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Answers: 2008 Series -  July 1, 2008 Lecture 27 of 53  NEXT»

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This 5-year-old boy was noted by his parents to have bulging eyes beginning two years ago and this has been gradually increasing. In the beginning he had fever and lymphadenopathy. He was treated with steroids and antibiotics. Biopsy of the enlarged right lacrimal gland was said to be inconclusive. Both lacrimal glands appear enlarged clinically. His refraction is OD and OS -0.50 and vision is best corrected at OD 20/60 and OS no light perception. Both pupils have a direct pupillary response and there is no apparent optic atrophy.

1. The severe vision loss in the left eye is most likely due to:

d -- compression of the optic nerve

The CT shows great enlargement of the extraocular muscles in the muscle cone that could cause compression of the optic nerve and subsequent vision loss.

2. What is the likely diagnosis?

c -- bilateral orbital pseudotumor

The most likely diagnosis is bilateral orbital psuedotumor.

3. What would you do now?

a -- incisional biopsy of the lacrimal gland

The diagnosis could be confirmed with biopsy of the lacrimal gland.

This case was seen by Cyber-Sight mentor Robert D. Yee, M.D., an expert neuro-ophthalmologist, and Professor of Ophthalmology and Chairman of the Department of Ophthalmology, Indiana University School of Medicine.  His comments were the following:

"Your diagnosis of bilateral inflammatory pseudotumor of the orbit is a good choice. It apparently is affecting the lacrimal glands and extraocular muscles. It is curious that the left eye has no light perception, but there is a direct pupillary response to light in that eye and there is no optic atrophy.

An incisional biopsy of a lacrimal gland would be a good idea. However, biopsy results do not always reliably differentiate between inflammatory lymphoproliferative disorders and neoplastic ones, such as lymphoma.

If the biopsy is consistent with inflammatory orbital pseudotumor, a trial of systemic corticosteroids would be indicated. If there is no light perception in the left eye, but no optic atrophy, the visual loss might be from compression. Decompression with steroids, radiotherapy or surgical decompression (orbital wall fenestrations) might be considered.

It would be good to get an opinion from our oculoplastic consultants too."

 


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