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Gorham Stout disease of the temporal bone with cerebrospinal fluid leak

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Abstract

Gorham Stout disease (GSD) is a rare disease characterized by the proliferation of endothelial lined vessels and replacement of bone by fibrous tissue. The main imaging features are progressive osteolysis and cortical resorption. Temporal bone involvement is rare but presents as a destructive bone lesion that may be misinterpreted as more common lytic processes in the pediatric population, such as infection or Langerhans cell histiocytosis. GSD of the temporal bone is associated with cerebrospinal fluid (CSF) leaks, may present with otorrhea, and can mimic other causes of ear drainage. Here, we report the clinical course, imaging features, and outcomes of a 3-year-old girl with GSD of the temporal bone presenting with CSF leak initially attributed to infection.

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Authors and Affiliations

  1. Department of Medical Imaging, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Pascale Aouad & Maura E. Ryan

  2. Division of Otolaryngology, Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

    Nancy M. Young

  3. Department of Otolaryngology - Head and Neck Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Nancy M. Young

  4. Department of Surgery, Division of Pediatric Neurosurgery, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Amanda M. Saratsis

  5. Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Amanda M. Saratsis

  6. Department of Biochemistry and Molecular Genetics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Amanda M. Saratsis

  7. Department of Pathology & Lab Medicine/Anatomic Pathology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Meredith A. Reynolds

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  1. Pascale Aouad
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  2. Nancy M. Young
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Correspondence to Pascale Aouad.

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Aouad, P., Young, N.M., Saratsis, A.M. et al. Gorham Stout disease of the temporal bone with cerebrospinal fluid leak. Childs Nerv Syst 38, 455–460 (2022). https://doi.org/10.1007/s00381-021-05245-1

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  • DOI: https://doi.org/10.1007/s00381-021-05245-1

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