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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.

Author information

1
Department of Dermatology, Medical College of Wisconsin, Milwaukee WI, USA.
2
Vascular Anomalies Program, Lenox Hill Hospital, Northshore-LIJ Healthcare System, New York, NY, USA.
3
Department of Pediatrics, Division of Pediatric Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
4
Department of Dermatology, Texas Children's Hospital, Houston, TX, USA.
5
Departments of Pediatrics and Neurology, Division of Pediatric Neurology, Nationwide Children's Hospital and The Ohio State University, Columbus, OH, USA geoffrey.heyer@nationwidechildrens.org.

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.

KEYWORDS:

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

PMID:
26271792
DOI:
10.1177/0883073815599261
[Indexed for MEDLINE]

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