title: High Le Fort I and bilateral split sagittal osteotomy in Crouzon syndrome.
abstract: Crouzon syndrome is a rare, autosomal dominant disease from a fibroblast growth factor receptor 2 gene mutation, characterized by premature craniosynostosis, hypertelorism, orbital proptosis, psittichorina, hypoplastic maxilla, and mandibular prognathism. We present an adult 32-year-old Crouzon syndrome patient who underwent an elective High Le Fort I and bilateral split sagittal osteotomy for midface advancement with a background of jaw malocclusion and obstructive respiratory symptoms. The operation features a potential dynamic movement of the secured airway in the surgical field and close proximity to exposed ocular structures. Permissive hypotensive anesthesia was employed to improve the surgical field and reduce intraoperative blood loss and dose of long-acting opioids. He was extubated at the end of an uneventful surgery and was monitored in the high dependency overnight before he was discharged to the general ward. Perioperative issues include potential difficult airway management; ocular, auditory, and neurological injury prevention; surgery-specific anesthetic technique; and postoperative analgesia. Understanding the multisystemic issues facilitates the dynamic anesthetic management during surgery. Good communication among the multidisciplinary team is essential to ensure a successful operation and uneventful recovery.
authors.affiliation: Department of Anaesthesiology, Singapore General Hospital, Singapore, Singapore. tay.yoong.chuan@sgh.com.sg
authors.forename: Yoong Chuan
authors.initials: YC
authors.lastname: Tay
authors.forename: Kian Hian
authors.initials: KH
authors.lastname: Tan
authors.forename: Vincent Kok-Leng
authors.initials: VK
authors.lastname: Yeow
mesh_terms: D000328:Adult; D058109:Airway Management / Q000379:methods; D000402:Airway Obstruction / Q000601:surgery; D016058:Analgesia, Patient-Controlled; D018686:Anesthetics, Intravenous / Q000008:administration & dosage; D001279:Atracurium / Q000008:administration & dosage; D016063:Blood Loss, Surgical / Q000517:prevention & control; D003394:Craniofacial Dysostosis / Q000601:surgery*; D006801:Humans; D007023:Hypotension, Controlled / Q000379:methods; D007442:Intubation, Intratracheal / Q000379:methods; D008297:Male; D008310:Malocclusion / Q000601:surgery; D009020:Morphine / Q000627:therapeutic use; D009294:Narcotics / Q000627:therapeutic use; D003473:Neuromuscular Nondepolarizing Agents / Q000008:administration & dosage; D056948:Orthognathic Surgical Procedures / Q000379:methods; D019340:Osteotomy, Le Fort / Q000379:methods*; D059229:Osteotomy, Sagittal Split Ramus / Q000379:methods*; D010880:Piperidines / Q000008:administration & dosage; D011378:Prognathism / Q000601:surgery; D000077208:Remifentanil
chemical_list: D018686:Anesthetics, Intravenous; D009294:Narcotics; D003473:Neuromuscular Nondepolarizing Agents; D010880:Piperidines; D001279:Atracurium; D009020:Morphine; D000077208:Remifentanil