脊髓性肌萎缩症患者脊柱矫形围手术期治疗药物管理

doi:10.12372/jcp.2025.24e1160

摘要/Abstract

摘要：

脊髓性肌萎缩症（SMA）是一种致命的遗传性神经肌肉罕见病，患者往往伴有不同程度的脊柱畸形。脊柱矫形手术作为改善脊柱畸形的常用手段，其风险较高，可能会引发感染、出血、神经损伤等并发症。鉴于这些风险，合理的围手术期用药对于控制合并症以及促进患者康复至关重要。然而，由于特殊的生理病理状态且缺乏统一的标准规范，SMA患者脊柱矫形围手术期的药物治疗管理面临诸多挑战。现本文对SMA患者脊柱矫形围手术期的药物治疗管理进行综述，旨在为临床提供循证依据和合理用药参考。

关键词: 脊髓性肌萎缩症, 脊柱矫形术, 围手术期, 治疗药物管理

Abstract:

Spinal muscular atrophy (SMA), a devastating hereditary neuromuscular disease, is often complicated by scoliosis. While scoliosis correction surgery is frequently indicated to correct spinal deformities, it is not without significant risks, including the potential for infection, hemorrhage, and neurological damage. Consequently, the management of perioperative medications is of paramount importance for the prevention and control of these complications, as well as for facilitating patient recovery. However, the distinctive physiological and pathological characteristics of SMA, coupled with the lack of standardized protocols, present considerable challenges in perioperative medication management. This review article offers an in-depth examination of the perioperative medication therapy management for patients with SMA undergoing scoliosis surgery. It aims to establish an evidence-based framework and provide a reference for the judicious use of drugs in clinical settings.

Key words: spinal muscular atrophy, scoliosis correction surgery, perioperative period, medication therapy management

幸小东, 刘艳, 刘昕竹, 蒋文高, 张健. 脊髓性肌萎缩症患者脊柱矫形围手术期治疗药物管理[J]. 临床儿科杂志, 2025, 43(1): 70-76.

XING Xiaodong, LIU Yan, LIU Xinzhu, JIANG Wengao, ZHANG Jian. Medication therapy management of patients with spinal muscular atrophy during the perioperative period of scoliosis correction surgery[J]. Journal of Clinical Pediatrics, 2025, 43(1): 70-76.

参考文献 42

[1]	Mercuri E, Sumner CJ, Muntoni F, et al. Spinal muscular atrophy[J]. Nat Rev Dis Primers, 2022, 8(1): 52. doi: 10.1038/s41572-022-00380-8 pmid: 35927425
[2]	杨军林, 隋文渊, 张天元. 脊髓性肌萎缩症合并脊柱侧凸的临床诊治[J]. 临床儿科杂志, 2022, 40(3): 161-164.
	Yang JL, Sui WY, Zhang TY. Clinical diagnosis and treatment of spinal muscular atrophy with scoliosis[J]. Linchuang Erke Zazhi, 2022, 40(3): 161-164.
[3]	Xing X, Liu X, Li X, et al. Insights into spinal muscular atrophy from molecular biomarkers[J]. Neural Regen Res, 2025, 20(7): 1849-1863.
[4]	Mercuri E, Pera MC, Scoto M, et al. Spinal muscular atrophy - insights and challenges in the treatment era[J]. Nat Rev Neurol, 2020, 16(12): 706-715. doi: 10.1038/s41582-020-00413-4 pmid: 33057172
[5]	Ljungqvist O, Scott M, Fearon KC. Enhanced recovery after surgery: a review[J]. JAMA Surg, 2017, 152(3): 292-288. doi: 10.1001/jamasurg.2016.4952 pmid: 28097305
[6]	Fujak A, Raab W, Schuh A, et al. Natural course of scoliosis in proximal spinal muscular atrophy type II and IIIa: descriptive clinical study with retrospective data collection of 126 patients[J]. BMC Musculoskelet Disord, 2013, 14: 283.
[7]	税敏, 吴丹, 吴安石. 脊柱侧凸患者的围手术期管理[J]. 国际麻醉学与复苏杂志, 2021, 42(9): 978-982.
	Shui M, Wu D, Wu AS. Perioperative management of the patient with scoliosis in spine surgery[J]. Guoji Mazuixue Yu Fusu Zazhi, 2021, 42(9): 978-982.
[8]	Daher YH, Lonstein JE, Winter RB, et al. Spinal surgery in spinal muscular atrophy[J]. J Pediatr Orthop, 1985, 5(4): 391-395.
[9]	Hensinger RN, Macewen GD. Spinal deformity associated with heritable neurological conditions: spinal muscular atrophy, Friedreich's ataxia, familial dysautonomia, and Charcot-Marie-Tooth disease[J]. J Bone Joint Surg Am, 1976, 58(1): 13-24. pmid: 1249100
[10]	Aprin H, Bowen JR, Macewen GD, et al. Spine fusion in patients with spinal muscular atrophy[J]. J Bone Joint Surg Am, 1982, 64(8): 1179-1187. pmid: 7130230
[11]	Lovely JK, Hyland SJ, Smith AN, et al. Clinical pharmacist perspectives for optimizing pharmacotherapy within Enhanced Recovery After Surgery (ERAS(®)) programs[J]. Int J Surg, 2019, 63: 58-62. doi: S1743-9191(19)30012-3 pmid: 30665004
[12]	Mueck KM, Kao LS. Patients at High-risk for surgical site infection[J]. Surg Infect (Larchmt), 2017, 18(4): 440-446.
[13]	Islander G. Anesthesia and spinal muscle atrophy[J]. Paediatr Anaesth, 2013, 23(9): 804-816. doi: 10.1111/pan.12159 pmid: 23601145
[14]	Argoff CE. Recent management advances in acute postoperative pain[J]. Pain Pract, 2014, 14(5): 477-487. doi: 10.1111/papr.12108 pmid: 23945010
[15]	Liu X, Zhang X, Chen Y, et al. Hidden blood loss after total hip arthroplasty[J]. J Arthroplasty, 2011, 26(7): 1100-1105. doi: 10.1016/j.arth.2010.11.013 pmid: 21256705
[16]	Engel JM, Hohaus T, Ruwoldt R, et al. Regional hemostatic status and blood requirements after total knee arthroplasty with and without tranexamic acid or aprotinin[J]. Anesth Analg, 2001, 92(3): 775-780. doi: 10.1097/00000539-200103000-00041 pmid: 11226117
[17]	Huang Z, Ma J, Shen B, et al. Combination of intravenous and topical application of tranexamic acid in primary total knee arthroplasty: a prospective randomized controlled trial[J]. J Arthroplasty, 2014, 29(12): 2342-2346. doi: 10.1016/j.arth.2014.05.026 pmid: 25007725
[18]	中国康复技术转化及发展促进会, 中国研究型医院学会关节外科学专业委员会, 中国医疗保健国际交流促进会关节疾病防治分会, 等. 中国骨科手术加速康复围手术期氨甲环酸与抗凝血药应用的专家共识[J]. 中华骨与关节外科杂志, 2019, 12(2): 81-88.
	China Association of Rehabilitation Technology Transformation and Promotion, Joint Surgery Committee of Chinese Research Hospital Association, Joint Diseases Prevention and Trearment Branch of China International Exchange and Promotion Association for Medical and Healthcare, et al. Expert consensus on the application of tranexamic acid and anticoagulant for the enhanced recovery after orthopedic surgery in China[J]. Zhonghua Gu Yu Guanjie Waike Zazhi, 2019, 12(2): 81-88.
[19]	国家卫生健康委加速康复外科专家委员会骨科专家组, 中国研究型医院学会骨科加速康复专业委员会, 中国康复技术转化及促进会骨科加速康复专业委员会. 骨科大手术加速康复围手术期静脉血栓栓塞症防治专家共识[J]. 中华骨与关节外科杂志, 2022, 15(10): 754-762.
	Orthopaedic Expert Group of ERAS profession Committee of National Health Commission, Orthopaedic ERAS profession Committee of Chinese Research Hospital Association, Orthopaedic ERAS profession Committee of China Association of Rehabilitation Technology Transformation and Promotion. Expert consensus on the prevention and treatment of perioperative venous thromboembolism in major orthopaedic surgery in enhanced recovery after surgery program[J]. Zhonghua Gu Yu Guanjie Waike Zazhi, 2022, 15(10): 754-762.
[20]	Kwon S, Thompson R, Dellinger P, et al. Importance of perioperative glycemic control in general surgery: a report from the Surgical Care and Outcomes Assessment Program[J]. Ann Surg, 2013, 257(1): 8-14. doi: 10.1097/SLA.0b013e31827b6bbc pmid: 23235393
[21]	Frisch A, Chandra P, Smiley D, et al. Prevalence and clinical outcome of hyperglycemia in the perioperative period in noncardiac surgery[J]. Diabetes Care, 2010, 33(8): 1783-1788. doi: 10.2337/dc10-0304 pmid: 20435798
[22]	Dungan KM, Braithwaite SS, Preiser JC. Stress hyperglycaemia[J]. Lancet, 2009, 373(9677): 1798-1807. doi: 10.1016/S0140-6736(09)60553-5 pmid: 19465235
[23]	Abdelmalak B, Abdelmalak JB, Knittel J, et al. The prevalence of undiagnosed diabetes in non-cardiac surgery patients, an observational study[J]. Can J Anaesth, 2010, 57(12): 1058-1064. doi: 10.1007/s12630-010-9391-4 pmid: 20922587
[24]	Freedman MK, Hilibrand AS, Blood EA, et al. The impact of diabetes on the outcomes of surgical and nonsurgical treatment of patients in the spine patient outcomes research trial[J]. Spine (Phila Pa 1976), 2011, 36(4): 290-307. doi: 10.1097/BRS.0b013e3181ef9d8c pmid: 21270715
[25]	NAGATA K, NAKAMOTO H, SUMITANI M, et al. Diabetes is associated with greater leg pain and worse patient-reported outcomes at 1 year after lumbar spine surgery[J]. Sci Rep, 2021, 11(1): 8142.
[26]	广东省药学会. 围手术期血糖管理医-药专家共识[J]. 今日药学, 2018, 28(2): 73-83.
	Guangdong Pharmaceutical Association. Expert consensus on perioperative blood glucose management by medical-pharmaceutical specialists[J]. Jinri Yaoxue, 2018, 28(2): 73-83.
[27]	江华, 肖增明, 邱勇. 脊柱畸形矫形术中脊髓缺血性神经损伤的研究进展[J]. 中华外科杂志, 2016, 54(5): 397-400.
	Jiang H, Xiao ZM, Qiu Y. Ischemic neurological ingury during spinal deformity surgery: current status[J]. Zhonghua Waike Zazhi, 2016, 54(5): 397-400.
[28]	Pahys JM, Guille JT, D'andrea LP, et al. Neurologic injury in the surgical treatment of idiopathic scoliosis: guidelines for assessment and management[J]. J Am Acad Orthop Surg, 2009, 17(7): 426-434. pmid: 19571298
[29]	Graham RJ, Athiraman U, Laubach AE, et al. Anesthesia and perioperative medical management of children with spinal muscular atrophy[J]. Paediatr Anaesth, 2009, 19(11): 1054-1063. doi: 10.1111/j.1460-9592.2009.03055.x pmid: 19558636
[30]	Horn CC, Wallisch WJ, Homanics GE, et al. Pathophysiological and neurochemical mechanisms of postoperative nausea and vomiting[J]. Eur J Pharmacol, 2014, 722: 55-66. doi: 10.1016/j.ejphar.2013.10.037 pmid: 24495419
[31]	广东省药学会. 临床药师术后疼痛管理指引[J]. 今日药学, 2019, 29(4): 217-227.
	Guangdong Pharmaceutical Association. Guidelines for postoperative pain management by clinical pharmacists[J]. Jinri Yaoxue, 2019, 29(4): 217-227.
[32]	中华医学会麻醉学分会. 肾上腺糖皮质激素围手术期应用专家共识(2017版)[J]. 临床麻醉学杂志, 2017, 33(7): 712-716.
	Chinese Society of Anesthesiology. Expert consensus on the perioperative application of glucocorticoids (2017 Edition)[J]. Linchuang Mazuixue Zazhi, 2017, 33(7): 712-716.
[33]	Chafetz RS, Vogel LC, Betz RR, et al. International standards for neurological classification of spinal cord injury: training effect on accurate classification[J]. J Spinal Cord Med, 2008, 31(5): 538-542. doi: 10.1080/10790268.2008.11753649 pmid: 19086711
[34]	Xie J, Ma J, Yao H, et al. Multiple boluses of intravenous tranexamic acid to reduce hidden blood loss after primary total knee arthroplasty without tourniquet: a randomized clinical trial[J]. J Arthroplasty, 2016, 31(11): 2458-2464. doi: S0883-5403(16)30133-4 pmid: 27262419
[35]	Xu H, Zhang S, Xie J, et al. Multiple doses of perioperative dexamethasone further improve clinical outcomes after total knee arthroplasty: a prospective, randomized, controlled study[J]. J Arthroplasty, 2018, 33(11): 3448-3454. doi: S0883-5403(18)30601-6 pmid: 30033064
[36]	Jørgensen CC, Pitter FT, Kehlet H. Safety aspects of preoperative high-dose glucocorticoid in primary total knee replacement[J]. Br J Anaesth, 2017, 119(2): 267-275. doi: 10.1093/bja/aex190 pmid: 28854533
[37]	Lei Y, Huang Q, Xu B, et al. Multiple low-dose dexamethasone further improves clinical outcomes following total hip arthroplasty[J]. J Arthroplasty, 2018, 33(5): 1426-1431. doi: S0883-5403(17)31062-8 pmid: 29258763
[38]	中国研究型医院学会罕见病分会, 中国罕见病联盟, 北京罕见病诊疗与保障学会, 等. 青少年成人脊髓性肌萎缩症临床诊疗指南[J]. 罕见病研究, 2023, 2(1): 70-84.
	Rare Disease Society of Chinese Research Hospital Association, China Alliance for Rare Diseases, Beijing Society of Rare Disease Clinical Care and Accessibility, et al. Clinical Practice Guideline for Adolescent & Adult Patients with Spinal Muscular Atrophy[J]. Hanjianbing Yanjiu, 2023, 2(1): 70-84.
[39]	Yan Y, Feng Y, Jiang L, et al. Safety of risdiplam in spinal muscular atrophy patients after short-term treatment with nusinersen[J]. Muscle Nerve, 2024, 70(5): 1095-1098.
[40]	Chiriboga CA, Bruno C, Duong T, et al. JEWELFISH: 24-month results from an open-label study in non-treatment-naïve patients with SMA receiving treatment with risdiplam[J]. J Neurol, 2024, 271(8): 4871-4884.
[41]	Foppa AA, Chemello C, Vargas-Peláez CM, et al. Medication therapy management service for patients with parkinson's disease: a before-and-after study[J]. Neurol Ther, 2016, 5(1): 85-99. doi: 10.1007/s40120-016-0046-4 pmid: 27271736
[42]	Mercuri E, Finkel RS, Muntoni F, et al. Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care[J]. Neuromuscul Disord, 2018, 28(2): 103-115.