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伴有颅内颈内动脉缺如和 Willis 环异常的 PHACE(S) 综合征

PHACE(S) Syndrome With Absent Intracranial Internal Carotid Artery and Anomalous Circle of Willis.

作者信息

Winter Pieta R, Itinteang Tinte, Leadbitter Philip, FitzJohn Trevor, Tan Swee T

机构信息

*Gillies McIndoe Research Institute †Maxillofacial and Burns Unit, Centre for the Study and Treatment of Vascular Birthmarks, Wellington Regional Plastic ‡Department of Paediatrics, Hutt Hospital, Wellington, New Zealand.

出版信息

J Craniofac Surg. 2015 Jun;26(4):e315-7. doi: 10.1097/SCS.0000000000001701.

DOI:10.1097/SCS.0000000000001701
PMID:26080245
Abstract

The authors present a case of PHACE(S) (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, eye anomalies, and sternal cleft or supraumbilical raphe) syndrome with a right-sided segmental infantile hemangioma, and describe in detail, the associated absent ipsilateral intracranial internal carotid artery and anomalous Circle of Willis. Propranolol therapy led to accelerated, complete involution. Nadolol may reduce the theoretical risk of treating PHACE(S) patients with β-blockers.

摘要

作者报告了一例患有右侧节段性婴儿血管瘤的PHACE(S)(后颅窝畸形、血管瘤、动脉异常、心脏缺陷、眼部异常以及胸骨裂或脐上缝)综合征病例,并详细描述了同侧颅内颈内动脉缺如及异常的Willis环。普萘洛尔治疗导致血管瘤加速、完全消退。纳多洛尔可能会降低使用β受体阻滞剂治疗PHACE(S)患者的理论风险。

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PHACE(S) Syndrome With Absent Intracranial Internal Carotid Artery and Anomalous Circle of Willis.伴有颅内颈内动脉缺如和 Willis 环异常的 PHACE(S) 综合征
J Craniofac Surg. 2015 Jun;26(4):e315-7. doi: 10.1097/SCS.0000000000001701.
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引用本文的文献

1
[Research progresses in the pathogenesis, diagnosis and treatment of infantile hemangioma with PHACE syndrome].[PHACE综合征相关性婴幼儿血管瘤发病机制、诊断及治疗的研究进展]
Zhongguo Dang Dai Er Ke Za Zhi. 2017 Dec;19(12):1291-1296. doi: 10.7499/j.issn.1008-8830.2017.12.013.