Perioperative Care of a Pediatric Patient With Cockayne Syndrome

Abstract

First described in 1936, Cockayne is an autosomal recessive neurodegenerative disorder characterized by dwarfism, premature aging, impaired development of the nervous system, dental complications, abnormal photosensitivity, and optic atrophy. Of primary concern to anesthesia providers is the potential for a difficult airway related to many physical features including micrognathia, a small mouth, large teeth, overbite, limited mouth opening, and restriction of temporomandibular joint movement. Additional comorbid involvement includes associated coronary artery disease, hypertension, and diabetes. The perioperative implications of Cockayne syndrome are discussed, previous perioperative reports are reviewed, and options for anesthetic care are presented.




J Med Cases. 2017;8(10):330-333
doi: https://doi.org/10.14740/jmc2913w