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November 1959

Lens Surgery in Marfan's Syndrome

AMA Arch Ophthalmol. 1959;62(5):764-767. doi:10.1001/archopht.1959.04220050026004

Marfan's syndrome, arachnodactyly, is a hereditary condition believed to be caused by disease of the connective tissues, mainly a defective production or excess destruction of chondroitin sulfate. The latter is an important ingredient of the ground substance of the connective tissues. All the features characteristic of Marfan's syndrome—kyphoscoliosis, periosteal new bone formation, and dissecting aneurysms of the aorta—have been reproduced in growing rats fed with diets containing 50% sweet-pea pods, a diet which interferes with the effective utilization of chondroitin sulfate. Aneurysmal dilatation of the aortic sinuses are so characteristic of this condition that its presence leads to a presumptive diagnosis of Marfan's syndrome and the search for other stigmata.

Marfan's syndrome is not frequently seen in an average office practice but, when encountered leads to very vexing problems. If dislocated lenses are present, vision is usually very poor and the children are handicapped in their proper development. Not only

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