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Child's Nervous System

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01-05-2007 | Case Report

A report of two cases with dolichosegmental intracranial arteries as a new feature of PHACES syndrome

Authors: Carlos E. Baccin, Timo Krings, Hortensia Álvarez, Augustin Ozanne, Pierre L. Lasjaunias

Published in: Child's Nervous System | Issue 5/2007

Abstract

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.

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Title: A report of two cases with dolichosegmental intracranial arteries as a new feature of PHACES syndrome
Authors: Carlos E. Baccin
Timo Krings
Hortensia Álvarez
Augustin Ozanne
Pierre L. Lasjaunias
Publication date: 01-05-2007
Publisher: Springer-Verlag
Published in: Child's Nervous System / Issue 5/2007
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-006-0247-8

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A report of two cases with dolichosegmental intracranial arteries as a new feature of PHACES syndrome

Abstract

Background

Case reports

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case reports

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