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Cerebrospinal fluid leakage and Chiari I malformation with Gorham's disease of the skull base: A case report
Affiliations- Department of Neurosurgery, Kobe University Graduate School of Medicine, Kobe, Japan
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Abstract
Gorham's syndrome is a rare bone disorder characterized by massive osteolysis of unknown etiology. There are no reports of comorbidity involving cerebrospinal fluid (CSF) leakage and Chiari I malformation with Gorham's syndrome. Here, we report an unusual case of an acute presyrinx state complicated by bacterial meningitis due to CSF leakage and Chiari I malformation associated with Gorham's disease of the skull base.
Case presentationA 25-year-old woman with Chiari I malformation associated with Gorham's syndrome presented with aggressive paresthesia following bacterial meningitis. Axial magnetic resonance imaging (MRI) and computed tomography (CT) cisternography revealed CSF leakage in the right petrous apex. A presyrinx state was diagnosed based on the clinical symptoms and MRI findings. With resolution of the bacterial meningitis, the spinal edema and tonsillar ectopia also improved. Surgical repair of the CSF leakage was performed by an endoscopic endonasal transsphenoidal approach to prevent recurrence of meningitis. The postoperative course was uneventful.
ConclusionSkull base osteolysis in Gorham's syndrome may induce Chiari I malformation and CSF leakage. We should pay attention to acute progression of clinical symptoms because Gorham's syndrome may predispose to development of Chiari I malformation and may be complicated by CSF leakage.
Abstract
Gorham's syndrome is a rare bone disorder characterized by massive osteolysis of unknown etiology. There are no reports of comorbidity involving cerebrospinal fluid (CSF) leakage and Chiari I malformation with Gorham's syndrome. Here, we report an unusual case of an acute presyrinx state complicated by bacterial meningitis due to CSF leakage and Chiari I malformation associated with Gorham's disease of the skull base.
Case presentationA 25-year-old woman with Chiari I malformation associated with Gorham's syndrome presented with aggressive paresthesia following bacterial meningitis. Axial magnetic resonance imaging (MRI) and computed tomography (CT) cisternography revealed CSF leakage in the right petrous apex. A presyrinx state was diagnosed based on the clinical symptoms and MRI findings. With resolution of the bacterial meningitis, the spinal edema and tonsillar ectopia also improved. Surgical repair of the CSF leakage was performed by an endoscopic endonasal transsphenoidal approach to prevent recurrence of meningitis. The postoperative course was uneventful.
ConclusionSkull base osteolysis in Gorham's syndrome may induce Chiari I malformation and CSF leakage. We should pay attention to acute progression of clinical symptoms because Gorham's syndrome may predispose to development of Chiari I malformation and may be complicated by CSF leakage.
Keywords
Gorham' s syndrome, Meningitis, Cerebrospinal fluid leakage, Chiari malformation, Syringomyelia
About this articleTitle
Cerebrospinal fluid leakage and Chiari I malformation with Gorham's disease of the skull base: A case report
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
427-431
Page views
300
Article views/downloads
497
DOI
10.1016/j.pjnns.2017.06.007
Bibliographic record
Neurol Neurochir Pol 2017;51(5):427-431.
Keywords
Gorham's syndrome
Meningitis
Cerebrospinal fluid leakage
Chiari malformation
Syringomyelia
Authors
Hiroaki Nagashima
Katsu Mizukawa
Masaaki Taniguchi
Yusuke Yamamoto
Eiji Kohmura
