RESUMO
AIM: To describe the rates of stroke and craniocervical vasculopathy progression in children with posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects, and eye abnormalities (PHACE) syndrome. METHOD: A single-center, retrospective natural history study of children with PHACE syndrome. Clinical and sequential neuroimaging data were reviewed to study the characteristics and progression of vasculopathy and calculate the rates of arterial ischemic stroke (AIS) and transient ischemic stroke (TIA). Vasculopathy progression was defined as worsening or new vascular findings on follow-up magnetic resonance angiography. RESULTS: Thirty-four children with cerebrovascular abnormalities at the PHACE syndrome diagnosis were studied (age range = 2 to 18 years, 85% females). Median age at the initial diagnosis was 5.5 months (interquartile range = 1-52 months); median age at the last follow-up was 8 years 6 months (range = 2-18 years). Overall, 10 (29%) patients had radiological progression of their vasculopathy, with a cumulative progression-free rate of 73% (95% confidence interval [CI] = 0.57-0.89), and a cumulative TIA-free and AIS-free rate of 87% (95% CI = 0.745-0.99). Vasculopathy was continuously progressive in six patients (18%) at the last follow-up. Three patients (9%) had TIA and all had progressive vasculopathy. One patient had presumed perinatal AIS at the initial PHACE diagnosis, while no other patient experienced an AIS during the follow-up. INTERPRETATION: In children with PHACE syndrome, craniocervical vasculopathy is non-progressive and asymptomatic in the majority of cases. The risk of ischemic stroke in these children is very low. Larger and prospective studies are necessary to confirm these findings.
RESUMO
BACKGROUND: Sinus headache is one of the most frequent misdiagnosis given to children with headache. The objective of this study is to evaluate the frequency of sinus disease in children with headache that do not fulfill the criteria for headache attributed to disorder of the nose or paranasal sinuses. METHODS: This is a prospective study conducted at the authors' pediatric neurology clinic. Data from children with headache was evaluated and compared with a disease control group composed of children without history of headache. All patients underwent neuroimaging assessment. Patients with diagnosis of acute infectious sinus disease were excluded from the analysis. The type of headache was classified according to the International Headache Society. Statistical analysis was performed using the Fisher exact test, with a level of significance of .05. RESULTS: A total of 62 patients with headache were evaluated; 24 boys, 38 girls, ages ranging from 3 to 18 years (mean = 9.7 years). Of the patients, 29 had migraine without aura, 4 had frequent episodic tension type headache, 3 had both migraine without aura and frequent episodic tension type headache, 3 had migraine with brainstem aura, 2 had episodic tension type headache, 1 had migraine with aura. In 20 patients the type of headache could not be established. The disease control group had 41 patients; 25 boys, 16 girls, ages ranging from 3 to 17 years (mean = 7.3 years). Sinus abnormalities detected by neuroimaging were present in 12 patients in the headache group and in 11 patients in the disease control group ( P = .469). CONCLUSION: The authors conclude that sinus abnormalities are a common finding in neuroimaging tests of children with or without headache. Sinus disease disclosed by neuroimaging evaluation should not preclude the diagnosis of migraine or other types of primary headache.
