Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome

Jiade Yu, Dawn H. Siegel, Beth A. Drolet, Francine Blei, Leon G. Epstein, Denise Metry, Geoffrey L. Heyer*

*Corresponding author for this work

Research output: Contribution to journalArticle

8 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)468-473
Number of pages6
JournalJournal of child neurology
Volume31
Issue number4
DOIs
StatePublished - Mar 1 2016

Keywords

  • PHACE
  • Pascual-Castroviejo syndrome type II
  • adolescent
  • headache
  • hemangioma
  • migraine
  • pediatric
  • vasculopathy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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  • Cite this

    Yu, J., Siegel, D. H., Drolet, B. A., Blei, F., Epstein, L. G., Metry, D., & Heyer, G. L. (2016). Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome. Journal of child neurology, 31(4), 468-473. https://doi.org/10.1177/0883073815599261