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BMC Neurol ; 23(1): 59, 2023 Feb 03.
Artículo en Inglés | MEDLINE | ID: mdl-36737721

RESUMEN

BACKGROUND: Gorham-Stout disease (GSD) is a very rare disorder characterized by massive osteolysis of poorly understood aetiology. The association between GSD involving the skull base and cerebrospinal fluid (CSF) leakage has been reported in the literature. However, few cases of CSF leakage and Chiari-like tonsillar herniation in GSD involving the spine have been reported. CASE PRESENTATION: We present the case of a 20-year-old man with GSD involving the thoracic and lumbar spine, which caused CSF leakage and Chiari-like tonsillar herniation. The patient underwent four spinal surgeries for osteolytic lesions of the spine over a 10-year period. Here, we discuss the possible aetiology of the development of CSF leakage. Epidural blood patch (EBP) was performed at the T11-T12 level to repair the CSF leakage. After EBP treatment, rebound intracranial hypertension (RIH) developed, and tonsillar herniation disappeared 2 months later. CONCLUSIONS: GSD involving the spine with CSF leakage and Chiari-like tonsillar herniation is relatively rare. For patients who have undergone multiple spinal surgeries, minimally invasive treatment is an alternative treatment for CSF leakage. EBP can repair CSF leakage secondary to GSD and improve chronic brain sagging, with reversibility of Chiari-like malformations.


Asunto(s)
Malformación de Arnold-Chiari , Osteólisis Esencial , Masculino , Humanos , Adulto Joven , Adulto , Osteólisis Esencial/complicaciones , Osteólisis Esencial/cirugía , Osteólisis Esencial/patología , Encefalocele/complicaciones , Encefalocele/cirugía , Encefalocele/patología , Pérdida de Líquido Cefalorraquídeo/diagnóstico por imagen , Pérdida de Líquido Cefalorraquídeo/etiología , Pérdida de Líquido Cefalorraquídeo/cirugía , Encéfalo/patología , Malformación de Arnold-Chiari/complicaciones , Malformación de Arnold-Chiari/cirugía
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