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A 16-year-old female with Klippel-Feil syndrome on home biphasic positive airway pressure (BiPAP) presents for bilateral lower-extremity osteotomies.


Airway examination shows a Mallampati class 3 airway with a normal mouth opening, a reduced thyromental distance (3 cm), and inability to flex or extend the neck.




Klippel-Feil syndrome is the result of fusions of the cervical vertebrae such that the head appears to sit on the shoulders. The degree of severity is variable: type 1 patients have fusion of C2 and C3 with occipitalization of the atlas, type 2 patients have long fusion below C2 with an abnormal occipital-cervical junction, and type 3 patients have the presence of thoracic and lumbar spine anomalies in association with types 1 and 2. It is characterized by a short, possibly webbed neck, a low posterior hairline, and limited mobility of the cervical spine. Numerous associated abnormalities may be present, including atlantooccipital fusion, skull malformations, facial asymmetry, hearing loss, cleft lip, micrognathia, primary and permanent oligodontia, torticollis, malformed laryngeal cartilage with voice abnormalities, scoliosis, thoracic or lumbar vertebral anomalies, genitourinary abnormalities, Sprengel’s deformity (wherein the scapulae ride high on the back), synkinesia, sacral agenesis, and cardiovascular abnormalities, specifically ventricular septal defect.


Patients with hypermobility of the upper cervical spine are at risk for neurological sequelae, such as paraplegia, hemiplegia, and cranial or cervical nerve palsies, whereas those with limited mobility in the lower cervical spine are more at risk for the development of degenerative disease. Syncope may be induced with sudden neck rotation.


Preoperative lateral flexion-extension radiographs of the cervical spine to identify patients with cervical spine instability should be considered.




  • An unstable cervical spine raises the possibility of neurological insult with head manipulation and positioning.

  • Mask ventilation is difficult.

  • Direct laryngoscopy and tracheal intubation may be extremely difficult or impossible owing to limited mobility of the cervical spine.

  • A previous uncomplicated tracheal intubation does not assure repeated easy success, since cervical fusion is progressive.

  • Adequacy of mask ventilation should be determined. At this point, the airway can be visualized or managed with a variety of airway adjuncts.

  • Use awake intubation or sedated fiberoptic intubation, while maintaining spontaneous ventilation, using dexmedetomidine and/or ketamine.

  • When patients have significant difficulty with ventilation or intubation, aside from oral, pharyngeal, and nasal airways, a suture (0 silk) can be placed at the base of the tongue to displace the tongue anteriorly to assist with ventilation or intubation.

  • If oral intubation is not desirable because of the nature of the surgery, nasal intubation (which may be difficult because of narrow anterior nares) or tracheostomy may be required. Tracheostomy may be difficult if the patient has had a previous tracheostomy as a child.




Extubation should be performed when the patient is fully awake and in the ...

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