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Case Reports
. 1992 Sep 1;44(1):90-3.
doi: 10.1002/ajmg.1320440121.

Upper and lower airway compromise in the Apert syndrome

Affiliations
Case Reports

Upper and lower airway compromise in the Apert syndrome

M M Cohen Jr et al. Am J Med Genet. .

Abstract

Both upper and lower airway compromise may be responsible for early death in some patients with the Apert syndrome. We report on two and review six cases with complete or partial cartilage sleeve abnormalities of the trachea. Possible mechanisms include tracheal stenosis and/or lack of tracheal distensibility which may result in respiratory inefficiency, inability to clear secretions, and/or increased liability to surface injury from tracheal suctioning. Upper airway compromise, consisting of obstructive sleep apnea and cor pulmonale, may result from reduced nasopharyngeal and oropharyngeal dimensions in the Apert craniofacial configuration.

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