Literature DB >> 16620231

PHACES syndrome presenting as hemangiomas, sternal clefting and congenital ulcerations on the helices.

Cicek Durusoy1, Ercan Mihci, Sukran Tacoy, Eda Ozaydin, Erkan Alpsoy.   

Abstract

Sternal malformation/vascular dysplasia association is a very rare condition comprised of midline defects and hemangiomas of the face and anterior trunk, that can be found as part of the PHACES phenotypic spectrum (posterior fossa malformations, hemangiomas, arterial anomalies, coarctations of the aorta, cardiac defects and eye abnormalities, sternal clefting). Herein, we describe a 6-month-old boy with sternal cleft, extensive segmental hemangiomas, and a depigmented scar on the tip of the xyphoid process, corresponding to a sternal malformation/vascular dysplasia association. He also had bilateral cutaneous ulcerations on the helices. Our case report indicates that ulceration of a hemangioma can occur before significant proliferation and may even be present congenitally.

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Year:  2006        PMID: 16620231     DOI: 10.1111/j.1346-8138.2006.00050.x

Source DB:  PubMed          Journal:  J Dermatol        ISSN: 0385-2407            Impact factor:   4.005


  3 in total

1.  Multiple facets of PHACE(S).

Authors:  Geetha Anand; Kilian Keaney; Michael Anthony; Gerardine Quaghebeur
Journal:  BMJ Case Rep       Date:  2010-03-08

Review 2.  Phenotypic spectrum and management of sternal cleft: literature review and presentation of a new series.

Authors:  Michele Torre; Giovanni Rapuzzi; Marcello Carlucci; Luca Pio; Vincenzo Jasonni
Journal:  Eur J Cardiothorac Surg       Date:  2012-01       Impact factor: 4.191

3.  A girl with sternal malformation/vascular dysplasia association.

Authors:  Na Yong Lee; Hye Kyung Cho; Kyung-Hyo Kim; Eun Ae Park
Journal:  Korean J Pediatr       Date:  2013-03-18
  3 in total

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