Some Unusual Features of the Marfan Syndrome

IT IS generally agreed that the Marfan syndrome is a heritable disorder of connective tissue characterized by widespread malformations affecting the musculoskeletal, cardiovascular, and ocular systems. It was first described by Marfan in 1896 under the name of dolichostenomelie.' In 1902 Archard presented a case and called the syndrome arachnodactyly.2 Thus, the musculoskeletal malformations were initially described in this syndrome. Later, attention was drawn to the frequency of associated cardiovascular and ocular abnormalities, particularly ectopia lentis. Other malformations have also been reported such as susceptibility to respiratory infections, developmental malformations of the lung, cystic disease of the lung, and spontaneous pneumothorax. Several reviews covering different aspects of this syndrome have been published.AThe purpose of this paper is to present some unusual findings in four patients with the Marfan syndrome.