Presentation
2 month history of headaches. MRI brain reported as normal. Represents with left eye pain and proptosis. O/E Bilateral papilledema. Palmar erythema.
Patient Data
In the interim a history of anti-phospholipid syndrome is established. Ds DNA antibiodies elevated.
Marked thickening of the left lateral rectus muscle, largely in the belly, which is displacing and compressing the proximal aspect of the intra-conal optic nerve. In addition similar changes in the left superior rectus muscle.
Associated enhancement and inflammatory change in the intraconal fat. This is contributing to impingement of the optic nerve at the level of the optic apex.
Mild enlargement and enhancement on the left superolateral lacrimal gland with associated inflammatory changes extending into the subcutaneous fat.
Normal right orbit.
Normal intracranial appearances.
Case Discussion
Orbital pseudotumor is an idiopathic inflammatory condition that most commonly involves the extraocular muscles. Less commonly there is inflammatory change involving the uvea, sclera, lacrimal gland and retrobulbar soft tissues.
The condition has been associated with many wider inflammatory and autoimmune conditions including systemic lupus erythematosus (SLE).
It may involve an isolated or multiple extra-ocular muscles. Additional inflammation can be seen in surrounding tissues, including the lacrimal gland ( as in this case ). It can appear as an infiltrative mass and that extends outside of the orbit via superior or inferior orbital fissures.
The patient made a prompt and good response to steriods. Following up imaging is intended.
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