Atypical presentation of Chiari I malformation with monoplegia and acquired torticollis

Abstract

There are several well-known causes for pediatric torticollis. However detailed review of the literature revealed only rarely association of torticollis with syringomyelia. We present a 4-year-old girl known to have torticollis since 18 months of age who presented acutely with right upper limb mono-paresis. Investigations revealed a Chiari type I malformation with extensive syringomyelia involving the whole of spinal cord extending from C1 to the conus. She responded very well to urgent posterior fossa decompression. Syringomyelia involving the entire spinal cord is very rare in children and presentation with monoplegia, to the best of our knowledge, has not been described before. We recommend that torticollis should always be taken seriously and a thorough clinical assessment supplemented by neuroimaging should be considered.

Keywords

Syringomyelia - torticollis - Chiari I malformation - monoplegia