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小儿脑海绵状血管畸形的临床特征与治疗

Clinical Features and Treatment of Pediatric Cerebral Cavernous Malformations.

作者信息

Phi Ji Hoon, Kim Seung-Ki

机构信息

Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.

出版信息

J Korean Neurosurg Soc. 2024 May;67(3):299-307. doi: 10.3340/jkns.2024.0047. Epub 2024 Mar 28.

Abstract

Cerebral cavernous malformation (CCM) is a vascular anomaly commonly found in children and young adults. Common clinical presentations of pediatric patients with CCMs include headache, focal neurological deficits, and seizures. Approximately 40% of pediatric patients are asymptomatic. Understanding the natural history of CCM is crucial and hemorrhagic rates are higher in patients with an initial hemorrhagic presentation, whereas it is low in asymptomatic patients. There is a phenomenon known as temporal clustering in which a higher frequency of symptomatic hemorrhages occurs within a few years following the initial hemorrhagic event. Surgical resection remains the mainstay of treatment for pediatric CCMs. Excision of a hemosiderin-laden rim is controversial regarding its impact on epilepsy outcomes. Stereotactic radiosurgery is an alternative treatment, especially for deepseated CCMs, but its true efficacy needs to be verified in a clinical trial.

摘要

脑海绵状血管畸形(CCM)是一种常见于儿童和年轻人的血管异常。患有CCM的儿科患者常见的临床表现包括头痛、局灶性神经功能缺损和癫痫发作。约40%的儿科患者无症状。了解CCM的自然病史至关重要,初始有出血表现的患者出血率较高,而无症状患者的出血率较低。有一种现象称为时间聚集,即在初始出血事件后的几年内,有症状出血的频率较高。手术切除仍然是儿科CCM治疗的主要方法。关于含铁血黄素边缘切除对癫痫结局的影响存在争议。立体定向放射外科是一种替代治疗方法,特别是对于深部CCM,但需要在临床试验中验证其真正疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1527/11079565/79e656f6b221/jkns-2024-0047f1.jpg

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