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J Child Neurol. 2016 Mar;31(4):468-73. doi: 10.1177/0883073815599261. Epub 2015 Aug 13.

Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome.

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Department of Dermatology, Medical College of Wisconsin, Milwaukee WI, USA.
Vascular Anomalies Program, Lenox Hill Hospital, Northshore-LIJ Healthcare System, New York, NY, USA.
Department of Pediatrics, Division of Pediatric Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Department of Dermatology, Texas Children's Hospital, Houston, TX, USA.
Departments of Pediatrics and Neurology, Division of Pediatric Neurology, Nationwide Children's Hospital and The Ohio State University, Columbus, OH, USA


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.


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

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