Paramaswamy Rathna
Department of Anaesthesiology, Saveetha Medical College and Hospital, Chennai, India.
J Dent Anesth Pain Med. 2019 Oct;19(5):307-312. doi: 10.17245/jdapm.2019.19.5.307. Epub 2019 Oct 30.
Klippel-Feil syndrome is characterized by congenital fusion of two or more cervical vertebrae, a low hair line at the back of the head, restricted neck mobility, and other congenital anomalies. We report a 16-year-old young man with Klippel-Feil syndrome, Sprengel deformity of the right scapula, thoracic kyphoscoliosis, and mandibular prognathism with an anterior open bite. He was treated with orthodontic treatment and maxillofacial surgery. An anticipated difficult airway due to a short neck with restricted neck movements and extrinsic restrictive lung disease due to severe thoracic kyphoscoliosis increased his anesthesia risk. Due to his deviated nasal septum and contralateral inferior turbinate hypertrophy, we chose awake fiber optic orotracheal intubation followed by submental intubation. Considering the cervical vertebral fusion, he was carefully positioned during surgery to avoid potential spinal injury. He recovered well and his postoperative course was uneventful.
克利佩尔-费尔综合征的特征是两个或更多颈椎先天性融合、头后部发际线低、颈部活动受限以及其他先天性异常。我们报告一名16岁患有克利佩尔-费尔综合征、右肩胛骨先天性高肩胛畸形、胸椎后凸侧弯以及下颌前突伴前牙开颌的青年男性。他接受了正畸治疗和颌面外科手术。由于颈部短且活动受限导致预期的困难气道,以及严重胸椎后凸侧弯引起的外在限制性肺病,增加了他的麻醉风险。由于他鼻中隔偏曲和对侧下鼻甲肥大,我们选择清醒纤维光导经口气管插管,随后行颏下插管。考虑到颈椎融合,手术期间他被小心安置体位以避免潜在的脊柱损伤。他恢复良好,术后过程平稳。