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。
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.
作者
仪晓立
高莹
袁新宇
YI Xiaoli;GAO Ying;YUAN Xinyu(Department of Radiology,Children Hospital,Capital Institute of Pediatrics,Beijing 100020,China;Department of Dermatology,Children Hospital,Capital Institute of Pediatrics,Beijing 100020,China)
出处
《中国医学影像技术》
CSCD
北大核心
2020年第7期1002-1006,共5页
Chinese Journal of Medical Imaging Technology
基金
首都儿科研究所培育计划基金(PY-2018-10)。
