The natural history of the Chiari malformation Type I (CM-I) is incompletely understood. The authors report on the outcome of a large group of patients with CM-I that were initially selected for nonsurgical management.
The authors identified 147 patients in whom CM-I was diagnosed on MR imaging, who were not offered surgery at the time of diagnosis, and in whom at least 1 year of clinical and MR imaging follow-up was available after the initial CM-I diagnosis. These patients were included in an outcome analysis.
Patients were followed clinically and by MR imaging for a mean duration of 4.6 and 3.8 years, respectively. Of the 147 patients, 9 had new symptoms attributed to the CM-I during the follow-up interval. During this time, development of a spinal cord syrinx occurred in 8 patients; 5 of these patients had a prior diagnosis of a presyrinx state or a dilated central canal. Spontaneous resolution of a syrinx occurred in 3 patients. Multiple CSF flow studies were obtained in 74 patients. Of these patients, 23 had improvement in CSF flow, 39 had no change, and 12 showed worsening CSF flow at the foramen magnum. There was no significant change in the mean amount of cerebellar tonsillar herniation over the follow-up period. Fourteen patients underwent surgical treatment for CM-I. There were no differences in initial cerebellar tonsillar herniation or CSF flow at the foramen magnum in those who ultimately underwent surgery compared with those who did not.
In patients with CM-Is that are selected for nonsurgical management, the natural history is usually benign, although spontaneous improvement and worsening are occasionally seen.
Abbreviations used in this paper: CM-I = Chiari malformation Type I; EMERSE = Electronic Medical Record Search Engine.
Address correspondence to: Cormac O. Maher, M.D., Department of Neurosurgery, University of Michigan, 1500 East Medical Center Drive, Room 3552 Taubman Center, Ann Arbor, Michigan 48109-5338. email: firstname.lastname@example.org.
BogdanovEIMendelevichEG: Syrinx size and duration of symptoms predict the pace of progressive myelopathy: retrospective analysis of 103 unoperated cases with craniocervical junction malformations and syringomyelia. Clin Neurol Neurosurg104:90–972002
EuleJMEricksonMAO'BrienMFHandlerM: Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population. Spine (Phila Pa 1976)27:1451–14552002
HarounRIGuarnieriMMeadowJJKrautMCarsonBS: Current opinions for the treatment of syringomyelia and chiari malformations: survey of the Pediatric Section of the American Association of Neurological Surgeons. Pediatr Neurosurg33:311–3172000
OldfieldEHMuraszkoKShawkerTHPatronasNJ: Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg80:3–151994