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Non-Infective Inflammatory disease
Idiopathic orbital inflammatory disease
Idiopathic orbital inflammatory disease (IOID), previously referred to as orbital pseudotumour, is
an uncommon disorder characterized by non-neoplastic, non-infective, space-occupying orbital
lesions. The inflammatory process may involve any or all of the orbital soft tissues, resulting in,
for example, myositis, dacryoadenitis, optic perineuritis or scleritis. Histopathological analysis
reveals pleomorphic inflammatory cellular infiltration followed by reactive fibrosis, but has thus
far shown no correlation between clinicopathological features and the subsequent course of the
disease. Unilateral disease is the rule in adults, although in children bilateral involvement may
occur. Simultaneous orbital and sinus involvement is a rare distinct entity.
Diagnosis
1 Presentation is with acute periorbital redness, swelling and pain (Fig. 3.16A).
2 Signs
Congestive proptosis and ophthalmoplegia may occur.
Optic nerve dysfunction, particularly if the inflammation involves the posterior
orbit.
3 CT shows ill-defined orbital opacification and loss of definition of contents (Fig. 3.16B
and C).
4 Course. This follows one of the following patterns:
Spontaneous remission after a few weeks without sequelae.
Intermittent episodes of activity with eventual remission.
Severe prolonged inflammation eventually leading to progressive fibrosis of orbital
tissues, resulting in a frozen orbit characterized by ophthalmoplegia, which may be
associated with ptosis and visual impairment caused by optic nerve involvement.

Fig. 3.16 (A) Left idiopathic orbital inflammatory disease. (B) CT axial view shows ill-defined
orbital opacification; (C) coronal view
(Courtesy of R Bates fig. A; A Pearson figs B and C)

Treatment
1 Observation, for relatively mild disease, in anticipation of spontaneous remission.

Biopsy is generally required in persistent cases to confirm the diagnosis and rule out
neoplasia.
3 NSAIDs are often effective and may precede steroid therapy.
4 Systemic steroids should be administered only after the diagnosis has been confirmed, as
they may mask other pathology such as infection and Wegener granulomatosis. Oral
prednisolone, initially 6080 mg/day, is later tapered and discontinued, depending on
clinical response, although it may need to be reintroduced in the event of recurrence.
5 Radiotherapy may be considered if there has been no improvement after 2 weeks of
adequate steroid therapy. Even a low dose treatment (i.e. 10 Gy) may produce remission.
6 Antimetabolites such as methotrexate or mycophenolate mofetil may be necessary in the
context of resistance to both steroids and radiotherapy.
7 Systemic infliximab, a tumour necrosis factor inhibitor, may be effective in recurrent or
recalcitrant cases that have failed to respond to conventional therapy.
Differential diagnosis
1 Bacterial orbital cellulitis should be considered when onset is acute and the anterior
orbital tissues are markedly inflamed. A trial of systemic antibiotics may be necessary
before the correct diagnosis becomes apparent.
2 Severe acute TED shares many features with IOID, but is commonly bilateral while IOID
is usually unilateral.
3 Systemic disorders such as Wegener granulomatosis, polyarteritis nodosa and
Waldenstrm macroglobulinaemia may manifest orbital involvement similar to IOID.
4 Malignant orbital tumours, particularly metastatic.
5 Ruptured dermoid cyst may evoke a secondary painful granulomatous inflammatory
reaction.
Orbital myositis
Orbital myositis is an idiopathic, non-specific inflammation of one or more extraocular muscles
and is considered a subtype of IOID.
1
2
3

4
5
6

Histology shows a chronic inflammatory cellular infiltrate in relation to muscle fibres

(Fig. 3.17A).
Presentation is usually in early adult life with acute pain, exacerbated by eye movement,
and diplopia.
Signs
Lid oedema, ptosis and chemosis.
Pain and diplopia associated with eye movements.
Vascular injection over the involved muscle (Fig. 3.17B).
In chronic cases the affected muscle may become fibrosed, with permanent
restrictive myopathy.
CT shows enlargement of the affected muscles (Fig. 3.17C), with or without involvement
of the tendons of insertion.
Differential diagnosis includes orbital cellulitis, TED and TolosaHunt syndrome (see
below).
Course

Acute non-recurrent involvement which resolves spontaneously within 6 weeks.

Chronic disease characterized by either a single episode persisting for longer than 2
months (often for years) or recurrent attacks.
Treatment is aimed at relieving discomfort and dysfunction, shortening the course and
preventing recurrences.
a NSAIDs may be adequate in mild disease.
b Systemic steroids are generally required and usually produce dramatic
improvement, although recurrences occur in 50% of cases.
c Radiotherapy is also effective, particularly in limiting recurrence.

Fig. 3.17 Orbital myositis. (A) Histology shows a chronic inflammatory cellular infiltrate in
relation to muscle fibres; (B) vascular injection over the insertion of the right medial rectus; (C)
coronal CT shows enlargement of the right medial rectus
(Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, ButterworthHeinemann 2001 fig. A; J Nerad, K Carter and M Alford, from Oculoplastic and
Reconstructive Surgery, in Rapid Diagnosis in Ophthalmology Mosby 2008 figs B and C)

Acute dacryoadenitis
Lacrimal gland involvement occurs in about 25% of patients with IOID. More commonly,
dacryoadenitis occurs in isolation, resolves spontaneously and does not require treatment.
Occasionally it may be caused by mumps, mononucleosis, and rarely bacteria.
1
2

3
4

Presentation is with acute discomfort in the region of the lacrimal gland.

Signs
Swelling of the lateral aspect of the eyelid giving rise to a characteristic S-shaped
ptosis and slight downward and inward dystopia (Fig. 3.18A).
Tenderness over the lacrimal gland fossa.
Injection of the palpebral portion of the lacrimal gland and adjacent conjunctiva
(Fig. 3.18B).
Lacrimal secretion may be reduced.
CT shows enlargement of the gland and involvement of adjacent tissues (Fig. 3.18C).
Differential diagnosis
a Ruptured dermoid cyst may cause localized inflammation in the region of the
lacrimal gland.
b Malignant lacrimal gland tumours may cause pain but the onset is not usually
acute.

Fig. 3.18 Left acute dacryoadenitis. (A) Swelling on the lateral aspect of the eyelid and an Sshaped ptosis; (B) injection of the palpebral portion of the lacrimal gland and adjacent
conjunctiva; (C) axial CT shows enlargement of the gland and opacification of adjacent tissues
(Courtesy of R Bates fig. B; A Pearson fig. C)

TolosaHunt syndrome
TolosaHunt syndrome is a diagnosis of exclusion. It is a rare idiopathic condition caused by
non-specific granulomatous inflammation of the cavernous sinus, superior orbital fissure and/or
orbital apex. The clinical course is characterized by remissions and recurrences.
1
2

Presentation is with diplopia associated with ipsilateral periorbital or hemicranial pain.

Signs
Proptosis, if present, is usually mild.
Ocular motor nerve palsies often with involvement of the pupil.
Sensory loss along the distribution of the first and second divisions of the trigeminal
nerve.
3 Treatment is with systemic steroids.
Wegener granulomatosis
Wegener granulomatosis may involve the orbit, often bilaterally, usually by contiguous spread
from the paranasal sinuses or nasopharynx. Primary orbital involvement is less common. The
possibility of Wegener granulomatosis should be considered in any patient with bilateral orbital
inflammation, particularly if associated with sinus pathology. The antineutrophilic cytoplasmic
antibody (cANCA) is a useful serological test.
1

Signs
Proptosis, orbital congestion and ophthalmoplegia.
Dacryoadenitis and nasolacrimal duct obstruction.
Coexistent manifestations include scleritis, peripheral ulcerative keratitis,
intraocular inflammation and retinal vascular occlusions.
2 Treatment
a Systemic cyclophosphamide and steroids are very effective. In resistant cases
ciclosporin, azathioprine, antithymocyte globulin or plasmapheresis may be useful.
b Surgical orbital decompression may be required for severe orbital involvement.
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A frequent cause of proptosis in adults and children is inflammatory pseudotumor. The term
"pseudotumor" was coined to indicate a nonneoplastic process that produces the sentinel sign of
an orbital neoplasm, ie, proptosis. In some cases there is an associated systemic vasculitis, such
as Wegener's granulomatosis. The site of inflammation is usually diffuse and not amenable to
excision. The process can be diffuse or localized, specifically involving any orbital structure (eg,
myositis, dacryoadenitis, superior orbital fissure syndrome, or optic perineuritis) or cell type (eg,
lymphocytes, fibroblasts, histiocytes, plasma cells). There may be extension to involve the
cavernous sinuses and intracranial meninges. Onset is usually rapid, and pain is often present.
Pseudotumor is usually unilateral; when both orbits are involved, it is more often a manifestation
of vasculitis. The differential diagnosis includes Graves' ophthalmopathy and orbital lymphoma.
Treatment with systemic NSAIDs, systemic corticosteroids, or radiation is usually effective, but
there is a sclerosing variant that is very resistant to treatment. Surgery tends to exacerbate the
inflammatory reaction, but biopsy may be required to confirm the diagnosis in recurrent or
recalcitrant cases