Li Jianzhong, Zhang Mingming, Dong Dalong, Xu Hong, Wang Ankui, Liu Qi, Duan Lei
Department of Anesthesiology, Norinco General Hospital, 12 Zhangba East Rd, Yanta District, Xi'an City, 710065, Shaanxi Province, China.
Department of Anesthesiology, Xi'an Aerospace General Hospital, 159 Jitai Road, Chang'an District, Xi'an City, 710100, Shaanxi Province, China.
BMC Anesthesiol. 2025 Apr 9;25(1):163. doi: 10.1186/s12871-025-03026-w.
Congenital insensitivity to pain with anhidrosis (CIPA), also known as hereditary sensory and autonomic neuropathies (HSAN I-V), is an exceptionally rare autosomal recessive disorder. The pathogenesis of CIPA remains not fully elucidated. Clinical manifestations primarily include the absence of pain perception, painless injuries to the extremities, oral mutilation, anhidrosis accompanied by hyperthermia, and delayed intellectual development. Given the autonomic dysfunction in CIPA patients, previous reports on anesthetic management for this rare disorder have predominantly depended on general anesthesia or neuraxial anesthesia, both of which pose significant challenges. This case report presents a unique approach to anesthetic management in a child with CIPA, who successfully underwent hollow nail internal fixation for femoral neck fracture under orthopedic navigation using ultrasound-guided femoral nerve block combined with lateral femoral cutaneous nerve block. The patient was sedated with dexmedetomidine and did not require any opioids during the procedure. Her vital signs remained stable throughout the surgery, and her recovery was uneventful, with discharge occurring 5 days post-operation. This case contributes to the medical literature by demonstrating a safe and effective anesthetic strategy in a pediatric CIPA patient, highlighting the potential of regional anesthesia techniques as a viable alternative to general anesthesia or neuraxial anesthesia in such high-risk cases.
先天性无痛无汗症(CIPA),也称为遗传性感觉和自主神经病(HSAN I-V),是一种极为罕见的常染色体隐性疾病。CIPA的发病机制尚未完全阐明。临床表现主要包括无痛觉、肢体无痛性损伤、口腔自残、无汗伴体温过高以及智力发育迟缓。鉴于CIPA患者存在自主神经功能障碍,既往关于这种罕见疾病麻醉管理的报道主要依赖全身麻醉或椎管内麻醉,而这两种麻醉方式都带来了重大挑战。本病例报告展示了一种针对CIPA患儿独特的麻醉管理方法,该患儿在骨科导航下,通过超声引导股神经阻滞联合股外侧皮神经阻滞成功接受了股骨颈骨折空心钉内固定术。术中患者使用右美托咪定镇静,未使用任何阿片类药物。整个手术过程中她的生命体征保持稳定,术后恢复顺利,术后5天出院。本病例通过展示在儿科CIPA患者中一种安全有效的麻醉策略,为医学文献做出贡献,突出了区域麻醉技术在这类高风险病例中作为全身麻醉或椎管内麻醉可行替代方案的潜力。