Abstract:
:PHACE and PHACES are acronyms for a syndrome of variable expression comprising posterior cranial fossa malformations, facial haemangiomas, arterial anomalies, aortic coarctation and other cardiac disorders, ocular abnormalities and stenotic arterial disease. We review five girls and three boys aged 1 month-14 years with disorders from this spectrum. Six had large facial haemangiomas but recent reports suggest that small haemangiomas may occur; hence our inclusion of two possible cases. We also focus on the recently recognised feature of progressive intracranial arterial occlusions, present in four of our patients, later than previously recognised, from 4 to 14 years of age. We suggest that many elements of this disorder could reflect an abnormality of cell proliferation and apoptosis.
journal_name
Neuroradiologyjournal_title
Neuroradiologyauthors
Bhattacharya JJ,Luo CB,Alvarez H,Rodesch G,Pongpech S,Lasjaunias PLdoi
10.1007/s00234-002-0902-zsubject
Has Abstractpub_date
2004-03-01 00:00:00pages
227-33issue
3eissn
0028-3940issn
1432-1920journal_volume
46pub_type
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