Kokita Atsushi, Chaki Tomohiro, Yamakage Michiaki
Department of Anesthesiology, Sapporo Medical University School of Medicine, South 1, West 16, Chuo-Ku, Sapporo, Hokkaido, 060-8543, Japan.
JA Clin Rep. 2023 Jun 12;9(1):33. doi: 10.1186/s40981-023-00627-7.
Hajdu-Cheney syndrome (HCS) is an extremely rare disorder characterized by progressive acro-osteolysis. A unique facial structure and deformity of the cervical spine are associated with a difficult airway. Although several reports describe general anesthesia with orotracheal intubation for patients with HCS, there have been no reports of nasotracheal intubation with a risk of skull base fracture. We describe nasotracheal intubation for oral surgery in a patient with HCS.
A 13-year-old girl with HCS was scheduled for dental surgery. Preoperative computed tomography revealed no abnormalities including fractures in the skull base or cervical spine. After confirming a lack of vocal cord paralysis by bronchofiberscopic inspection from the nose, general anesthesia was induced with sevoflurane, remifentanil, and rocuronium. Fiber-optic nasotracheal intubation was successfully performed without complications such as depletion of oxygen saturation and massive epistaxis, and the surgery was completed uneventfully. She was discharged the day after surgery with no anesthesia-related complications.
We were able to safely manage the airway of a patient with HCS by nasotracheal intubation under general anesthesia.
哈伊杜-切尼综合征（HCS）是一种极为罕见的疾病，其特征为进行性肢端骨质溶解。独特的面部结构和颈椎畸形与气道困难相关。尽管有几篇报道描述了HCS患者经口气管插管全身麻醉的情况，但尚无关于存在颅底骨折风险的鼻气管插管的报道。我们描述了1例HCS患者口腔手术的鼻气管插管情况。
一名13岁HCS女童拟行牙科手术。术前计算机断层扫描显示无异常，包括颅底或颈椎骨折。经鼻纤维支气管镜检查确认无声带麻痹后，用七氟醚、瑞芬太尼和罗库溴铵诱导全身麻醉。成功实施了纤维支气管镜引导下鼻气管插管，未出现诸如氧饱和度降低和大量鼻出血等并发症，手术顺利完成。术后第1天她出院，无麻醉相关并发症。
我们能够在全身麻醉下通过鼻气管插管安全地处理HCS患者的气道。
