Drosou Anna, Benjamin Latanya, Linfante Italo, Mallin Karen, Trowers Andrea, Wakhloo Ajay K, Thaller Seth R, Schachner Lawrence A
Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, Florida 33131, USA.
J Am Acad Dermatol. 2006 Feb;54(2):348-52. doi: 10.1016/j.jaad.2005.05.037.
In the majority of cases, infantile hemangiomas are not associated with any other abnormalities. Occasionally, they may indicate the presence of systemic malformations. PHACE syndrome includes the coexistence of hemangioma, posterior fossa brain abnormalities, arterial anomalies, coarctation of the aorta, cardiac defects, and eye abnormalities. We report a case of a 2-month-old female with PHACE syndrome who also had sinus pericranii.
A 2-month-old girl was seen for a plaque-like, segmental, midfacial hemangioma, with recurrent hemorrhages, noted at birth. As part of the PHACE syndrome, she had a midline facial hemangioma, absent corpus callosum, hypoplastic internal carotid artery, and an abnormal tortuous dysplastic basilar artery. Digital subtraction angiography showed sinus pericranii. The patient underwent successful endovascular embolization of the hemangioma that prevented further bleeding.
Sinus pericranii is a rare finding that has not, to our knowledge, been previously associated with PHACE syndrome. Central nervous system, not only posterior fossa, abnormalities are frequently encountered in PHACE syndrome. Endovascular embolization of the facial hemangioma in our patient was a useful therapy.
在大多数情况下，婴儿血管瘤与任何其他异常无关。偶尔，它们可能提示存在全身畸形。PHACE综合征包括血管瘤、后颅窝脑异常、动脉异常、主动脉缩窄、心脏缺陷和眼部异常同时存在。我们报告一例患有PHACE综合征且伴有头皮静脉窦的2个月大女性病例。
一名2个月大的女孩因出生时即发现的斑块状、节段性、面中部血管瘤伴反复出血前来就诊。作为PHACE综合征的一部分，她患有面中部中线血管瘤、胼胝体缺失、颈内动脉发育不全以及异常迂曲发育不良的基底动脉。数字减影血管造影显示有头皮静脉窦。患者接受了血管瘤的成功血管内栓塞治疗，防止了进一步出血。
头皮静脉窦是一种罕见的发现，据我们所知，此前尚未与PHACE综合征相关联。PHACE综合征中经常会遇到中枢神经系统异常，不仅是后颅窝异常。我们患者面部血管瘤的血管内栓塞是一种有效的治疗方法。
