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Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome. J Child Neurol 2016 Mar;31(4):468-73 PMID: 26271792

Pubmed ID

26271792

Abstract

PHACE (posterior fossa brain malformation, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) syndrome is a neurocutaneous disorder often involving the cerebral vasculature. PHACE patients appear to have early-onset and severe headaches more commonly than children without PHACE. The objective of this study was to characterize the clinical features and prevalence of headache by conducting a cross-sectional survey of families in 2 large PHACE registries. Sixty-six percent of eligible families completed the survey in which 62.7% of respondents reported headaches. Average age of headache onset was 48.8 months. Females were more likely to have headaches (68.6% vs 30.8%, P = .014). Families reported associated migrainous features including nausea (62.5%), vomiting (37.5%), photophobia (75%), and phonophobia (75%). Headaches occurred at least weekly in 29.4%, lasted ≥1 hour in 85.4%, and led to ≥1 hospital admission in 15.7%. Three respondents with headaches had at least 1 ischemic stroke. We demonstrated that headaches are common among PHACE patients, develop at an early age, and have migrainous features.

Author List

Yu J, Siegel DH, Drolet BA, Blei F, Epstein LG, Metry D, Heyer GL

Authors

Beth A. Drolet MD Professor in the Dermatology department at Medical College of Wisconsin
Dawn Siegel MD Associate Professor in the Dermatology department at Medical College of Wisconsin




Scopus

2-s2.0-84957939677   6 Citations

MESH terms used to index this publication - Major topics in bold

Age of Onset
Aortic Coarctation
Child
Child, Preschool
Cross-Sectional Studies
Eye Abnormalities
Family
Female
Headache
Humans
Infant
Male
Neurocutaneous Syndromes
Prevalence
Registries
Sex Factors
jenkins-FCD Prod-296 4db9d02597e0a2e889e230f853b641c12f1c3ee3