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Vol. 18. Issue 3.
Pages 227-231 (January 2007)
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Vol. 18. Issue 3.
Pages 227-231 (January 2007)
Regression of syringomyelia and tonsillar herniation after posterior fossa arachnoid cyst excision. Case report and literature review
Resolución de siringomielia y herniación de las amígdalas cerebelosas tras la escisión de un quiste aracnoideo de fosa posterior. Descripción de un caso y revisión de la literatura
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J.F. Martínez-Lage
, M.J. Almagro, J. Ros de San Pedro, A. Ruiz-Espejo, M. Felipe-Murcia
Servicio Regional de Neurocirugía. Hospital Universitario Virgen de la Arrixaca. El Palmar. Murcia
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Summary
Background

Some reports have documented posterior fossa cysts resulting in syringomyelic obstruction of cerebrospinal fluid (CSF) flow caused by cyst displacement within the foramen magnum. Rarely the syringomyelia is caused by acquired Chiari malformation due to a retrocerebellar arachnoid cyst.

Objective

To report the case of a 38-year-old man with hydrocephalus and syringomyelia, who was found to have a Chiari malformation secondary to a posterior fossa arachnoid cyst. After endoscopic third ventriculostomy, the patient was submitted to foramen magnum decompression and arachnoid cyst removal that were followed by resolution of both the Chiari malformation and the syringomyelia.

Discussion

In most published cases the syringomyelia has been attributed to obstruction of CSF flow at the foramen magnum by the arachnoid cyst itself. There is only one previous report of a posterior fossa arachnoid cyst producing tonsillar descent and syringomyelia.

Conclusions

Posterior fossa arachnoid cysts can result in acquired Chiari malformation and syringomyelia. In our view, the management of these patients should be directed at decompressing the foramen magnum and include the removal of the walls of the coexistent arachnoid cyst as it seems to be the crucial factor that accounts for the development of the syringomyelia that these patients present.

Keywords:
Acquired Chiari malformation
Syringomyelia
Arachnoid cyst
Foramen magnum decompression
Endoscopic third ventriculostomy
Resumen
Antecedentes

Algunos trabajos han descrito la existencia de quistes aracnoideos de fosa posterior que producían siringomielia al obstruir la circulación de liquido cefalorraquídeo (LCR) por bloqueo del agujero magno ocasionado por el propio quiste. Rara vez la siringomielia asociada a quiste aracnoideo de fosa posterior es producida por malformación de Chiari adquirida.

Objetivos

Publicar el caso de un hombre de 38 años con hidrocefalia y siringomielia, en el que se encontró descenso de las amígdalas cerebelosas por la presión ejercida por un quiste aracnoideo retrocerebeloso. Tras tratar primero la hidrocefalia mediante ventriculostomía neuroendoscópica, el paciente fue operado de descompresión del agujero magno y extirpación de las membranas del quiste que condujeron a la resolución tanto del Chiari como de la siringomielia.

Discusión

En la mayoría de los casos publicados la siringomielia se debía a la obstrucción de LCR en el agujero magno ocasionada por el propio quiste aracnoideo. Hemos encontrado solamente un caso previo en el que el quiste de fosa posterior era causante del descenso de las amígdalas cerebelosas y éste, a su vez, producía la siringomielia.

Conclusiones

. Los quistes aracnoideos de fosa posterior pueden producir descenso de las amígdalas y siringomielia. En nuestra opinión, el manejo de estos pacientes debe de encaminarse no sólo a descomprimir el agujero magno, sino también a comunicar las paredes del quiste ya que éste parece ser el principal responsable del complejo cuadro de siringomielia en estos casos.

Palabras clave:
Malformación de Chiari adquirida
Siringomielia
Quiste aracnoideo
Descompresion del agujero magno
Ventriculostomía neuroendoscópica

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