| 仪晓立,高莹,袁新宇.PHACES综合征颅内影像学表现[J].中国医学影像技术,2020,36(7):1002~1006 |
| PHACES综合征颅内影像学表现 |
| Intracranial imaging manifestations of PHACES syndrome |
| 投稿时间：2019-06-17 修订日期：2020-01-30 |
| DOI：10.13929/j.issn.1003-3289.2020.07.012 |
| 中文关键词: PHACES综合征 磁共振成像 体层摄影术,X线计算机 婴儿 |
| 英文关键词:PHACES syndrome magnetic resonance imaging tomography, X-ray computed infant |
| 基金项目:首都儿科研究所培育计划基金（PY-2018-10）。 |
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| 中文摘要: |
| 目的 分析PHACES综合征颅内影像学表现。方法 回顾性分析9例符合PHACES综合征诊断标准患儿的影像学资料，包括4例颅脑MRI、3例增强CT和2例增强CT及MRI，观察有无颅内大血管、脑结构异常及其他伴随异常。结果 6例存在颅内大血管异常，包括2例单侧颈内动脉发育不良，1例单侧颈内动脉发育不良伴同侧大脑中动脉狭窄，1例单侧颈内动脉缺如伴大脑后动脉起始部囊性动脉瘤，1例寰前节间动脉，1例原始眼动脉。8例存在颅后窝脑结构异常，其中6例单侧小脑发育不良，2例Dandy-Walker畸形；6例合并颅内血管瘤，位于桥小脑角区，与皮下血管瘤不相连。6例伴发眼眶内血管瘤，其中5例同侧存在颅内血管瘤，但不与之相连。3例存在幕上脑积水。结论 单侧小脑发育不良和颈内动脉异常是PHACES综合征最常见颅内表现；颅内血管瘤在PHACES综合征患儿中并不少见。 |
| 英文摘要: |
| Objective To observe intracranial imaging manifestations of PHACES syndrome. Methods Imaging data of 9 children with PHACES syndrome who met the diagnostic criteria were retrospectively analyzed, including craniocerebral MRI of 4 cases, enhanced CT of 3 cases as well as enhanced CT and MRI of 2 cases, and whether there were abnormal intracranial vessels, brain structures and other accompanying abnormalities were observed. Results Six cases were found with intracranial vascular abnormalities, including 2 cases of unilateral internal carotid artery dysplasia, 1 case of unilateral internal carotid artery dysplasia with ipsilateral middle cerebral artery stenosis, 1 case of unilateral internal carotid artery absence with cystic aneurysm in the origin of posterior cerebral artery, 1 case of proatlantal intersegmental artery and 1 case of primitive ophthalmic artery. Abnormal brain structures in the posterior cranial fossa were detected in 8 cases, including 6 cases of unilateral cerebellar dysplasia and 2 cases of Dandy-Walker malformation, while intracranial hemangioma located in the cerebellopontine angle but not associated with subcutaneous hemangioma were noticed in 6 cases. There were 6 cases of intraorbital hemangioma, 5 of which were accompanied by ipsilateral intracranial hemangioma but not connected. Supratentorial hydrocephalus was found in 3 cases. Conclusion Unilateral cerebellar dysplasia and abnormal internal carotid artery are the most common intracranial manifestations of PHACES syndrome. Intracranial hemangioma is not uncommon among children with PHACES syndrome. |
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