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.
Service d'oto-rhino-laryngologie et de chirurgie cervico-faciale, centre hospitalier Métropole Savoie, Chambéry 73000, France.
Eur Ann Otorhinolaryngol Head Neck Dis. 2019 Jun;136(3):215-217. doi: 10.1016/j.anorl.2019.02.015. Epub 2019 Mar 12.
PHACE syndrome is characterized by posterior fossa malformations, haemangioma, arterial anomalies, coarctation of the aorta, and eye abnormalities.
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).
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.
PHACE 综合征的特征为后颅窝畸形、血管瘤、动脉异常、主动脉缩窄和眼部异常。
我们报告了一例 6 岁女孩的病例,她自出生以来一直患有 PHACE 综合征和左侧面部血管瘤,并伴有左侧听力损失。计算机断层扫描显示左侧永存镫骨动脉(PSA)。
PHACE 综合征中可观察到两种类型的动脉异常:胚胎动脉的持续存在和脑动脉的异常。PSA 可在 PHACE 综合征中观察到。PHACE 综合征患儿需要定期进行听力随访,以发现听力损失并避免其对言语和语言发育的影响。