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

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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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Acknowledgements

We are grateful to Dr. Rudy Van Hemert, division chief of Neuroradiology, University of Arkansas for Medical Sciences, for his constant support, guidance and encouragement.

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The authors have no relevant financial or non-financial interests to disclose.

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Authors and Affiliations

  1. University of Arkansas for Medical Sciences, Little Rock, AR, USA

    Venkatram Krishnan & Abhilasha Rana

  2. Rush University Medical Center, Chicago, IL, USA

    Surjith Vattoth

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  1. Venkatram Krishnan
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  2. Abhilasha Rana
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  3. Surjith Vattoth
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Correspondence to Venkatram Krishnan.

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Waived by the Institutional Ethics Committee as the study involved retrospective reporting of a case and did not involve any interventions on human or animal subjects.

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Krishnan, V., Rana, A. & Vattoth, S. Unruptured giant lateral thoracic meningocele: extremely rare cause of cerebrospinal fluid (CSF) hypotension in neurofibromatosis type 1. Neuroradiology 66, 1235–1238 (2024). https://doi.org/10.1007/s00234-024-03381-4

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  • DOI: https://doi.org/10.1007/s00234-024-03381-4

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