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Research Progress of Moyamoya Disease in Children

机译:儿童烟雾病的研究进展

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摘要

During the onset of Moyamoya disease (MMD), progressive occlusion occurs at the end of the intracranial internal carotid artery, and compensatory net-like abnormal vessels develop in the skull base, generating the corresponding clinical symptoms. MMD can affect both children and adults, but MMD in pediatric patients exhibits distinct clinical features, and the treatment prognoses are different from adult patients. Children are the group at highest risk for MMD. In children, the disease mainly manifests as ischemia, while bleeding is the primary symptom in adults. The pathogenesis of MMD in children is still unknown, and some factors are distinct from those in adults. MMD in children could result in progressive, irreversible nerve functional impairment, and an earlier the onset corresponds to a worse prognosis. Therefore, active treatment at an early stage is highly recommended. The treatment methods for MMD in children mainly include indirect and direct surgeries. Indirect surgeries mainly include multiple burr-hole surgery (MBHS), encephalomyosynangiosis (EMS), and encephaloduroarteriosynangiosis (EDAS); direct surgeries mainly include intra- and extracranial vascular reconstructions that primarily consist of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis. Indirect surgery, as a treatment for MMD in children, has shown a certain level of efficacy. However, a standard treatment approach should combine both indirect and direct procedures. Compared to MMD in adults, the treatment and prognosis of MMD in children has higher clinical significance. If the treatment is adequate, a satisfactory outcome is often achieved.
机译:在Moyamoya病(MMD)发作期间,进行性闭塞发生在颅内颈内动脉的末端,并且在颅底中形成了代偿网状异常血管,从而产生了相应的临床症状。 MMD可影响儿童和成人,但是小儿患者的MMD具有明显的临床特征,并且治疗预后与成人患者不同。儿童是MMD风险最高的人群。在儿童中,该疾病主要表现为局部缺血,而出血是成人的主要症状。儿童MMD的发病机制仍然未知,某些因素与成年人不同。儿童MMD可能导致进行性,不可逆转的神经功能障碍,而且起病越早,预后就越差。因此,强烈建议在早期进行积极治疗。儿童MMD的治疗方法主要包括间接和直接手术。间接手术主要包括多孔钻孔手术(MBHS),脑肌管血管病(EMS)和脑十二指肠动脉硬化症(EDAS)。直接手术主要包括颅内和颅外血管重建,主要由颞浅动脉-大脑中动脉(STA-MCA)吻合组成。间接手术作为儿童MMD的治疗方法已显示出一定的疗效。但是,标准的治疗方法应将间接和直接程序结合起来。与成人MMD相比,儿童MMD的治疗和预后具有更高的临床意义。如果治疗足够,通常会获得令人满意的结果。

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