Liu Jin-Xing, Hu Rong, Sun Yu, Jiang Hong
Department of Anesthesiology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University Shanghai, China.
Int J Clin Exp Med. 2014 May 15;7(5):1474-9. eCollection 2014.
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.
进行性骨化性纤维发育不良(FOP)是一种罕见且严重的结缔组织遗传性疾病,其特征为异位骨形成和进行性肌肉骨骼残疾。软组织创伤可能会加重这种情况并导致进一步骨化。我们描述了一名计划接受全身麻醉下拔牙的23岁FOP男性患者的麻醉及围手术期管理。术前检查显示多系统受累,包括颅骨和颈椎强直以及严重的限制性肺病。我们为患者选择了经鼻纤维光导气管插管。术中采用全静脉麻醉维持麻醉,整个手术过程中控制通气。气管导管保留用于机械通气直至术后第二天,拔管后进行积极的呼吸物理治疗。此外,对股血管穿刺和动脉置管格外小心。患者体位摆放精细,并使用空气加温毯以尽量减少软组织创伤。未记录到明显的术中及术后不良事件可归因于麻醉。