Winter Pieta R, Itinteang Tinte, Leadbitter Phillip, Tan Swee T
Gillies McIndoe Research Institute, Wellington, New Zealand.
Centre for the Study & Treatment of Vascular Birthmarks, Wellington Regional Plastic, Maxillofacial & Burns Unit, Wellington, New Zealand.
Acta Paediatr. 2016 Feb;105(2):145-53. doi: 10.1111/apa.13242. Epub 2015 Nov 27.
PHACE syndrome comprises a spectrum of anomalies including posterior fossa malformations, haemangioma, arterial anomalies, cardiac defects and eye anomalies. PHACE should be considered in any patient with a large facial segmental infantile haemangioma (IH), and multidisciplinary management is crucial. Low-dose propranolol is effectively for the treatment of IH associated with PHACE syndrome. Recent evidence suggests IH is comprised of mesoderm-derived haemogenic endothelium.
The embryonic developmental anomaly nature of IH provides an insight into the origin of PHACE syndrome.
PHACE综合征包括一系列异常，包括后颅窝畸形、血管瘤、动脉异常、心脏缺陷和眼部异常。任何患有大面积面部节段性婴儿血管瘤（IH）的患者都应考虑PHACE综合征，多学科管理至关重要。低剂量普萘洛尔对治疗与PHACE综合征相关的IH有效。最近的证据表明，IH由中胚层来源的造血内皮组成。
IH的胚胎发育异常性质为深入了解PHACE综合征的起源提供了线索。
