Posterior fossa arachnoid cyst, tonsillar herniation, and syringomyelia in trichorhinophalangeal syndrome Type I.
J Neurosurg
; 109(4): 746-50, 2008 Oct.
Article
en En
| MEDLINE
| ID: mdl-18826365
ABSTRACT
The authors report the case of a patient with Chiari malformation Type I (CM-I) and syringomyelia probably caused by a retrocerebellar arachnoid cyst. The patient's phenotype corresponded to trichorhinophalangeal syndrome Type I. The authors attributed the origin of both the retrocerebellar cyst and the abnormal posterior fossa to endochondral ossification anomalies that occur in this syndrome. The patient's spinal pain was most likely a result of the combination of CM-I and syringomyelia. To the best of the authors' knowledge, this is the first report on the association of CM-I and syringomyelia with a retrocerebellar arachnoid cyst occurring in a patient with trichorhinophalangeal syndrome Type I. The authors discuss the pathogenetic mechanisms involved in the production of tonsillar descent and syringomyelia in this patient, and review the current literature on related conditions that can result in this association.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Malformación de Arnold-Chiari
/
Quistes Aracnoideos
/
Fosa Craneal Posterior
/
Meningocele
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
J Neurosurg
Año:
2008
Tipo del documento:
Article
País de afiliación:
España