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Abstract
Osteogenesis imperfecta, a rare connective tissue disorder, is known to be associated
sometimes with the invagination of the basilar skull. This deformity may disturb respiratory
function secondary to brain stem compression and hydrocephalus. In addition, the deformed
thoracic cage and fragile ribs make pulmonary care more complicated. A case of 24-year-old
man is presented with brain stem compression syndrome secondary to osteogenesis imperfecta
congenita with basilar impression. He developed respiratory failure and became tracheostomy
positive-pressure ventilator dependent at the age of 21 years. He also suffered multiple
skeletal abnormalities and mental retardation, and following the brain stem compression,
severe quadriparesis. The patient's condition is stable since he has been using the
ventilator and he is currently living in the community.
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Article info
Publication history
Accepted:
November 23,
1993
Received:
July 30,
1993
Identification
Copyright
© 1994 Published by Elsevier Inc.
