Sun L L, Sun B, Ma Y C, Lei H Z, Dong C X
Department of Hemangioma Surgery, People's Hospital of Zhengzhou University, Zhengzhou 450003, China.
Zhonghua Kou Qiang Yi Xue Za Zhi. 2017 Nov 9;52(11):700-703. doi: 10.3760/cma.j.issn.1002-0098.2017.11.011.
PHACE syndrome is a syndrome of multiple organ and multisystem abnormalities associated with infantile segmental hemangioma, characterized by abnormal posterior fossa development, infant hemangioma, aortic abnormalities, aortic coarctation and heart defects, eye anomalies and other symptoms. The incidence of the disease is low, but there exist life-threatening factors. Once clinically diagnosed, it should be highly valued and multidisciplinary consultation must be conducted. This article reviews the diagnostic criteria of PHACE syndrome and its associated facial segmental hemangioma, as well as the treatment and prognosis of brain abnormalities.
PHACE综合征是一种与婴儿节段性血管瘤相关的多器官和多系统异常综合征，其特征为后颅窝发育异常、婴儿血管瘤、主动脉异常、主动脉缩窄和心脏缺陷、眼部异常等症状。该疾病发病率低，但存在危及生命的因素。一旦临床诊断，应高度重视并进行多学科会诊。本文综述了PHACE综合征及其相关面部节段性血管瘤的诊断标准，以及脑部异常的治疗和预后。
