The most typical symptom of Chiari malformation type I in children is headache. The authors describe a 14-year-old girl who presented with a 3-year history of gait decline and no headache, which is very unusual. After surgery to correct the Chiari I malformation, the patient's gait improved; however, she went on to develop decreased hand use with joint deformities. She was diagnosed with a probable connective tissue disorder. Patients with connective tissue disorders are at increased risk for developing Chiari I malformation. The authors discuss the possible reasons for the unusual presentation of the Chiari I malformation and possible mechanisms. The unusual presentation of Chiari I delayed this young patient's diagnosis and treatment.
- atrophic scarring
- connective tissue disorder
ASJC Scopus subject areas
- Clinical Neurology
- Pediatrics, Perinatology, and Child Health