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Headache in children with Chiari I malformation
Irene Toldo1, Marta Tangari, Rodica Mardari
1Juvenile Headache Centre, Department of Woman and Child Health, University of Padua, Padua, Italy.
Insights
Headache is the most common symptom in children with Chiari 1 malformation. Headache attributed to Chiari 1 malformation was the most frequent pattern observed in this pediatric group.
Area of Science:
- Neurology
- Pediatrics
- Neurosurgery
Background:
- Chiari 1 malformation involves cerebellar tonsil herniation through the foramen magnum.
- Headache is a primary symptom, but its pattern in pediatric cases is not well-defined.
- Understanding headache characteristics is crucial for diagnosis and management in children.
Purpose of the Study:
- To investigate the frequency and characteristics of headaches in children diagnosed with Chiari 1 malformation.
- To analyze headache patterns at initial evaluation and during follow-up.
- To explore associations between headache patterns, clinical signs, and the extent of tonsillar ectopia.
Main Methods:
- Retrospective chart review of 45 pediatric patients (<18 years) with tonsillar ectopia.
- Neuroimaging data analyzed between 2002 and 2010.
- Semistructured interviews and headache classification using the International Classification of Headache Disorders (ICHD-2).
Main Results:
- Headache was reported by 73% of pediatric Chiari 1 malformation patients.
- Headache attributed to Chiari 1 malformation was identified in 27% of these patients.
- Associations found between clinical presentation, headache patterns, and the degree of tonsillar ectopia (borderline, mild, severe).
Conclusions:
- Headache is the predominant symptom in pediatric Chiari 1 malformation.
- Headache attributed to Chiari 1 malformation represents the most common headache pattern in this population.
- The presence of headache attributed to Chiari 1 malformation plus three other signs/symptoms strongly predicts severe tonsillar ectopia.
Background:
Headache is the most common symptom of Chiari 1 malformation, a condition characterized by the herniation of cerebellar tonsils through the foramen magnum. However, the headache pattern of cases with Chiari 1 malformations is not well defined in the literature, especially in children.
Objective:
The aim of this retrospective chart review was to evaluate the frequency and the characteristics of headache in children with Chiari 1 malformation at initial evaluation and during follow up.
Methods:
Forty-five cases with tonsillar ectopia were selected among 9947 cases under 18 years of age who underwent neuroimaging between 2002 and 2010. A semistructured clinical interview (mean follow-up: 5.2 years) was conducted. Headache was classified according to the second edition of the International Classification of Headache Disorders.
Results:
Possible associations between clinical picture, in particular headache pattern, but also other signs and symptoms attributable to Chiari 1 malformation, and the extent of tonsillar ectopia were found for 3 different groups: those with borderline (<5 mm, N = 12), mild (5-9 mm, N = 27), and severe tonsillar ectopia (≥10 mm, N = 6), respectively. Twenty-four out of 33 (73%) cases with Chiari 1 malformation complained of headache, and 9/33 (27%) of those patients (5 with mild and 4 with severe tonsillar ectopia) reported headache attributed to Chiari 1 malformation.
Conclusions:
In our studied pediatric population, the most common symptom for cases diagnosed with Chiari 1 malformation was headache, and headache attributed to Chiari 1 malformation was the most common headache pattern in patients with Chiari 1 malformation. The presence of headache attributed to Chiari 1 malformation along with 3 other signs or symptoms of Chiari 1 malformation were highly predictive of severe tonsillar ectopia.
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