Orbital Involvement in Linear Scleroderma: A Review of The Literature With an Unusual Case.
Jai Paris, Charlotte Mackenzie, Abdullah I Almater et al.
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Case in brief
A 29-year-old woman with right-sided linear scleroderma en coup de sabre presented with progressive vision loss. Examination showed an enlarged blind spot with global depression, 6 mm of enophthalmos, ptosis, circumferential optic disc edema, and an Adie’s pupil. MRI demonstrated severe orbital fat atrophy, enlarged extraocular muscles, and a contorted, enhancing optic nerve, without bone abnormalities or inflammation. She was managed conservatively with corticosteroids. The report’s literature review identified 26 cases of orbital involvement, commonly associated with enophthalmos and extraocular muscle atrophy.
What made this case unusual
Despite severe orbital fat atrophy, the extraocular muscles were enlarged rather than atrophic—a paradoxical imaging finding highlighted as an uncommon manifestation of orbital en coup de sabre.
Diagnostic / clinical pearl
Orbital en coup de sabre may involve the optic nerve and show extraocular muscle enlargement, not only the more familiar enophthalmos and muscle atrophy; correlate ophthalmic findings with orbital MRI.
Why it matters
The authors emphasize regular ophthalmic monitoring in patients with en coup de sabre because orbital disease can include vision-threatening manifestations.