often been suggested as the primary fault in pathogenesis.^1,2

It will become apparent below that the cardiac lesions of the Marfan syndrome may involve any valve or portion of the heart and aorta, as well as the direct branches of the latter. This syndrome is unusual, but its importance has increased with the era of cardiovascular surgery.^1-3 In 1896, Marfan,⁴ in reporting the first case, described disproportionately long extremities and long spidery fingers. Achard,⁵ coined the term "arachnodactyly," but the eponym, preferred by some,¹ has continued to be used. General abnormalities, including arachnodactyly, relaxation of joints, lack of muscle tone, scant subcutaneous panniculus, dolichocephaly, high arched palate, large misshapen ears, pigeon breast, kyphoscoliosis pectus excavatum, and pes . . . [Full Text PDF of this Article]

Author Affiliations
Alexandria, Va.

Footnotes
Submitted for publication March 31, 1958.

McKusick preferred the designation, "the Marfan Syndrome," since Marfan described only skeletal abnormalities, while the syndrome encompasses cardiovascular and eye involvement described later by others (personal communication).

The study reported herein was done during a tenure as Instructor in Internal Medicine and Fellow in Pathology, University of Virginia Department of Medicine, Charlottesville, Va. Present address: 312 S. Washington St., Alexandria, Va.

The following people helped in reviewing tissue sections or editing the mansucript: Dr. Edward P. Cawley, Professor of Dermatology and Syphilogy; Dr. James E. Kindred, Professor of Anatomy; Dr. William Parson, Professor of Internal Medicine, and Dr. David E. Smith, Professor of Pathology, University of Virginia Department of Medicine; Dr. Victor A. McKusick, Associate Professor of Medicine, The Johns Hopkins University School of Medicine, Baltimore.

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