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Posterior choroidal involvement in Letterer-Siwe disease
Journal of Pediatric Ophthalmology and Strabismus
|March 1, 1978
Summary
A child in remission for Letterer-Siwe disease developed decreased vision due to a choroidal mass. Imaging revealed a vascularized lesion causing retinal detachment and optic nerve involvement.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Medical Imaging
Background:
- Letterer-Siwe disease, a rare form of Langerhans cell histiocytosis, can have systemic manifestations.
- Ocular involvement in Letterer-Siwe disease is uncommon but can lead to significant visual impairment.
Observation:
- A five-year-old child with Letterer-Siwe disease in remission presented with unilateral decreased vision.
- Ophthalmic examination revealed a choroidal mass causing secondary retinal detachment with cystic degeneration.
Findings:
- Echography demonstrated a vascularized, disciform-like mass potentially involving the optic nerve.
- Fluorescein angiography confirmed a highly vascular lesion with optic disc edema, retinal pigment epithelium destruction, and cystoid macular edema.
Implications:
- This case highlights the potential for late ocular complications in Letterer-Siwe disease survivors.
- Prompt diagnosis and management of such choroidal masses are crucial to preserve vision.