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Published on: September 20, 2018
Erdheim-Chester disease: two cases of orbital involvement
T G Sheidow1, D A Nicolle, J G Heathcote
1Department of Ophthalmology, University of Western Ontario, London, Canada. tgsheido@julian.uwo.ca
Eye (London, England)
|October 21, 2000
Summary
Erdheim-Chester disease (ECD) can affect the eyes, presenting varied symptoms like orbital pseudotumors and exophthalmos. Treatment responses, including cladribine and radiation, highlight the complex and unpredictable nature of ECD
Area of Science:
- Ophthalmology
- Pathology
- Oncology
Background:
- Erdheim-Chester disease (ECD) is a rare fibro-inflammatory condition characterized by lipid-laden histiocytes.
- Ophthalmic involvement in ECD is infrequently reported, with only 22 cases documented previously.
- This study focuses on the ophthalmic manifestations and treatment outcomes in two new ECD cases.
Observation:
- The first patient presented with diabetes insipidus, later developing orbital pseudotumors and retroperitoneal fibrosis.
- The second patient experienced exophthalmos and diplopia, indicative of retro-orbital disease.
- Both patients underwent different treatment modalities, including cladribine and radiation therapy.
Findings:
- The first patient treated with cladribine experienced sudden bilateral blindness.
- The second patient developed radiation retinopathy following radiation therapy for retro-orbital ECD.
- These cases illustrate the diverse clinical presentations and potential complications of ophthalmic ECD.
Implications:
- Ophthalmic manifestations of ECD can be severe and lead to significant visual impairment.
- Treatment strategies for ECD-related eye conditions require careful consideration due to potential adverse effects.
- Increased awareness and further research are needed to improve the management of ocular ECD.
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