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October 25, 1971

Marfan's Syndrome

JAMA. 1971;218(4):597. doi:10.1001/jama.1971.03190170075032

To the Editor.—  In a recent report (217:335,1971) a 26-year-old man with "Marfan's Syndrome with Complete Heart Block and Junctional Rhythm" was described. The diagnosis of Marfan's syndrome was based on arachnodactyly, dolichocephaly, congenital cataracts, high arched palate, absence of subcutaneous tissue, and arm span greater than height by 5 cm.This diagnosis must be questioned.The Marfan syndrome is a heritable disorder of connective tissue with characteristic ocular, skeletal, and vascular abnormalities. The patient described had arachnodactyly and dolichocephaly and the quoted indexes support this. Cataracts in patients with the Marfan's syndrome only occur secondary to the uveitis, retinal detachment, and glaucoma resulting from ectopia lentis. Cataract without ectopia lentis is not a part of this syndrome. A cardiac catheterization study revealed no aortic dilatation or insufficiency nor any valvular lesion to support the diagnosis in this patient of Marfan's syndrome.In the absence of a family history

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