caa a22 4500 463180801 CHVBK 20180406164838.0 cr unu---uuuuu 170326e20070501xx s 000 0 eng 10.1007/s00381-006-0247-8 doi (NATIONALLICENCE)springer-10.1007/s00381-006-0247-8 A report of two cases with dolichosegmental intracranial arteries as a new feature of PHACES syndrome [Elektronische Daten] [Carlos Baccin, Timo Krings, Hortensia Álvarez, Augustin Ozanne, Pierre Lasjaunias] Background: We describe two previously unreported cases with complete or incomplete expression of PHACES syndrome, a rare congenital syndromal pediatric disorder, which is characterized by posterior cranial fossa malformations, large facial hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, abnormalities of the eye, sternal and supraabdominal raphe defects. Case reports: These two children exhibited a feature not reviewed extensively in the literature, namely, segmental elongation and dilatation of intracranial arteries associated with intracranial occlusive arterial disease, predominantly on the anterior division of the internal carotid artery (ICA) and on the P2 segment of the posterior cerebral artery. This dolichoectasia was found at the distal cervical internal carotid artery, the intradural segment of the ICA before the division, the trigeminal artery, and the posterior division of the ICA. We presume that the different forms of arterial involvement in PHACES syndrome (arterial stenoses, segmental agenesis of vessels, and the dolichoectasia described in this study) constitute a spectrum of angiogenetic dysfunctions related to an embryonic event involving several cephalic neural crest segments of the dorsal aorta. Springer-Verlag, 2006 PHACES syndrome nationallicence Hemangiomas nationallicence Arterial anomalies nationallicence Segmental arterial identity nationallicence Dolichoectasia nationallicence Cerebral arteries nationallicence Baccin Carlos Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut Krings Timo Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut Álvarez Hortensia Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut Ozanne Augustin Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut Lasjaunias Pierre Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut Child's Nervous System Springer-Verlag 23/5(2007-05-01), 559-567 0256-7040 23:5<559 2007 23 381 https://doi.org/10.1007/s00381-006-0247-8 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00381-006-0247-8 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Baccin Carlos Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut NATIONALLICENCE 700 1- Krings Timo Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut NATIONALLICENCE 700 1- Álvarez Hortensia Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut NATIONALLICENCE 700 1- Ozanne Augustin Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut NATIONALLICENCE 700 1- Lasjaunias Pierre Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, 78 rue du General-Leclerc, 94275, Le Kremlin Bicêtre, France aut NATIONALLICENCE 773 0- Child's Nervous System Springer-Verlag 23/5(2007-05-01), 559-567 0256-7040 23:5<559 2007 23 381 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer