Article Figures & Data
Figures
- Fig 1.
Coronal fluid-sensitive MR images through the bilateral Meckel caves of all patients with asymmetric Meckel cave enlargement. The vascular structure in the enlarged Meckel cave in patients 6, 9, 15, and 22 represents an ipsilateral aberrant ophthalmic artery arising from the basilar artery. The vascular structure in the enlarged right Meckel cave in patient 23 (Fig 2) represents an ectatic persistent trigeminal artery supplying the distal internal carotid artery.
- Fig 2.
A persistent right trigeminal artery in a 1-year-old girl (patient 23) with PHACES syndrome. A, Axial maximum-intensity-projection 3D time-of-flight image demonstrates a persistent right trigeminal artery (white arrows). Note that the contralateral left cavernous segment internal carotid artery is tortuous. B, Volume-rendered 3D time-of-flight reconstruction demonstrates the persistent trigeminal artery (white arrows) connecting the right cavernous segment of the ICA to the tortuous and ectatic basilar artery. Note that the distal right ICA is aplastic proximal to the cavernous segment and is reconstituted via collateral vessels arising from external carotid artery branches.
Tables
Cohort summary data and statistics
All PHACES Definite PHACES Possible PHACES Not PHACES No. (% total) 25 (29) 23 (27) 2 (2) 60 (71) Female (No.) (% category) 21 (84) 19 (82) 2 (100) 50 (83) Age (mean ± SD) 20 ± 39 mo 22 ± 40 mo 1 ± 0.5 mo 10 ± 17 mo Age (range) (median) 5 days to 14 yr (4 mo) 5 days to 14 yr (5 mo) 1–2 mo (1.5 mo) 1 mo to 7 yr (4 mo) Meckel cave enlargement (No.) (% category) 19 (76) 19 (82) 0 (0) 0 (0) Posterior fossa anomalies (No.) (% category) 15 (60) 15 (65) 0 (0) 0 (0) Facial hemangioma (No.) (% category) 24 (96) 23 (100) 1 (50) 60 (100) Arterial anomalies (No.) (% category) 21 (84) 20 (90) 1 (50) 0 (0) Cardiac anomalies (No.) (% category) 13 (52) 12 (52) 1 (50) 0 (0) Eye anomalies (No.) (% category) 0 (0) 0 (0) 0 (0) 0 (0) Sternal or midline abdominal anomalies (No.) (% category) 1 (4) 0 (0) 1 (50) 0 (0)
