Persistent stapedial artery in PHACE syndrome.


Clinique universitaire d'oto-rhino-laryngologie et de chirurgie cervico-faciale, pôle PALCROS, CHU de Grenoble, hôpital Nord, Grenoble cedex 9 CS 10217, France; Pôle médecine, université Grenoble Alpes, domaine de la Merci, La Tronche 38700, France; Service d'oto-rhino-laryngologie et de chirurgie cervico-faciale, centre hospitalier Métropole Savoie, Chambéry 73000, France. Electronic address: [Email]


BACKGROUND : PHACE syndrome is characterized by posterior fossa malformations, haemangioma, arterial anomalies, coarctation of the aorta, and eye abnormalities.
METHODS : We present the case of a 6-year-old girl followed since birth for PHACE syndrome and left hemifacial haemangioma, who presented with left hearing loss. Computed tomography scan showed left persistent stapedial artery (PSA).
CONCLUSIONS : Two types of arterial anomalies may be observed in PHACE syndrome: persistence of embryonic arteries and anomalies of cerebral arteries. PSA can be observed in the context of PHACE syndrome. Children with PHACE syndrome require regular audiometric follow-up to detect hearing loss and avoid its consequences on speech and language development.


Haemangioma,PHACE syndrome,Persistent stapedial artery,Unilateral hearing loss,

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