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PHACES: Are There Cutaneous Manifestations Associated with Cerebrovascular Anomalies?
Marc Serret M.D., Brendan Alleyne B.S., Arun Gosain M.D.
Case Western Reserve University/Department of Plastic & Reconstructive Surgery
2012-01-02
Presenter: Marc Serret M.D.
Affidavit:
Also being prepared for Senior Resident's Conference, but has not been presented to date; nor has this been published in any format.
Director Name: Arun Gosain M.D.
Presentation Category: ClinicalAbstract Category: Craniomaxillofacial
How does this presentation meet the established conference educational objectives?
Helps provide useful information regarding PHACES that is new to the literature.
How will your presentation be used by practicing physicians in the audience?
Introduces a feature of PHACES that helps physicians better screen patients who are at risk for cerebrovascular anomalies.
INTRODUCTION: PHACES Syndrome has been described as the association of several clinically recognizable features in patients who present with segmental cervical/facial hemangiomas. Current standards identify patients with infantile hemangiomas in one of 4 segmental cervicofacial distributions to be at risk for PHACES. The present study was performed to better identify hemangiomas in the cervicofacial distribution that are associated with cerebrovascular abnormalities, to provide guidelines as to which patients presenting with infantile hemangiomas warrant further investigation.
METHODS: A retrospective review of five patients with infantile hemangiomas of the head/neck and cerebrovascular anomalies was performed. Each patient reviewed was confirmed to have PHACES and assessed for potential associations. Percentages of facial hemangioma involvement were also reviewed through retrospective clinical photography analysis.
RESULTS: A spectrum of facial hemangioma involvement was seen ranging from sole eyelid involvement to hemi-face involvement. All cases reviewed demonstrated features compatible with PHACES syndrome (table 1). One patient studied did not demonstrate segmental hemangioma presentation, but instead demonstrated complete upper eyelid involvement. All five patients had positive upper eyelid hemangioma involvement, which directly correlated with cerebrovascular malformation occurrences (table 1).
CONCLUSIONS: Reports of PHACES typically showcase large percentage of cervicofacial hemangioma involvement, but smaller percentage involvement can be equally suggestive if involving the complete upper eyelid. In our series, complete upper eyelid hemangioma involvement was found to be a reliable marker for cerebrovascular anomalies. This suggests complete upper eyelid involvement can be useful for prompting a PHACES workup, irrespective of the percentage of cervicofacial hemangioma involvement.
