title: General anesthesia in fibrodysplasia ossificans progressive: a case report and clinical review.
abstract: Fibrodysplasia ossificans progressiva (FOP) is a rare and devastating genetic disorder of connective tissue characterized by heterotopic bone formation and progressive musculoskeletal disability. Soft-tissue trauma may exacerbate this condition and lead to further ossification. We described the anesthetic and perioperative management of a 23-year-old male with FOP scheduled for dental extraction requiring general anesthesia. Preoperative examination revealed multisystem involvement including cranial and cervical ankylosis and severe restrictive lung disease. Nasal fiber-optic endotracheal intubation was chosen in our patient. Anesthesia was maintained with total intravenous anesthesia and ventilation was controlled throughout the surgery. Endotracheal tube was remained for mechanical ventilation until the second postoperative day and aggressive respiratory physiotherapy was performed after extubation. Additionally, extreme caution was taken to a femoral vascular access and an arterial catheter. Positioning of the patient was meticulous and air warming blanket was used to minimize soft tissue trauma. No significant documented intraoperative and postoperative adverse events appeared attributable to the anesthesia.
authors.affiliation: Department of Anesthesiology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University Shanghai, China.
authors.forename: Jin-Xing
authors.initials: JX
authors.lastname: Liu
authors.affiliation: Department of Anesthesiology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University Shanghai, China.
authors.forename: Rong
authors.initials: R
authors.lastname: Hu
authors.affiliation: Department of Anesthesiology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University Shanghai, China.
authors.forename: Yu
authors.initials: Y
authors.lastname: Sun
authors.affiliation: Department of Anesthesiology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University Shanghai, China.
authors.forename: Hong
authors.initials: H
authors.lastname: Jiang
keywords: Fibrodysplasia ossificans progressiva; anesthesia