Clinical Presentation of Pediatric Chiari I Malformations



  • Clinical presentation of the Chiari I malformation varies with patient age, and children typically present with shorter symptom duration than adults. Symptoms and signs of Chiari I malformation in children are most often attributable to impairment of cerebrospinal fluid (CSF) flow, direct neural compression, and/or syringomyelia. Comparison of contemporary and older literature reveals a trend toward earlier diagnosis, prior to manifestation of significant neurologic morbidity. General availability and use of magnetic resonance imaging likely explains this dramatic shift. Occipital and/or cervical pain is the most common presentation, particularly if Valsalva-induced, followed by scoliosis, syringomyelia, and lower cranial nerve dysfunction. Headache not localized to the occipital/cervical region correlates poorly with Chiari I malformation. The natural history of asymptomatic children with Chiari I malformation (absent syringomyelia) appears benign. Surgical treatment should be reserved for patients with localized pain, syringomyelia, or cervicomedullary/cerebellar dysfunction.
  • Authors

    Digital Object Identifier (doi)

    International Standard Book Number (isbn) 13

  • 9783030448615
  • Start Page

  • 375
  • End Page

  • 379