摘要
脊髓海绵状血管瘤(spinal cavernous hemangioma,SCH)是海绵状血管瘤(cavernous hemangioma,CH)的少见类型。目前文献数据显示SCH多发于胸段,但不同脊柱节段发病率的差异尚缺乏合理解释。目前研究表明SCH的发生可能与TEK、KRIT1、MGC4607和PDCD10等基因的突变有关,也可由放射、外伤、感染等外界因素诱发,但放射剂量和(或)放射类型对SCH的发病率及严重程度的具体影响有待深入研究。SCH起病隐匿,可引起严重的运动、感觉障碍,症状大多缓慢进展或逐渐消失,但也有急性发病或突然加重者。近年的研究提示较常见的MRI特征是"火焰状"或"线状"的髓内出血,而非既往研究指出的"爆米花"或"桑葚样"状混杂信号。目前普遍认为症状较重或进行性加重、病灶体积大和(或)出血风险高,或复查显示病灶体积增长快是SCH的手术指征。在症状发生3个月内进行手术疗效更佳,手术治疗目前多经后正中入路行椎板切除或椎板成形术尽可能彻底切除病灶,但较深病变的手术仍存在较高难度及风险。因此,临床上不断在手术入路和手术方式上进行探索和改进,如应用脊髓后根入髓区前方或背外侧入路、应用骨科手术机器人系统等。术中监测技术如神经电生理监测、术中超声等技术已广泛应用,但预警标准尚未统一。普萘洛尔、西罗莫司等少数药物已应用于临床治疗,但疗效尚需多中心研究验证。在病程管理方面,既往有脊髓病变、蛛网膜下腔出血病史或较大病灶的患者预后相对更差,但高血压、糖尿病等基础疾病对出血风险的影响尚不明确。SCH患者再出血的风险随时间累积明显增加,但不同病情和病程患者的保守治疗方案有待细化。
Spinal cavernous hemangiomas(SCH)represent a rare subtype of cavernous hemangiomas(CH).Existing literature predominantly indicates a higher prevalence of SCH in the thoracic region,although a comprehensive explanation for segmental distribution variations remains elusive.Current investigations suggest that the etiology of SCH may be linked to mutations in genes such as TEK,KRIT1,MGC4607,and PDCD10,and can also be precipitated by external factors including radiation,trauma,and infection.However,the specific impact of radiation dosage and modality on the incidence and severity of SCH warrants further in-depth study.The onset of this disease is concealed,but it can cause serious motor and sensory disorders.The symptoms mostly progress slowly or disappear gradually,but there are also cases of acute onset or sudden exacerbation.Recent research emphasizes the more prevalent magnetic resonance imaging(MRI)features of"flame-like"or"linear"intramedullary hemorrhages over the formerly identified"popcorn"or"mulberry-like"heterogeneous signals.Surgical intervention is generally recommended for patients with severe or progressively worsening symptoms,large lesion volumes,high risk of hemorrhage,or those with rapid increase in lesion size upon follow-up.Optimal outcomes are achieved when surgery is performed within three months of symptom onset.Surgical strategies typically involve laminectomy or laminoplasty via a posterior approach to achieve maximal lesion resection,although deep-seated lesions still pose considerable surgical challenges and risks.Ongoing clinical innovations are focusing on optimizing surgical approaches and techniques,such as employing anterior or dorsolateral approaches into the spinal cord via dorsal root entry zones and utilizing orthopedic robotic systems.Intraoperative monitoring technologies like neurophysiological monitoring and intraoperative ultrasound have been widely implemented,yet uniform warning criteria are yet to be established.A limited array of pharmacotherapies,including propranolol and
作者
卞瀚锋
唐福宇
Bian Hanfeng;Tang Fuyu(Guangxi University of Chinese Medicine,Nanning 530200,China;Liuzhou Traditional Chinese Medicine Hospital(Liuzhoi Zhuang Medical Hospital),Liuzhou 545001,China)
出处
《中华骨科杂志》
CAS
CSCD
北大核心
2023年第18期1248-1256,共9页
Chinese Journal of Orthopaedics
基金
广西自然科学基金项目(2020GXNSFAA259082)
广西中医药适宜技术开发与推广项目(GZSY21-65)。
关键词
脊髓
血管瘤
海绵状
中枢神经系统
诊断
外科手术
药物疗法
出血
预后
Spinal cord
Hemangioma,cavernous,central nervous system
Diagnosis
Surgical procedures,oper Drug therapy
Hemorrhage
Prognosis
