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J Clin Anesth. 2008 Sep;20(6):447-51. doi: 10.1016/j.jclinane.2008.04.009.

Congenital cervical spine fusion and airway management: a case series of Klippel-Feil syndrome.

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Department of Anesthesiology, University of Michigan Medical School, Ann Arbor, MI 48109-0048, USA.



To determine the ideal airway management modalities in general anesthesia for pediatric patients with Klippel-Feil syndrome, a disorder that is characterized by abnormalities of the cervical spine and craniofacial structures that may impede successful airway management.


Retrospective review of electronic anesthesia database.


University hospital.


The electronic charts of 10 pediatric patients with Klippel-Feil syndrome (KFS) who received treatment at our institution from 2005 to 2007 were reviewed with a focus on diagnosis, level of lesion, comorbid diseases, age, procedure, history of perioperative difficulties, and airway management during general anesthesia.


A total of 10 pediatric patients, 6 girls and 4 boys, underwent 11 procedures from 2005 to 2007. Average age was 11 (range, 4-16 yrs). Six patients were ASA physical status II and 4 were ASA physical status III. Four patients had spinal fusion, 6 underwent magnetic resonance imaging, and one patient underwent removal of halo hardware. Eight patients had undergone previous surgeries; of the 8, one patient had a history of difficult intubation. Six patients underwent tracheal intubations, 4 had a Laryngeal Mask Airway (LMA; Orthovent Intafix, Maidenhead, UK) placed, and one had mask ventilation with an oral airway. Of 6 tracheal intubations, 5 were achieved on the first or second attempt without difficulty. Three had grade 1 laryngoscopic views; only one patient needed fiberoptic intubation. Because the fiberoptic intubation took several attempts, it was noted as a difficult intubation in the chart. All LMAs were placed without difficulty, and the cases were uneventful.


The airways of individuals with KFS may be successfully managed in a variety of ways, often with little degree of difficulty.

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