[Anesthetic management of a patient with osteogenesis imperfecta combined with mandibular defect]

Osteogenesis imperfecta (OI) is a rare hereditary disorder characterized by an excessive tendency to bone fractures and retarded growth. We report an anesthetic management of the patient with OI who has the history of vertebral bone fracture by coughing. A 44-year-old female underwent mandibular resection and reconstruction with a metal instrument due to ossifying fibroma 35 years ago. Since then, she had undergone mandibular resection and shaving the instrument several times because of recurrence of the tumor and/or fracture of the instrument. This time, some parts of the instrument were removed under general anesthesia since it had exposed from the skin. Difficulty in mask ventilation and intubation was predicted due to the defect of mandible and some muscles supporting the tongue and the pharynx. Awake fiber-optic nasotracheal intubation, therefore, was performed in consideration of airway obstruction. Dexmedetomidine was administered to reduce the risk of bone fracture in addition to low doses of midazolam and fentanyl. Considering incomplete respiration after extubation, the tracheal tube was extubated after inserting the tube exchanger into the trachea through the tube. The tube exchanger was pulled out after confirming spontaneous respiration and upper airway patency. The patient was cooperative, and respiratory and hemodynamic conditions were stable throughout.