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Published on: June 2, 2014
Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome
JiaDe Yu1, Dawn H Siegel1, Beth A Drolet1
1Department of Dermatology, Medical College of Wisconsin, Milwaukee WI, USA.
Insights
Headaches are common in children with PHACE syndrome, often starting early and presenting with migraine-like symptoms. This highlights the need for early diagnosis and management of headaches in this population.
Area of Science:
- Neurology
- Genetics
- Pediatrics
Background:
- PHACE syndrome is a complex neurocutaneous disorder involving brain malformations and vascular anomalies.
- Headaches are frequently reported in PHACE patients, suggesting a potential link to the syndrome's underlying pathology.
Purpose of the Study:
- To investigate the prevalence and clinical characteristics of headaches in children with PHACE syndrome.
- To identify associated migrainous features and the impact of headaches on affected individuals.
Main Methods:
- A cross-sectional survey was administered to families registered in two large PHACE syndrome registries.
- Data collected included headache prevalence, age of onset, associated symptoms, frequency, duration, and impact on hospital admissions.
Main Results:
- Headaches were reported by 62.7% of surveyed families, with an average onset age of 48.8 months.
- Females were more likely to experience headaches (68.6% vs 30.8%).
- Common associated features included nausea, vomiting, photophobia, and phonophobia. Headaches occurred weekly in 29.4% and led to hospital admissions in 15.7%.
Conclusions:
- Headaches are a prevalent and early-onset symptom in PHACE syndrome.
- The migrainous nature of these headaches warrants further investigation and targeted management strategies.
- Early identification and treatment of headaches may reduce associated morbidity, including ischemic stroke risk.
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.
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