A patient with Klippel-Feil Syndrome may have a number of airway-related problems in the perioperative period. This may pose problems if emergency intubation is needed.
Classic triad for the Klippel-Feil Syndrome:
(1) short neck
(2) low posterior hairline
(3) fused cervical vertebrae with restricted neck motion
Other findings may include:
(1) micrognathia, mandibular hypoplasia
(2) limited mouth opening
(3) cleft lip and/or palate
(4) scoliosis
(5) congenital heart disease
(6) impaired nutrition
(7) other skeletal defects
(8) cervical instability
Perioperative issues:
(1) difficult intubation
(2) need for reintubation
(3) neurological injury related to cervical instability
(4) cardiac complications related to congenital heart disease
(5) aspiration
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