Repeated anesthetic management for a patient with Klippel-Feil syndrome

Klippel-Feil syndrome (KFS) is a rare disease characterized by a classic triad comprising a short neck, a low posterior hairline, and restricted motion of the neck due to fused cervical vertebrae. We report repeated anesthetic management for orthognathic surgeries for a KFS patient with micrognathia. Because KFS can be associated with a number of other anomalies, we therefore performed a careful preoperative evaluation to exclude them. The patient had an extremely small mandible, significant retrognathia, and severe limitation of cervical mobility due to cervical vertebral fusion. As difficult intubation was predicted, awake nasal endotracheal intubation with a fiberoptic bronchoscope was our first choice for gaining control of the patient's airway. Moreover, the possibility of respiratory distress due to postoperative laryngeal edema was considered because of the surgeries on the mandible. In the operating room, tracheotomy equipment was always kept ready if a perioperative surgical airway control was required. Three orthognathic surgeries and their associated anesthetics were completed without a fatal outcome, although once the patient was transferred to the intensive care unit for precautionary postoperative airway management and observation. Careful preoperative examination and preparation for difficult airway management are important for KFS patients with micrognathia.