• Masui · Sep 2013

    Case Reports

    [Anesthetic management of a child with fibrodysplasia ossificans progressiva (FOP)].

    • Haruka Okubo, Koji Hara, and Takeyoshi Sata.
    • Department of Anesthesiology, University of Occupational and Environmental Health, Kitakyushu 807-8555.
    • Masui. 2013 Sep 1;62(9):1117-9.

    AbstractGeneral anesthesia was successfully performed in a 9-year-old boy with FOP. FOP is a very rare inherited disease of the connective tissue, characterized by progressive heterotopic ossification of skeletal muscles, tendons, and ligaments. Trauma and invasive medical procedures can induce heterotopic ossification. Anesthetic concerns for FOP patients include particular attention to airway management and susceptibility to respiratory complications. Regarding the airway management in general anesthesia, excessive stretching of the jaw and extension of the head may lead to the ankylosis of the temporo-mandibular joint and the neck stiffness. Ankylosis of the costvertebral joints induces restrictive ventilatory impairment, which causes atelectasis and lung infection in the perioperative period. Relating to anesthetic management for a child with FOP, anesthesiologists should keep in mind the prevention of exacerbation of the symptoms and subsequent impairment of activities of daily living postoperatively.

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