Emergent decompression of Chiari malformation in a patient with progressive quadriparesis

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