Emergent decompression of Chiari malformation in a patient with progressive quadriparesis

https://doi.org/10.1016/j.inat.2021.101359Get rights and content
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Highlights

  • Chiari malformations can cause headache; rapid functional decline is not typical.

  • Surgical enlargement of the posterior fossa is a mainstay of symptomatic treatment.

  • Here a 3yo with CM experienced acute progressive quadriparesis.

  • She underwent emergent suboccipital decompression with full motor recovery.

  • This reports a rare instance when emergent Chiari decompression may be warranted.

Abstract

Background

Chiari I malformations are marked by the congenital underdevelopment of the posterior fossa, leading to downward herniation of the cerebellar tonsils and crowding of the contents of the foramen magnum with an altered state of cerebrospinal fluid (CSF) flow. These malformations may or may not be accompanied by syringomyelia, and clinical symptoms vary from occipital headache to cranial nerve dysfunction, including oropharyngeal or respiratory dysfunction. Surgical enlargement of the posterior fossa is a mainstay of treatment and is generally performed as an elective procedure in response to an insidious progression of symptoms. Rapid decline is not a hallmark of the condition and urgent interventions are only scarcely reported in the literature.

Observation(s)

Here we present the case of a 3-year-old female with a Chiari I malformation and sickle cell disease, who experienced acute progressive neurologic deficits prompting emergent suboccipital decompression. She subsequently sustained a full recovery of neurologic function.

Lessons

This report adds to the literature and understanding of these rare instances when emergent Chiari decompression may be warranted.

Keywords

Chiari malformation
Decompression
Quadriparesis

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