The Wrist Sign

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Abstract
The Marfan syndrome is inherited as an autosomal dominant trait, but there is often incomplete penetrance of the gene resulting in a variable phenotype. This variability leads to problems in diagnosis when one is faced with a tall gangly individual with minimal ocular or cardiovascular involvement. In order to confirm the clinical impression of arachnodactyly in patients with the Marfan syndrome, Steinberg1 in 1966 popularized the thumb sign, which was first described by Parker and Hare2 in 1945. This sign is elicited by having the patient make a fist over the clenched thumb. When the result is positive the thumb extends significantly beyond the ulnar margin of the hand (Fig 1). Unfortunately this sign is negative in a sufficiently high proportion of patients with the Marfan syndrome as to reduce its usefulness when used alone (Table). It is also positive in a small proportion of normal children (1.1%

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