Two differential cavities in syringomyelia of pediatric Chiari I malformation presenting with unilateral foot drop

Published:December 16, 2022DOI:



      Patients with Chiari I malformation (CM1) may have chronic symptoms of syringomyelia, including numbness and weakness of the upper limbs, typically during young adulthood. Acute or subacute presentation of unilateral foot drop has been rarely reported as a first symptom of CM1-associated syringomyelia exclusively in childhood or adolescence. Why these patients do not show any symptoms of the upper limbs although holocord syringomyelia is always observed on magnetic resonance imaging (MRI) is unclear.

      Case presentation

      A four-year-old girl presented rapidly with isolated left foot drop. Conventional MRI revealed holocord syringomyelia associated with CM1. Three-dimensional constructive interference in steady state (3D-CISS) imaging further demonstrated that the syringomyelia was comprised of two differential cavities that communicated with each other via a small pore: a centrally positioned upper cavity and a left-deviated lower one. Surgical decompression of the foramen magnum resolved the symptom with radiological improvement of the two cavities.


      In contrast to a centrally enlarged syrinx that is often asymptomatic, a paracentrally extended syrinx usually produces segmental signs related to its levels. Thus, the left foot drop in this case would have been due to the ipsilaterally deviated lower cavity that was distinguished from the central upper cavity by 3D-CISS imaging. Further reports using this imaging technique are needed to verify the hypothetic pathology.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Brain and Development
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Jayamanne C.
        • Fernando L.
        • Mettananda S.
        Chiari malformation type 1 presenting as unilateral progressive foot drop: a case report and review of literature.
        BMC Pediatr. 2018; 18: 34
        • Karras C.L.
        • Murthy N.K.
        • Trybula S.J.
        • Alden T.D.
        Chiari malformation type 1 presenting as isolated unilateral foot drop with rapid recovery following posterior fossa decompression.
        Childs Nerv Syst. 2022; 38: 821-825
        • Panda A.K.
        • Kaur M.
        Rapidly progressive foot drop: an uncommon and underappreciated cause of Chiari I malformation and holocord syrinx.
        BMJ Case Rep. 2013; 2013bcr2013009644
        • McMillan H.J.
        • Sell E.
        • Nzau M.
        • Ventureyra E.C.
        Chiari 1 malformation and holocord syringomyelia presenting as abrupt onset foot drop.
        Childs Nerv Syst. 2011; 27: 183-186
        • Laufer I.
        • Engel M.
        • Feldstein N.
        • Souweidane M.M.
        Chiari malformation presenting as a focal motor deficit. Report of two cases.
        J Neurosurg Pediatr. 2008; 1: 392-395
        • Henderson D.
        • Alix J.J.P.
        • Sinha S.
        Holocord syrinx presenting with foot drop.
        J Surg Case Rep. 2019; 2019: rjz082
        • Muhn N.
        • Baker S.K.
        • Hollenberg R.D.
        • Meaney B.F.
        • Tarnopolsky M.A.
        Syringomyelia presenting as rapidly progressive foot drop.
        J Clin Neuromuscul Dis. 2002; 3: 133-134
        • Milhorat T.H.
        • Johnson R.W.
        • Milhorat R.H.
        • Capocelli Jr., A.L.
        • Pevsner P.H.
        Clinicopathological correlations in syringomyelia using axial magnetic resonance imaging.
        Neurosurgery. 1995; 37: 206-213
        • Hirai T.
        • Korogi Y.
        • Shigematsu Y.
        • Sugahara T.
        • Takahashi M.
        • Ushio Y.
        • et al.
        Evaluation of syringomyelia with three-dimensional constructive interference in a steady state (CISS) sequence.
        J Magn Reson Imaging. 2000; 11: 120-126
        • Urbizu A.
        • Toma C.
        • Poca M.A.
        • Sahuquillo J.
        • Cuenca-León E.
        • Cormand B.
        • et al.
        Chiari malformation type I: a case-control association study of 58 developmental genes.
        PLoS One. 2013; 8: e57241
        • Tubbs R.S.
        • Rutledge S.L.
        • Kosentka A.
        • Bartolucci A.A.
        • Oakes W.J.
        Chiari I malformation and neurofibromatosis type 1.
        Pediatr Neurol. 2004; 30: 278-280