Research progresses in the pathogenesis, diagnosis and treatment of infantile hemangioma with PHACE syndrome.
- Author:
Su-Hua PENG
1
;
Kai-Ying YANG
;
Si-Yuan CHEN
;
Yi JI
Author Information
1. Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China. jijiyuanyuan@163.com.
- Publication Type:Journal Article
- MeSH:
Abnormalities, Multiple;
diagnosis;
etiology;
therapy;
Eye Abnormalities;
diagnosis;
etiology;
therapy;
Heart Defects, Congenital;
diagnosis;
etiology;
therapy;
Hemangioma;
diagnosis;
etiology;
therapy;
Humans;
Infant
- From:
Chinese Journal of Contemporary Pediatrics
2017;19(12):1291-1296
- CountryChina
- Language:Chinese
-
Abstract:
Infant hemangioma, the most common benign tumor in children, is characterized by rapid proliferation, followed by slower spontaneous involution. However, some patients with facial segmental hemangioma are associated with PHACE syndrome. PHACE syndrome is characterized by vascular nerve and vascular cutaneous lesions of multiple systemic systems, often resulting in structural and functional impairments. Recent studies have demonstrated that the possible pathogeneses of PHACE syndrome mainly include hypoxia, abnormality of mesodermal vascular endothelial cells, genetic abnormality, and abnormality of interstitial mesenchymal stem cells. The current medications for hemangioma with PHACE syndrome include beta blockers, glucocorticoids, and mTOR inhibitors. This review article mainly describes the pathogenesis, diagnoses and treatments of PHACE syndrome, in order to provide directions for diagnosis and treatment of this disorder.