Abstract
Study design: Case study on a 45-year-old female with progressive weakness and paresthesias in her lower extremities and a magnetic resonance image of an intramedullary mass at the level of C5–6 and a Klippel–Feil (KF) deformity ventral to the lesion.
Objective: Present an interesting case of an intramedullary mass coexisting with a ventral KF deformity with review of the medical literature on intramedullary masses and cervical spine biomechanics.
Setting: New York city, New York, USA.
Methods: Case study with discussion of neurosurgical and neuropathological findings and review of the literature.
Results: The patient underwent open neurosurgical excisional biopsy of the intramedullary mass which revealed a non-neoplastic inflammatory mass that slowly resolved with medical management.
Conclusions: While no definitive etiology was found in this case we offer two interesting mechanisms: (1) maldevelopment of the cervical spine or (2) this inflammatory mass is in response to an abnormal motion at the level of the Klippel–Feil.
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References
Sheehan JP, Sheehan JM, Lopes MB, Jane Sr JA . Thoracic diastematomyelia with concurrent intradural epidermoid spinal cord tumor and cervical syrinx in an adult. Case Report. J Neurosurg 2002; 97: 231–234.
Steinbok P . Dysraphic lesions of the cervical spinal cord. Neurosurg Clin N Am 1995; 6: 367–376.
Whittle IR, Besser M . Congenital neural abnormalities presenting with mirror movements in a patient with Klippel–Feil syndrome. Case Report. J Neurosurg 1983; 59: 891–894.
Nagashima H, Morio Y, Teshima R . No neurological involvement for more than 40 years in Klippel–Feil syndrome with severe hypermobility of the upper cervical spine. Arch Orthop Trauma Surg 2001; 121: 99–101.
Kinne RW, Brauer R, Stuhlmuller B, Palombo-Kinne E, Burmester G . Macrophages in rheumatoid arthritis. Arthritis Res 2000; 2: 189–202.
Blight AR . Macrophages and inflammatory damage in spinal cord injury. J Neurotrauma 1992; 9: 83–91.
Benzel EC . Biomechanics of Spine Stabilization. American Association of Neurological Surgeons: Rollins Meadows, Illinois, USA 2001, pp 36–37.
Lee M, Epstein FJ, Rezai AR, Zagzag D . Nonneoplastic intramedullary spinal cord lesions mimicking tumors. Neurosurgery 1998; 43: 788–794.
Vaquero J, Herrero J, Cabezudo J, Leunda G . Klippel–Feil syndrome with epidural fibroblastoma in the area of vertebral fusion. Arch Neurol 1982; 39: 318–319.
Young WF, Boyko O . Magnetic resonance imaging confirmation of resolution of periodontoid pannus formation following C1/2 posterior transarticular screw fixation. J Clin Neurosci 2002; 9: 434–436.
Vaidyanathan S, Hughes PL, Soni BM, Singh G, Sett P . Klippel–Feil syndrome – the risk of cervical spinal cord injury: case report. BMC Fam Pract 2002; 3: 6.
Kulkarni V, Rajshekhar V, Raghuram L . Accelerated spondylotic changes adjacent to the fused segment following central cervical corpectomy: magnetic resonance imaging evidence. J Neurosurg 2004; 100(1 Suppl): 2–6.
Whiting DM, Chou SM, Lanzieri CF, Kalfas IH, Hardy RW . Cervical neurenteric cyst associated with Klippel–Feil syndrome: a case report and review of the literature. Clin Neuropathol 1991; 10: 285–290.
Cohen-Gadol AA, Zikel OM, Miller GM, Aksamitt A, Scheithauer BW, Krauss WE . Spinal cord biopsy: a review of 38 cases. Neurosurgery 2003; 52: 806–816.
Dias MS, Walker ML . The embryogenesis of complex dysraphic malformations: a disorder of gastrulation? Pediatr Neurosurg 1992; 18: 229–253.
David KM, Copp AJ, Stevens JM, Hayward RD, Crockard HA . Split cervical spinal cord with Klippel–Feil syndrome: seven cases. Brain 1996; 119: 1859–1872.
Epstein NE, Epstein JA, Zilkha A . Traumatic myelopathy in a seventeen-year old child with cervical stenosis (without fracture or dislocation) and a C2–C3 Klippel–Feil fusion. A case report. Spine 1984; 4: 344–347.
Holliday PO, Davis C, Angelo J . Multiple meningiomas of the cervical spinal cord associated with Kippel–Feil malformation and atlantooccipital assimilation. Neurosurgery 1984; 14: 353–357.
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Dickerman, R., Colle, K. & Mittler, M. Intramedullary inflammatory mass dorsal to the Klippel–Feil deformity: error in development or response to an abnormal motion segment?. Spinal Cord 42, 720–722 (2004). https://doi.org/10.1038/sj.sc.3101645
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DOI: https://doi.org/10.1038/sj.sc.3101645
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