Abstract

Introduction: Chiari malformation type I (CM-I) is a condition characterized by an abnormal craniocervical transition whose main finding is cerebellar tonsillar ectopia of > 5 mm below the foramen magnum. The main clinical manifestations are occipital headache, truncal ataxia, corticospinal tract dysfunctions, and scoliosis.
 Objective: The scope of this article is to report a rare case of papilledema and visual impairment in a child diagnosed with CM-I.
 Methods/case presentation: Presentation of the case of an 8-year-old child with difficulty in performing school activities due to visual blurring in the 3 days prior to hospitalization. She was taken by her parents to the ophthalmologist who identified bilateral papilledema and was urgently referred to the pediatric emergency unit. She underwent magnetic resonance imaging of the brain and CM-I was identified. She underwent urgent surgical posterior cranial fossa decompression and progressed to complete visual improvement, confirmed by ophthalmological examination.
 Conclusion: Papilledema may be the initial clinical manifestation of CM-I in children and urgent surgical decompression of the posterior cranial fossa may prevent or even reverse the visual impairment.

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