Erdheim–Chester Disease With Eyelid and Orbital Involvement: A Review of Treatment Modalities at One Institution From 2014 to 2022

Author:

Sharma Meghan1,Stevens Shanlee M.2,Maeng Michelle M.3,Nagornaya Natalya1,Bhatia Rita G.1,Wester Sara T.1

Affiliation:

1. Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida, U.S.A.

2. Department of Ophthalmology, New York University Grossman School of Medicine, New York City, New York, U.S.A.

3. Department of Ophthalmology & Visual Science, Yale School of Medicine, New Haven, Connecticut, U.S.A.

Abstract

Purpose: To review all cases of Erdheim–Chester disease (ECD) with orbital involvement treated at Bascom Palmer Eye Institute in Miami, Florida from 2014 to 2022 and compare presentations, treatment modalities, and outcomes. Methods: A retrospective chart review of all patients diagnosed with ECD who presented to Bascom Palmer Eye Institute from 2014 to 2022 was performed. Data collected included demographics, pretreatment history and ophthalmic examination, pathology report, treatment, subsequent examination, and relevant laboratory results. Histopathology, treatments, and outcomes were reviewed and compared between patients. Results: Four cases were included. Primary treatments included vemurafenib (n = 2), cobimetinib (n = 1), and prednisone (n = 1). All patients demonstrated improvement of ophthalmic symptoms. Vemurafenib was the only medical treatment that was tolerated well and resulted in significant improvement in proptosis despite some reported dry eye; all other medications were discontinued due to intolerable side effects. Conclusions: BRAF inhibitors such as vemurafenib have been used as novel therapy in the treatment of ECD. Vemurafenib demonstrated its utility in reducing proptosis in ECD patients at one ophthalmic institution. Vemurafenib may be a favorable treatment option for BRAF-positive ECD patients presenting with orbital disease.

Publisher

Ovid Technologies (Wolters Kluwer Health)

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