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; Lilangi S. Ediriwickrema; Neil R. Miller
Affiliation Division of Neuro-Ophthalmology, Department of Ophthalmology, Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland.
Subject Gorham-Stout Disease; Acute Unilateral Proptosis
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.
OCR Text Show
Publisher Lippincott, Williams & Wilkins
Date 2018-03
Type Text
Source Journal of Neuro-Ophthalmology, December 2018, Volume 38, Issue 1
Language eng
Rights Management © North American Neuro-Ophthalmology Society
Publication Type Journal Article
ARK ark:/87278/s68m1p70
Setname ehsl_novel_jno
Date Created 2019-03-11
Date Modified 2019-03-11
ID 1404051
Reference URL https://collections.lib.utah.edu/ark:/87278/s68m1p70
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