An Unusual Cause of Neck Pain: Acquired Chiari Malformation Leading to Brainstem Herniation and Death

Abstract Background Chiari malformations are structural defects in which portions of the cerebellum are located below the foramen magnum. Of the four types of Chiari malformation, emergency physicians are most likely to encounter Type I (Chiari I). Chiari I malformations may be congenital or acquired. Congenital Chiari I malformations are most frequently encountered in the emergency department (ED) setting due to an exacerbation of subacute or chronic Chiari-related symptoms. However, acute Chiari-associated symptoms from an occult congenital or a secondary (acquired) Chiari malformation may occur. Objective To present a literature-guided approach to the identification and initial management of patients with Chiari I malformations in the ED setting. Case Report We present the case of a 30-year-old man who presented to the ED with isolated cervical region pain, and who subsequently died as a result of acute brainstem herniation from an acquired Chiari I malformation. Conclusions Although rare, acute Chiari I malformation may present to the ED. The new finding of a Chiari I malformation should be presumed acquired until proved otherwise, and should trigger an evaluation for central nervous system lesions or hydrocephalus. Brain imaging to exclude increased intracranial pressure and, in certain cases, specialty consultation, are important considerations.