Recio-Rodríguez M, Martín Fernández-Mayoralas D, Jiménez-de-la-Peña M, Fernández-Jaén A
Servicio de Radiodiagnóstico, Hospital Universitario Quirón, Madrid, España.
Radiologia. 2013 Nov-Dec;55(6):537-40. doi: 10.1016/j.rx.2010.08.014. Epub 2011 Jul 5.
The neurocutaneous syndrome known by the acronym PHACE consists of the association of a segmental facial hemangioma with, among other entities, posterior fossa anomalies, cerebrovascular anomalies, cardiac involvement/aortic coarctation, and eye abnormalities. When ventral developmental defects are also present, the syndrome is referred to as PHACES. We report the prenatal and postnatal MRI findings in a case of PHACES with involvement of the right posteroinferior cerebellar artery. This case is exceptional because, to our knowledge, it is the first to report the findings at both prenatal and postnatal MRI and because of the unique vascular anomaly that widens the spectrum of possible intracranial arterial anomalies in this syndrome.
首字母缩略词PHACE所指的神经皮肤综合征是指节段性面部血管瘤与其他病症(包括后颅窝异常、脑血管异常、心脏受累/主动脉缩窄和眼部异常)的关联。当还存在腹侧发育缺陷时,该综合征被称为PHACES。我们报告了一例累及右后下小脑动脉的PHACES病例的产前和产后MRI检查结果。该病例很特殊,因为据我们所知,它是首例报告产前和产后MRI检查结果的病例,而且其独特的血管异常拓宽了该综合征可能出现的颅内动脉异常的范围。