Gorham-Stout Disease Presenting as Acute Unilateral Proptosis

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Title Gorham-Stout Disease Presenting as Acute Unilateral Proptosis
Creator Inna G. Stroh, MD, PhD, Lilangi S. Ediriwickrema, MD, Neil R. Miller, MD
Affiliation Division of Neuro-Ophthalmology, Department of Ophthalmology, Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland
Abstract Gorham-Stout disease (GSD) is a rare angiomatous disorder characterized by osteolysis. We report the case of a 66-year-old woman who developed sudden left eye proptosis and facial pain. Neuroimaging demonstrated a destructive mass involving the roof and lateral wall of the left orbit with adjacent abnormal orbital soft tissue. An initial biopsy of the soft tissue was nondiagnostic; however, transcranial biopsy of the orbital roof revealed an intraosseous cavernous angioma, with infiltration of orbital fat by angiomatous tissue. Despite resection of the orbital roof and lateral wall, the orbital lesion continued to expand, leading to signs of a compressive optic neuropathy. The patient then reported severe back pain, prompting imaging that demonstrated disseminated bony involvement of the axial skeleton, eventually leading to the diagnosis of GSD. The patient was treated with a bisphosphonate and a vascular endothelial growth factor inhibitor with stabilization of disease.
Subject Gorham-Stout Disease; Acute Unilateral Proptosis
OCR Text Show
Date 2018-03
Language eng
Format application/pdf
Type Text
Publication Type Journal Article
Source Journal of Neuro-Ophthalmology, December 2018, Volume 38, Issue 1
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890
Rights Management © North American Neuro-Ophthalmology Society
ARK ark:/87278/s68m1p70
Setname ehsl_novel_jno
ID 1404051
Reference URL https://collections.lib.utah.edu/ark:/87278/s68m1p70
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