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17-05-2024 | Neurofibromatosis Type 1 | Case Report

Unruptured giant lateral thoracic meningocele: extremely rare cause of cerebrospinal fluid (CSF) hypotension in neurofibromatosis type 1

Authors: Venkatram Krishnan, Abhilasha Rana, Surjith Vattoth

Published in: Neuroradiology | Issue 7/2024

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Abstract

Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral thoracic meningoceles can develop in NF1 and progressively enlarge due to cerebrospinal fluid (CSF) pulsations. Large meningoceles can cause compressive symptoms in the thorax. We are reporting a case of a NF1 presenting with acute onset respiratory distress, who also had chronic orthostatic headaches. CT chest showed unruptured enlarging bilateral lateral thoracic meningoceles causing lung compression. MRI of the brain and spine showed features of CSF hypotension, explaining the headaches. CSF hypotension with unruptured meningoceles is extremely rare. Management of the condition is challenging since surgical removal is prone to complications due to underlying mesodermal abnormalities. Cystoperitoneal shunting to relieve lung compression may worsen CSF hypotension. A shunt with a programmable valve allowed controlled drainage and successfully relieved lung compression without worsening of orthostatic headaches in our case.
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Metadata
Title
Unruptured giant lateral thoracic meningocele: extremely rare cause of cerebrospinal fluid (CSF) hypotension in neurofibromatosis type 1
Authors
Venkatram Krishnan
Abhilasha Rana
Surjith Vattoth
Publication date
17-05-2024
Publisher
Springer Berlin Heidelberg
Published in
Neuroradiology / Issue 7/2024
Print ISSN: 0028-3940
Electronic ISSN: 1432-1920
DOI
https://doi.org/10.1007/s00234-024-03381-4

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