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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