orbital pseudotumor.ppt

8/17/2019 Orbital Pseudotumor.ppt

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Leo Rinaldi


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Orbital pseudotumor is a swelling of the tissuesbehind the eye in an area called the orbit. The orbit isthe bony cavity in the skull where the eye sits. Itprotects the eyeball and the muscles and tissue that

surround it

Orbital pseudotumor is a benign intraorbital processconned to the orbit but extra orbital involvement canoccur.

The cause is unknown. It most commonly aectsyoung women! although it can still occur at any age.


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"irst described by #irch$%irscheld in &'()

*fter +raves, disease and lymphoproliferativedisorders! orbital pseudotumor is the -rd most

common ophthalmologic disease of the orbit andaccount for approximately $&&/ of all the orbitaltumors.


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0ain in eye $ may be severe Restricted eye movement 1ecreased vision

1ouble vision 2ye swelling 3proptosos4 Red eye 3rare4


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2tiology unknown but infection! autoimmune disorder! and

aberrant wound healings have been putforward as possibilities.

associated with infectious diseases such as5treptococcal pharyngitis! viral upperrespiratory infection and #orrelia

burgdorferi infection.


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5igns of pseudotumor can be seen when theeye is examined. Tests must be done to tellthe dierence between pseudotumor and acancerous tumor and eye problems that can

occur in people with thyroid disease.


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Tests may include7 8T scan of the head 9RI of the head

:ltrasound of the head 5kull ;$Ray #iopsy


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On 8T$scan and 9RI studies! pseudotumorsmay present with diuse orbital mass!uveoscleral thickening! contrastenhancement of Tenon,s potential space!

proptosis and optic nerve and extraocularmuscle enlargement 3"ig.& < =4.


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Figured 1. 2xternal photograph of a >($year$old woman withhistory ofseveral episodes ofrecurrent bilateral chemosis! restriction ofextraocular motility and vision 3*4. 8T$scan 3axial and coronal4 revealedbilateral inltrative processes 3# and 84.Treatment with a course ofcorticosteroids and radiationtherapy resulted in resolution ofher

symptoms 314.


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Figured 2. The orbital 9RI reveals bilateral advanced proptosis with diuseinltration of orbital fat! obliteration of optic nerves! extraocular muscles with xation of intraorbital structures and enlarged lacrimal glands. 2xtraocularmuscles show diuse enlargement 3both tendons as well muscle bundlesenlarged in a tubular conguration4! with involvement of perineural opticsheath


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* biopsy 3called an orbitotomy4 iscommonly performed to conrm thediagnosis of orbital pseudotumor and ruleout other diseases that cause orbitalin?ammation.


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Figured 3. * biopsy specimen taken from right supra orbitalregion of patient in gure &3*4! reveled granulo$matousin?ammatory process 3#4.


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9ild cases may go away without treatment.9ore severe cases will usually respond totreatment with corticosteroids. @ery severecases may develop damaging pressure onthe eye! and reAuire surgical movement ofthe bones of the orbit to relieve pressure on

the eyeball.


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starting dosages of 0rednisone &.( to =.(mgBkgBday are adeAuate. Chenimprovement is noted! dosages should becontinued with a slow tapering.


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Other therapies with promise includecytotoxic agents! 38yclophosphamide and8hlorambucil4! immunosuppressants39ethotrexater! 8yclosporine! *Dathioprine4!

I@ immunoglobulins! TE"$alpha inhibitor!monoclonal antibody 3In?eximab and*dalimumab4 and 9ycophenolate 9oftilwhich inhibit denovo purine synthesis and

prevent # < T lymphocyte replication.


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Figured 4.1. 0re treatment F 8ontrast enhanced 8T scan of orbit axial viewshowing anterior displacement of the left globe 3arrow4 and non enhancing sof tissue mass in the left orbit. 3double arrow4


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Figured 4.2 8oronal view of sinuses Fmaxillary sinus showinghaDiness and hypertrophy of the mucosa. 3arrow4


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Figured 4.3. *fter > weeks of starting steroid therapy contrastenhanced 8T 5can of orbit axial view showing partial resolution of thesoft tissue mass. 3arrow4


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Figured 4.4. 0ost treatment F after &( months F contrast enhanced 8T scanof orbit$ axial view showing complete resolution of proptosis and soft tissuemass. 3arrow4


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Figured 5. Response of the patient after corticosteriodtreatment with decreased proptosis! decreased lid edema and

conGunctival inGection after eight weeks of treatment

Before Therapy After Therapy


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9ost cases are mild and do well. 5everecases may be resistant to treatment andvisual loss may occur. Orbital pseudotumorusually involves only one eye.

orbital pseudotumor.ppt

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