M D Mamlouk1,2, A Vossough3, L Caschera3,4, M Maheshwari5, C P Hess2. 1. From the Department of Radiology (M.D.M.), The Permanente Medical Group, Kaiser Permanente Medical Center, Santa Clara, Santa Clara, California mark.d.mamlouk@kp.org mark.mamlouk@ucsf.edu. 2. Department of Radiology and Biomedical Imaging (M.D.M., C.P.H.), University of California, San Francisco, San Francisco, California. 3. Department of Radiology (A.V., L.C.), Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania. 4. Department of Radiology (L.C.), La Fondazione Institute for Research, Hospitalization and Health Care Ca' Granda Ospedale Maggiore di Milano Policlinico, Milan, Italy. 5. Department of Radiology (M.M.), Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee, Wisconsin.
Abstract
BACKGROUND AND PURPOSE: Arterial stroke is a rare-but-reported complication in patients with posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities (PHACE) syndrome. Currently, stroke risk is inferred by the severity of arterial anomalies identified on MRA, though no evidenced-based data exist. The purpose of our study was to determine whether arterial spin-labeling MR imaging perfusion can detect alterations in CBF in patients with PHACE syndrome. MATERIALS AND METHODS: Records were reviewed from 3 institutions for all patients with PHACE syndrome who underwent arterial spin-labeling from 2000 to 2019. CBF was qualitatively investigated with arterial spin-labeling to determine whether there was decreased or normal perfusion. Arterial anomalies were characterized on MRA imaging, and parenchymal brain findings were evaluated on conventional MR imaging sequences. RESULTS: Forty-one patients with PHACE syndrome had arterial spin-labeling imaging. There were 30 females and 11 males (age range, 7 days to 15 years). Of the 41 patients, 10 (24%) had decreased CBF signal corresponding to a major arterial territory. Ten of 10 patients had decreased CBF signal in the anterior circulation, 2/10 had decreased anterior and posterior circulation CBF signal, 2/10 had decreased bilateral anterior circulation CBF signal, and 1/10 had globally decreased CBF signal. Forty of 41 (97.5%) patients had at least 1 arteriopathy, and in those with decreased CBF signal, the arteriopathy corresponded to the CBF signal alteration in 10/10 patients. CONCLUSIONS: Arterial spin-labeling can potentially characterize hemodynamic changes in patients with PHACE syndrome.
BACKGROUND AND PURPOSE: Arterial stroke is a rare-but-reported complication in patients with posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities (PHACE) syndrome. Currently, stroke risk is inferred by the severity of arterial anomalies identified on MRA, though no evidenced-based data exist. The purpose of our study was to determine whether arterial spin-labeling MR imaging perfusion can detect alterations in CBF in patients with PHACE syndrome. MATERIALS AND METHODS: Records were reviewed from 3 institutions for all patients with PHACE syndrome who underwent arterial spin-labeling from 2000 to 2019. CBF was qualitatively investigated with arterial spin-labeling to determine whether there was decreased or normal perfusion. Arterial anomalies were characterized on MRA imaging, and parenchymal brain findings were evaluated on conventional MR imaging sequences. RESULTS: Forty-one patients with PHACE syndrome had arterial spin-labeling imaging. There were 30 females and 11 males (age range, 7 days to 15 years). Of the 41 patients, 10 (24%) had decreased CBF signal corresponding to a major arterial territory. Ten of 10 patients had decreased CBF signal in the anterior circulation, 2/10 had decreased anterior and posterior circulation CBF signal, 2/10 had decreased bilateral anterior circulation CBF signal, and 1/10 had globally decreased CBF signal. Forty of 41 (97.5%) patients had at least 1 arteriopathy, and in those with decreased CBF signal, the arteriopathy corresponded to the CBF signal alteration in 10/10 patients. CONCLUSIONS: Arterial spin-labeling can potentially characterize hemodynamic changes in patients with PHACE syndrome.
Authors: Denise W Metry; Maria C Garzon; Beth A Drolet; Peter Frommelt; Anita Haggstrom; Judith Hall; Christopher P Hess; Geoffrey L Heyer; Dawn Siegel; Eulalia Baselga; William Katowitz; Moise L Levy; Anthony Mancini; Mandi L Maronn; Thuy Phung; Elena Pope; Grace Sun; Ilona J Frieden Journal: Pediatr Dermatol Date: 2009 Jul-Aug Impact factor: 1.588
Authors: G Boulouis; V Dangouloff-Ros; O Boccara; N Garabedian; V Soupre; A Picard; V Couloigner; N Boddaert; O Naggara; F Brunelle Journal: AJNR Am J Neuroradiol Date: 2017-01-19 Impact factor: 3.825