摘要
先天性无痛无汗症(CIPA)伴夏科氏关节病是一种罕见的临床综合征,目前对此类患者的治疗经验有限。通过1例长达10年的患者随访,望就手术策略的选择及有关术后并发症方面为临床医生提供新的思路。该例患者确诊为CIPA,并出现了严重的夏科氏关节病,脊柱受累为著。手术治疗后发生了多次术后并发症,包括植入物移位、脊柱邻近节段病变和椎弓根螺钉松动等,后续进行了5次翻修手术。结合文献,在处理CIPA相关夏科氏脊柱关节病的有限经验中,手术矫形仍是首选治疗方法。本文回顾的16例病例中,植入物移位、脊柱邻近节段病变和椎弓根螺钉松动是常见的术后并发症。基于目前经验,不建议大范围切除受损椎体后重建,这可能会增加植入物移位风险;而360°长节段融合可能有助于减少邻近节段病变的风险。此外,本文还讨论了夏科氏脊柱关节病手术后翻修的潜在原因,以及此类手术病例的围术期管理策略。细致的护理、合理的康复锻炼和骨矿物代谢治疗,对于该病的治疗都至关重要。
Congenital insensitivity to pain with anhidrosis(CIPA)is associated with Charcot arthropa-thy and is a rare clinical syndrome,with limited treatment options.Through a decade-long follow-up of a sin-gle case,we aim to provide new insights for clinicians regarding the choice of surgical strategies and postoper-ative complications.The diagnosed patient exhibited congenital insensitivity to pain and anhidrosis,accompa-nied by severe Charcot arthropathy affecting the spine.Multiple postoperative complications,including implant displacement,adjacent segment pathology,and pedicle screw loosening,occurred after surgical inter-vention,leading to five subsequent revision surgeries.Considering the limited experience in managing CIPA-related Charcot spinal arthropathy in the literature,surgical correction remains the preferred treatment.Among the 16 cases reviewed,common postoperative complications included implant displacement,adjacent segment pathology,and pedicle screw loosening.Based on current experience,we do not recommend extensive re-section and reconstruction after removing the affected vertebral body,as this may increase the risk of im-plant displacement.Instead,a 360°long-segment fusion may help reduce the risk of adjacent segment de-generation.Additionally,we discuss potential reasons for revision surgery after Charcot spinal arthropathy surgery and perioperative management strategies for such cases.Meticulous care,appropriate rehabilitation exercises,and metabolic therapy for bone mineralization are crucial components of the treatment for this condition.
作者
焦禹豪
田野
蔡思逸
JIAO Yuhao;TIAN Ye;CAI Siyi(Internal Medicine Department,Beijing Key Laboratory for Genetic Research of Skeletal Deformity,Key Laboratory of Big Data for Spinal Deformities,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Orthopaedic Surgery,Beijing Key Laboratory for Genetic Research of Skeletal Deformity,Key Laboratory of Big Data for Spinal Deformities,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)
出处
《罕见病研究》
2023年第4期616-625,共10页
Journal of Rare Diseases
关键词
先天性无痛无汗症
夏科氏脊柱关节病
手术治疗
并发症
congenital insensitivity to pain with anhidrosis
Charcot spine arthropathy
surgical treatment
complications
