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

doi:10.1177/0883073815599261

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

Pediatrics

Research output: Contribution to journal › Article › peer-review

11 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 language	English (US)
Pages (from-to)	468-473
Number of pages	6
Journal	Journal of child neurology
Volume	31
Issue number	4
DOIs	https://doi.org/10.1177/0883073815599261
State	Published - 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

Access to Document

10.1177/0883073815599261

Cite this

@article{56c9c100dffc4bb3b15e33b537343a88,

title = "Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome",

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",

author = "Jiade Yu and Siegel, {Dawn H.} and Drolet, {Beth A.} and Francine Blei and Epstein, {Leon G.} and Denise Metry and Heyer, {Geoffrey L.}",

note = "Funding Information: This work was performed at the Medical College of Wisconsin and the Children''s Hospital of Wisconsin in Milwaukee, Wisconsin. The authors acknowledge Shawna Joachim (Medical College of Wisconsin) for her assistance with IRB approval, editing, and submission assistance. We want to thank all the patients and their respective families for responding to our survey and to helping further our understanding of PHACE syndrome. We would also like to thank the PHACE Syndrome Community for their continued support for our research and, finally, we would like to thank the NIH for their generous grant support and the Greater Milwaukee Foundation for supporting this study. The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: Funding for this study was provided by the National Institute of Health (R01 AR064258-01A1). Publisher Copyright: {\textcopyright} SAGE Publications.",

year = "2016",

month = mar,

day = "1",

doi = "10.1177/0883073815599261",

language = "English (US)",

volume = "31",

pages = "468--473",

journal = "Journal of Child Neurology",

issn = "0883-0738",

publisher = "SAGE Publications Inc.",

number = "4",

}

TY - JOUR

T1 - Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome

AU - Yu, Jiade

AU - Siegel, Dawn H.

AU - Drolet, Beth A.

AU - Blei, Francine

AU - Epstein, Leon G.

AU - Metry, Denise

AU - Heyer, Geoffrey L.

N1 - Funding Information: This work was performed at the Medical College of Wisconsin and the Children''s Hospital of Wisconsin in Milwaukee, Wisconsin. The authors acknowledge Shawna Joachim (Medical College of Wisconsin) for her assistance with IRB approval, editing, and submission assistance. We want to thank all the patients and their respective families for responding to our survey and to helping further our understanding of PHACE syndrome. We would also like to thank the PHACE Syndrome Community for their continued support for our research and, finally, we would like to thank the NIH for their generous grant support and the Greater Milwaukee Foundation for supporting this study. The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: Funding for this study was provided by the National Institute of Health (R01 AR064258-01A1). Publisher Copyright: © SAGE Publications.

PY - 2016/3/1

Y1 - 2016/3/1

N2 - 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.

AB - 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.

KW - PHACE

KW - Pascual-Castroviejo syndrome type II

KW - adolescent

KW - headache

KW - hemangioma

KW - migraine

KW - pediatric

KW - vasculopathy

UR - http://www.scopus.com/inward/record.url?scp=84957939677&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84957939677&partnerID=8YFLogxK

U2 - 10.1177/0883073815599261

DO - 10.1177/0883073815599261

M3 - Article

C2 - 26271792

AN - SCOPUS:84957939677

SN - 0883-0738

VL - 31

SP - 468

EP - 473

JO - Journal of Child Neurology

JF - Journal of Child Neurology

IS - 4

ER -

Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this