open access

Vol 51, No 5 (2017)
Case reports
Submitted: 2016-09-16
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Cerebrospinal fluid leakage and Chiari I malformation with Gorham's disease of the skull base: A case report

Hiroaki Nagashima, Katsu Mizukawa, Masaaki Taniguchi, Yusuke Yamamoto, Eiji Kohmura
DOI: 10.1016/j.pjnns.2017.06.007
·
Neurol Neurochir Pol 2017;51(5):427-431.

open access

Vol 51, No 5 (2017)
Case reports
Submitted: 2016-09-16

Abstract

Background

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 presentation

A 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.

Conclusion

Skull 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

Background

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 presentation

A 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.

Conclusion

Skull 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.

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Keywords

Gorham' s syndrome, Meningitis, Cerebrospinal fluid leakage, Chiari malformation, Syringomyelia

About this article
Title

Cerebrospinal fluid leakage and Chiari I malformation with Gorham's disease of the skull base: A case report

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 51, No 5 (2017)

Pages

427-431

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

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