Purpose

In past pediatric and adult cohort studies of moyamoya disease, the fetal posterior cerebral artery has received less attention. Its relationship with the clinical manifestations and collateral circulation of moyamoya disease or ipsilateral cerebral hemispheres remains unclear.

Method

We summarize the clinical features of patients with and without fetal posterior cerebral artery moyamoya disease from consecutive cases.We explored the relationship between fetal posterior cerebral arteries and collateral circulation in the ipsilateral cerebral hemispheres, as well as differences among different subgroups of patients.According to the morphology, the fetal posterior cerebral artery is divided into complete fetal posterior cerebral artery and partial fetal posterior cerebral artery. Clinical features were classified as: infarction,hemorrhage,and non-stroke in unilateral/bilateral cerebral hemispheres. Collateral circulation is divided into extracranial vascular compensation and leptomeningeal collateral circulation. Digital subtraction angiography and CT/MR were used to evaluate the blood flow status and clinical characteristics of patients with moyamoya disease.

Result

A total of 960 cerebral hemispheres from 142 pediatric patients and 338 adult patients were included in the study. A total of 273 (56.9%) patients had 399 cerebral hemispheres (41.6%) with fetal posterior cerebral arteries. Adults with fetal posterior cerebral arteries had lower rates of infarction (24.6%vs37.3%, P =0.005) and were less likely to have bilateral stroke (8.4%vs11.5%, P =0.038). Cerebral hemispheres with fetal posterior cerebral artery were more likely to have anterior cerebral artery and middle cerebral artery stenosis and less likely to have occlusion (P =0.002, 0.001), and less likely to involve the posterior circulation (P < 0.001). The cerebral hemispheres of the fetal posterior cerebral artery had higher leptomeningeal collateral circulation scores. There are significant differences in extracranial vascular compensation between cerebral hemispheres with and without fetal posterior cerebral artery. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage (P =0.017).

Conclusions

Our results suggest that fetal posterior cerebral artery is associated with infarct manifestations in pediatric and adult moyamoya disease. In the cerebral hemispheres, the fetal posterior cerebral artery is associated with ipsilateral hemispheric anterior and posterior circulation artery injury, extracranial vascular compensation, leptomeningeal collateral circulation compensation, and infarction. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage.

中文翻译：

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儿科和成人烟雾病中的胎儿大脑后动脉：480 例患者的单中心经验

目的

在过去的烟雾病儿科和成人队列研究中，胎儿​​大脑后动脉受到的关注较少。其与烟雾病或同侧大脑半球的临床表现和侧支循环的关系尚不清楚。

方法

我们从连续病例中总结了胎儿大脑后动脉烟雾病患者的临床特征。我们探讨了胎儿大脑后动脉与同侧大脑半球侧支循环的关系，以及不同亚组患者之间的差异。根据从形态上看，胎儿大脑后动脉分为完全胎儿大脑后动脉和部分胎儿大脑后动脉。临床特征分类为：单侧/双侧大脑半球梗死、出血和非卒中。侧支循环分为颅外血管代偿和软脑膜侧支循环。

结果

该研究共纳入了来自 142 名儿童患者和 338 名成人患者的 960 个大脑半球。共有273名（56.9%）患者的399个大脑半球（41.6%）有胎儿大脑后动脉。有胎儿大脑后动脉的成人梗死率较低（24.6% vs 37.3%，P = 0.005），双侧卒中发生率较低（8.4% vs 11.5%，P = 0.038）。有胎儿大脑后动脉的大脑半球更容易有大脑前动脉和大脑中动脉狭窄且闭塞的可能性较小（P = 0.002，0.001），并且不太可能累及后循环（P <0.001）。胎儿大脑后动脉的大脑半球有较高的软脑膜侧支循环评分。有和没有胎儿大脑后动脉的大脑半球颅外血管代偿存在显着差异。胎儿大脑后动脉成年患者Suzuki分期更晚（P=0.017）。

结论

我们的研究结果表明，胎儿大脑后动脉与儿童和成人烟雾病的梗塞表现有关。在大脑半球，胎儿大脑后动脉与同侧半球前后循环动脉损伤、颅外血管代偿、软脑膜侧支循环代偿和梗死有关。胎儿大脑后动脉成年患者在Suzuki分期时更晚期。