Klippel-Feil syndrome is characterized by congenital fusion of two or more cervical vertebrae, a low hair line at the back of the head, restricted neck mobility, and other congenital anomalies. We report a 16-year-old young man with Klippel-Feil syndrome, Sprengel deformity of the right scapula, thoracic kyphoscoliosis, and mandibular prognathism with an anterior open bite. He was treated with orthodontic treatment and maxillofacial surgery. An anticipated difficult airway due to a short neck with restricted neck movements and extrinsic restrictive lung disease due to severe thoracic kyphoscoliosis increased his anesthesia risk. Due to his deviated nasal septum and contralateral inferior turbinate hypertrophy, we chose awake fiber optic orotracheal intubation followed by submental intubation. Considering the cervical vertebral fusion, he was carefully positioned during surgery to avoid potential spinal injury. He recovered well and his postoperative course was uneventful.
Adolescent ; Anesthesia ; Cervical Vertebrae ; Congenital Abnormalities ; Female ; Genioplasty ; Hair ; Head ; Humans ; Hypertrophy ; Intubation ; Klippel-Feil Syndrome ; Lung Diseases ; Mandible ; Nasal Septum ; Neck ; Open Bite ; Orthognathic Surgery ; Osteotomy ; Prognathism ; Scapula ; Spinal Injuries ; Surgery, Oral ; Turbinates

Complex cervical spine fractures are a serious complications of maxillofacial trauma and associated with high mortality and neurological morbidity. Strict vigilance in preventing further insult to the cervical spine is a crucial step in managing patients who are at risk for neurologic compromise. We report a rare case of a right transverse process of atlas fracture with right-sided vertebral artery injury that was associated with a comminuted fracture of the body and angle of the mandible, which restricted mouth opening. Airway management was performed by an awake fiber-optic nasotracheal intubation, where neck movement was avoided with a cervical collar. Vertebral artery injuries may have disastrous consequences, such as basilar territory infarction and death, and should be suspected in patients with head and neck trauma. After mandibular plating, the patient was on cervical collar immobilization for 12 weeks and anti-coagulant therapy.
Airway Management ; Fractures, Comminuted ; Head ; Humans ; Immobilization ; Infarction ; Intubation ; Mandible ; Mandibular Fractures ; Mortality ; Mouth ; Neck ; Spinal Fractures ; Spine ; Vertebral Artery