Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome
Publication Date
2016
Journal Title
J Child Neurol
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.
Volume Number
31
Issue Number
4
Pages
468-73
Document Type
Article
EPub Date
2015/08/15
Status
Faculty
Facility
School of Medicine
Primary Department
General Pediatrics
PMID
DOI
10.1177/0883073815599261