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

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


Study objective: 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.

Design: Retrospective review of electronic anesthesia database.

Setting: University hospital.

Measurements: 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.

Main results: 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.

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

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Anesthesia, General / methods*
  • Cervical Vertebrae / surgery*
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Intubation, Intratracheal / methods*
  • Klippel-Feil Syndrome / surgery*
  • Male
  • Retrospective Studies
  • Spinal Fusion*
  • Treatment Outcome