Orbital pseudotumor

Case contributed by Dr Dalia Ibrahim

Presentation

Bilateral proptosis and decreased visual acuity .

Patient Data

Age: 45 years
Gender: Female
MRI

Bilateral (more on the right side) intra and extra-conal soft tissue sheets of abnormal signal and enhancement eliciting dark signal on T2 WI. Also, the extra-ocular muscles appear involved. Bilateral proptosis is noted.

Diffuse pachymeningeal thickening and enhancement.

Based on radiological findings, inflammatory pseudotumor was considered.

CT scan of the brain

CT

The intra-orbital sheets are seen.

The diffuse pachymeningeal thickening showing calcific foci.

Photo

Biopsy revealed inflammatory pseudotumor.

CT scan of the chest,abdomen and pelvis

CT

The CT scan of the chest, abdomen, and pelvis appears unremarkable.

Case Discussion

In this case, the inflammatory changes involve the extra-ocular muscles, intra and extra-conal planes as well as the pachymeninges, with the typical dark signal on T2 WI, which raised the possibility of inflammatory pseudotumor.

Biopsy confirmed inflammatory pseudotumor.

CT scan of the chest, abdomen and pelvis were unremarkable. No evidence of sarcoid or Wegener.

Orbital pseudotumor is an idiopathic autoimmune inflammatory condition that most commonly involves the extraocular muscles. Less commonly there is inflammatory changes involving the uvea, sclera, lacrimal gland, and retrobulbar soft tissue.

It can appear as an infiltrative mass and extends outside of the orbit via superior or inferior orbital fissures. Extension into the cavernous sinus, meninges, and dura can occur.

The condition has been associated with other inflammatory and autoimmune conditions:

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