Pascual-Castroviejo type II syndrome (P-CIIS). Importance of the presence of persistent embryonic arteries.
BACKGROUND
Cutaneous hemangioma and vascular malformation are two vascular abnormalities frequently associated with absence or hypoplasia of one or both carotid and/or vertebral arteries, presence of persistent embryonic arteries, especially the trigeminal, cerebellar malformations, and coarctation of the aortic arch and/or congenital cardiopathy. This disease is known as Pascual-Castroviejo type II syndrome (P-CIIS) and by the acronym PHACE.


METHODS
Three patients (two females and one male) with facial hemangioma are studied during the first years of age by magnetic resonance angiography (MRA) and their vascular evolution to adult age followed through several MRA controls.


RESULTS
All the three patients showed persistence of the trigeminal artery associated to other intra- and extracranial vascular abnormalities of type hemangioma or hemangiomatous arteries that presented progressive involution with decreased arterial caliber without appearing cerebrovascular stroke or hypoxic zones because, at the same time, collateral vascularization appeared through connections between the embryonic arteries and the peripheral branches of the internal carotids or connections between branches of the external and internal carotids. Only one patient had obstruction of a branch of the left middle cerebral artery after 3 days, with gastroenteritis with elevated fever at 17 months of life that caused parenchymal infarct in the left cerebral region supplied by the obstructed artery.


CONCLUSIONS
The presence of embryonic arteries, especially the trigeminal, and connections between branches of the internal and external carotids, mainly through the internal maxillary and ophthalmic arteries, ensure the cerebral supply in the P-CIIS despite the progressive involvement of the cerebral arteries.
Pascual-Castroviejo
Ignacio
I
Pediatric Neurology Service, University Hospital La Paz, Madrid, Spain. i.pcastroviejo@neurologia.e.telefonica.net
Alvarez-Linera
Juan
J
Coya
Juan
J
Viaño
Juan
J
Pascual-Pascual
Samuel-Ignacio
SI
Velázquez-Fragua
Ramón
R
López-Gutiérrez
Juan-Carlos
JC
D000293:Adolescent; D000328:Adult; D001158:Arteries / Q000002:abnormalities*; D001921:Brain / Q000098:blood supply* / Q000000981:diagnostic imaging; D001932:Brain Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006391:Hemangioma / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology; D018810:Magnetic Resonance Angiography; D008297:Male; D011859:Radiography; D055815:Young Adult