Intracranial imaging manifestations of PHACES syndrome
10.13929/j.issn.1003-3289.2020.07.012
- VernacularTitle: PHACES综合征颅内影像学表现
- Author:
Xiaoli YI
1
Author Information
1. Department of Radiology, Children Hospital, Capital Institute of Pediatrics
- Publication Type:Journal Article
- Keywords:
Infant;
Magnetic resonance imaging;
PHACES syndrome;
Tomography, X-ray computed
- From:
Chinese Journal of Medical Imaging Technology
2020;36(7):1002-1006
- CountryChina
- Language:Chinese
-
Abstract:
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.
