Rare hereditary disorder of connective tissue .
Affected persons are tall, with long, thin limbs and spiderlike fingers (arachnodactyly). The lens of the eye is dislocated, and many have glaucoma or detached retina . Heart muscle abnormalities and various malfunctions and malformations occur; rupture of the aorta is the most common cause of death. Severity varies; affected individuals may die young or live essentially normal lives. The underlying abnormality cannot be cured, but some of the defects can be surgically corrected.