Highlights spontaneous atlantoaxial subluxation as a potential non-cardiovascular cause of sudden death in patients with Marfan syndrome.
Alerts clinicians to rare non-cardiac sudden death in Marfan syndrome; hypothesis-generating and requires validation in larger cohorts.
Marfan syndrome is 1 of the commonest inherited connective tissue disorders. Sudden death may occur and is usually attributed to cardiovascular manifestations of the syndrome. Atlantoaxial hypermobility, increased odontoid height, and rotatory subluxation are well described in this syndrome, but this paper details what seems to be the first reported case of sudden and unexpected death due to spontaneous atlantoaxial subluxation in Marfan syndrome.
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MacKenzie et al. (2003) studied this question.
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