Cervical and Intracranial Arterial Anomalies in 70 Patients with PHACE Syndrome
Cervical and Intracranial Arterial Anomalies in 70 Patients with PHACE Syndrome
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DOI：
10.3174/ajnr.a2206
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发表时间：
2010-11-01
影响因子：
3.5
通讯作者：
Barkovich, A. J.
中科院分区：
文献类型：
--
作者：
Hess, C. P.;Fullerton, H. J.;Barkovich, A. J.
背景和目的：脑和颈动脉异常是PHACE综合征中最常见的非皮肤异常，但尚未在大型队列中系统评估发生的动脉病变的位置和类型。我们的目的是描述动脉病的表型谱，评估不同动脉受累的频率，并评估PHACE综合征中动脉病、脑结构病变和血管瘤之间的空间关系。材料和方法：颅内MRA和/或对70例儿童动脉病变和PHACE综合征患者的CTA或CTA图像和59例患者的头颅MR图像进行回顾性分析，动脉病变和脑部异常的位置。确定了五类动脉病并用于分类：发育不全、狭窄、不可见、原始胚胎颈动脉-椎基底动脉连接和异常动脉路线或起源。单变量逻辑回归分析进行测试之间的关联动脉病变的位置，血管瘤，脑abnormal.RESULTS：通过研究设计，所有患者动脉异常，57%有> 1种形式的动脉病变。发育不良是最常见的异常（56%），其次是异常的路线和/或起源（47%），狭窄（39%）和不可见（20%）。20%的儿童存在原始的胚胎颈动脉-椎基底动脉连接。血管瘤除1例外均与动脉病变同侧。97%的病例累及额颞和/或下颌面部段，但未检测到动脉病变位置与血管瘤部位之间的其他特定相关性。所有病例与后颅窝异常ICA异常或持续胚胎颈动脉基底动脉connecting.CONCLUSIONS：动脉病变PHACE综合征通常涉及ICA及其胚胎分支，同侧的皮肤血管瘤，发育不良和异常的动脉过程中最常见的异常。大脑异常通常也是同侧的。
BACKGROUND AND PURPOSE: Cerebral and cervical arterial abnormalities are the most common non-cutaneous anomaly in PHACE syndrome, but the location and type of arterial lesions that occur have not been systematically assessed in a large cohort. Our aim was to characterize the phenotypic spectrum of arteriopathy, assess the frequency with which different arteries are involved, and evaluate spatial relationships between arteriopathy, brain structural lesions, and hemangiomas in PHACE syndrome.MATERIALS AND METHODS: Intracranial MRA and/or CTA images from 70 children and accompanying brain MR images in 59 patients with arteriopathy and PHACE syndrome were reviewed to identify the type and location of arterial lesions and brain abnormalities. Five categories of arteriopathy were identified and used for classification: dysgenesis, narrowing, nonvisualization, primitive embryonic carotid-vertebrobasilar connections, and anomalous arterial course or origin. Univariate logistic regression analyses were performed to test for associations between arteriopathy location, hemangiomas, and brain abnormalities.RESULTS: By study design, all patients had arterial abnormalities, and 57% had >1 form of arteriopathy. Dysgenesis was the most common abnormality (56%), followed by anomalous course and/or origin (47%), narrowing (39%), and nonvisualization (20%). Primitive embryonic carotid-vertebrobasilar connections were present in 20% of children. Hemangiomas were ipsilateral to arteriopathy in all but 1 case. The frontotemporal and/or mandibular facial segments were involved in 97% of cases, but no other specific associations between arteriopathy location and hemangioma sites were detected. All cases with posterior fossa anomalies had either ICA anomalies or persistent embryonic carotid-basilar connections.CONCLUSIONS: The arteriopathy of PHACE syndrome commonly involves the ICA and its embryonic branches, ipsilateral to the cutaneous hemangioma, with dysgenesis and abnormal arterial course the most commonly noted abnormalities. Brain abnormalities are also typically ipsilateral.