Leo Rinaldi

Orbital pseudotumor is a swelling of the tissues

behind the eye in an area called the orbit. The orbit is
the bony cavity in the skull where the eye sits. It
protects the eyeball and the muscles and tissue that
surround it

Orbital pseudotumor is a benign intraorbital process

confined to the orbit but extra orbital involvement can
occur.

The cause is unknown. It most commonly affects

young women, although it can still occur at any age.

First described by Birch-Hirschfield in 1905

After Graves' disease and lymphoproliferative

disorders, orbital pseudotumor is the 3rd most
common ophthalmologic disease of the orbit and
account for approximately 8-11% of all the orbital
tumors.

Pain in eye - may be severe

Restricted eye movement
Decreased vision
Double vision
Eye swelling (proptosos)
Red eye (rare)

Etiology unknown
but infection, autoimmune disorder, and
aberrant wound healings have been put
forward as possibilities.
associated with infectious diseases such as
Streptococcal pharyngitis, viral upper
respiratory infection and Borrelia
burgdorferi infection.

Signs of pseudotumor can be seen when the

eye is examined. Tests must be done to tell
the difference between pseudotumor and a
cancerous tumor and eye problems that can
occur in people with thyroid disease.

Tests may include:

CT scan of the head
MRI of the head
Ultrasound of the head
Skull X-Ray
Biopsy

On CT-scan and MRI studies, pseudotumors

may present with diffuse orbital mass,
uveoscleral thickening, contrast
enhancement of Tenon's potential space,
proptosis and optic nerve and extraocular
muscle enlargement (Fig.1 & 2).

Figured 1. External photograph of a 60-year-old woman withhistory of

several episodes ofrecurrent bilateral chemosis, restriction of
extraocular motility and vision (A). CT-scan (axial and coronal) revealed
bilateral infiltrative processes (B and C).Treatment with a course of
corticosteroids and radiationtherapy resulted in resolution ofher
symptoms (D).

Figured 2. The orbital MRI reveals bilateral advanced proptosis with diffuse
infiltration of orbital fat, obliteration of optic nerves, extraocular muscles with
fi xation of intraorbital structures and enlarged lacrimal glands. Extraocular
muscles show diffuse enlargement (both tendons as well muscle bundles
enlarged in a tubular configuration), with involvement of perineural optic
sheath

A biopsy (called an orbitotomy) is

commonly performed to confirm the
diagnosis of orbital pseudotumor and rule
out other diseases that cause orbital
inflammation.

Figured 3. A biopsy specimen taken from right supra orbital

region of patient in figure 1(A), reveled granulo-matous
inflammatory process (B).

Mild cases may go away without treatment.

More severe cases will usually respond to
treatment with corticosteroids. Very severe
cases may develop damaging pressure on
the eye, and require surgical movement of
the bones of the orbit to relieve pressure on
the eyeball.

starting dosages of Prednisone 1.0 to 2.0

mg/kg/day are adequate. When
improvement is noted, dosages should be
continued with a slow tapering.

Other therapies with promise include

cytotoxic agents, (Cyclophosphamide and
Chlorambucil), immunosuppressants
(Methotrexater, Cyclosporine, Azathioprine),
IV immunoglobulins, TNF-alpha inhibitor,
monoclonal antibody (Infleximab and
Adalimumab) and Mycophenolate Moftil
which inhibit denovo purine synthesis and
prevent B & T lymphocyte replication.

Figured 4.1. Pre treatment Contrast enhanced CT scan of orbit axial view
showing anterior displacement of the left globe (arrow) and non enhancing soft
tissue mass in the left orbit. (double arrow)

Figured 4.2 Coronal view of sinuses maxillary sinus showing

haziness and hypertrophy of the mucosa. (arrow)

Figured 4.3. After 6 weeks of starting steroid therapy contrast

enhanced CT Scan of orbit axial view showing partial resolution of the
soft tissue mass. (arrow)

Figured 4.4. Post treatment after 10 months contrast enhanced CT scan

of orbit- axial view showing complete resolution of proptosis and soft tissue
mass. (arrow)

Before Therapy

After Therapy

Figured 5. Response of the patient after corticosteriod

treatment with decreased proptosis, decreased lid edema and
conjunctival injection after eight weeks of treatment

Most cases are mild and do well. Severe

cases may be resistant to treatment and
visual loss may occur. Orbital pseudotumor
usually involves only one eye.