Various complications of Le Fort osteotomies have been reported. We describe a lethal complication of Le Fort III osteotomy we encountered in a 9-year-old boy with Crouzon syndrome. A standard Le Fort III osteotomy, including pterygomaxillary dysjunction with a curved osteotome and down-fracture manipulation, was performed uneventfully. When the intraoral buccal wound was closed after fixation of the external midface distraction devices, we discovered hemorrhage originating from the right posterior maxillary region. Although it was stopped with pressure on the osteotomized maxilla, the volume of intraoperative blood loss was nearly 2,000 ml. During the observation period in the intensive care unit, the patient suffered brain death, and he died 3 months later. A computed tomography scan obtained the day after surgery revealed vigorous subarachnoid and intraventricular hemorrhage and transverse fracture of the middle cranial fossa. This skull base fracture was believed to result from intraoperative maneuvers, including the pterygomaxillary dysjunction and down-fracture manipulation. We emphasize the risk of intracranial hemorrhage with Le Fort osteotomy and advise discussing this risk with patients and family members during preoperative consultations.

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