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Clinical Pediatrics
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Notes

The Association of Chiari Type I Malformation and Neurofibromatosis Type 1

Joseph Dooley, M.B., F.R.C.P.C.

Departments of Pediatrics (Neurology) and Radiology Dalhousie University and The IWK Children's Hospital Halifax, Nova Scotia, Canada

Daniel Vaughan, M.D.

Departments of Pediatrics (Neurology) and Radiology Dalhousie University and The IWK Children's Hospital Halifax, Nova Scotia, Canada

Michael Riding, M.B., F.R.C.P.C.

Departments of Pediatrics (Neurology) and Radiology Dalhousie University and The IWK Children's Hospital Halifax, Nova Scotia, Canada

Peter Camfield, M.D., F.R.C.P.C.

Departments of Pediatrics (Neurology) and Radiology Dalhousie University and The IWK Children's Hospital Halifax, Nova Scotia, Canada

The association of neurofibromatosis type 1 (NF1) with Chiari malformations of the cerebellum and brain stem has been reported on only two previous occasions.1,2 The pathogenesis of both conditions has remained unclear, although the Chiari type I malformation is most likely due to hypoplasia of the posterior fossa with subsequent extension of the cerebellum through the foramen magnum.3 NF1 is also associated with a variety of cerebral dysplasias.4 We present a patient with both of these dysplastic lesions whose Chiari malformation was asymptomatic.

Clinical Pediatrics, Vol. 32, No. 3, 189-190 (1993)
DOI: 10.1177/000992289303200316
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