Introduction: Syringomyelia is a neurological disease
characterized by the presence of fluid-filled cavities within the spinal cord,
resulting in progressive neurological deficits. Intramedullary
hemangioblastomas are often accompanied by syringomyelia. However, holochordial
syringomyelia is rare. The most common cause of syringomyelia remains Chiari
disease, and only 10 cases of hemangioblastomas with holochordial syringomyelia
have been described so far.
Case Report: A case of a 35-year-old patient with a two-month
history of cervicobrachialgia at the C7-C8 root level, previously preceded by
pain at the D1-D2 level, has been presented. Cervico-dorso-lumbar MRI revealed
a medullar tumor with an uptake mural nodule at the conus medullaris level
accompanied by an extensive syringomyelic cavity from C5 to L1 compatible with
medullary hemangioblastoma. The patient underwent surgery for tumor resection
with subsequent resolution of her painful symptoms. Syringomyelia is mainly
associated with Chiari malformation and also with inflammatory pathologies,
spinal attacks, trauma, and intramedullary tumors of the spinal cord.
Conclusion: The management of holochordal syringomyelia can be a
great challenge. It is important to emphasize that surgical treatment is aimed at
treating the origin of this syringomyelia and not the syringomyelia itself. It
is mandatory to rule out the existence of Chiari syndrome as a first
possibility, without forgetting the possibility of focal intramedullary spinal
tumors as a cause of syringomyelia.
Author
(s) Details
Alejandra
Arévalo-Sáenz
Department of Neurosurgery, Hospital Clinico San Carlos, Madrid,
Spain.
Borja
Ferrández-Pujante
Department of Neurosurgery, Hospital Clinico San Carlos, Madrid,
Spain.
Fernando
J. Rascón-Ramírez
Department of Neurosurgery, Hospital Clinico San Carlos, Madrid,
Spain.
Please see the book here:- https://doi.org/10.9734/bpi/mmrnp/v7/2275
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