PHACES综合征研究进展

中国口腔颌面外科杂志 ›› 2016, Vol. 14 ›› Issue (5): 467-470.

PHACES综合征研究进展

张凌, 郑家伟

上海交通大学医学院附属第九人民医院·口腔医学院口腔颌面-头颈肿瘤科, 上海市口腔医学重点实验室,上海 200011

收稿日期:2016-01-05 出版日期:2016-09-20 发布日期:2016-10-14
通讯作者: 郑家伟,Tel: 021-23271063, E-mail: zhjw@omschina.org.cn
作者简介:张凌（1978-）,男,主治医师,E-mail：topgun1128@126.com
基金资助:
国家自然科学基金（81271163）

Research advances in PHACES syndrome

ZHANG Ling, ZHENG Jia-wei

Department of Oral-Maxillofacial Head and Neck Oncology, Shanghai Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, Shanghai Key Laboratory of Stomatology. Shanghai 200011, China

Received:2016-01-05 Online:2016-09-20 Published:2016-10-14

摘要/Abstract

摘要： 血管瘤是儿童期最常见的良性肿瘤,一些少见病例可造成畸形。PHACES综合征是新近明确的以首字母缩写词组合而命名的一类病变,包括后颅窝畸形、血管瘤、动脉异常、主动脉狭窄和（或）心脏缺损、眼部异常及胸骨裂隙。其流行病学尚不清楚,但女性明显多发。全身系统性异常以神经及心脏畸形为主。本文就PHACES综合征的临床表现、诊断及治疗作一综述。

关键词: PHACES综合征, 血管瘤, 血管畸形

Abstract: Hemangiomas are the most common benign tumors in infancy, and in rare cases, they can be associated with dysmorphic malformation. The acronym, PHACES syndrome is a cutaneous condition characterized by multiple congenital abnormalities, including: posterior fossa brain malformations, hemangiomas of the face, arterial cerebrovascular anomalies, cardiovascular anomalies, eye anomalies and sternal defects/supraumbilical raphe. Its prevalence is unknown, but has a striking female preponderance. Among the systemic abnormalities, neurological and cardiac malformations predominate. PHACES syndrome had been recognized as a new and independent entity. This review highlighted the recent findings in clinical feature, diagnosis and management of PHACES syndrome.

Key words: PHACES syndrome, Hemangioma, Vascular malformation

中图分类号:

R739.8

张凌, 郑家伟. PHACES综合征研究进展[J]. 中国口腔颌面外科杂志, 2016, 14(5): 467-470.

ZHANG Ling, ZHENG Jia-wei. Research advances in PHACES syndrome[J]. China J Oral Maxillofac Surg, 2016, 14(5): 467-470.

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