| Literature DB >> 24995115 |
Jin-Xing Liu1, Rong Hu1, Yu Sun1, Hong Jiang1.
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.Entities:
Keywords: Fibrodysplasia ossificans progressiva; anesthesia
Year: 2014 PMID: 24995115 PMCID: PMC4073776
Source DB: PubMed Journal: Int J Clin Exp Med ISSN: 1940-5901