Idiopathic Orbital inflammation, Non-specific orbital inflammation - Ophthalmology

1. Non specific Orbital inflammation
Dr. Henok Samuel (Ophthalmology Resident)
Moderator:
Dr. Meseret Ejigu, Consultant Ophthalmologist and
Oculoplastic surgeon

2. Table of Contents.
Introduction Sub-classification Clinical manifestation
Diagnosis &
Investigations
Treatment
01. 02. 03.
04. 05.

3. Introduction
 Also known as Idiopathic orbital inflammation/orbital
pseudotumour.
 A heterogeneous group of disorders characterized by orbital
inflammation without any identifiable local or systemic causes.
 A diagnosis of exclusion

4. Introduction
 In 1930 Birch-Hirschfeld first used the term pseudotumor to describe
orbital inflammation that was associated with proptosis and
presumed to be caused by a neoplasm.

5. Introduction
 comprises about 6% of all orbital lesions
 No strong sex predilection, except for orbital myositis, which has
a 2:1 female predominance
 most common in the third through fifth decades of life
 tends to be unilateral - bilateral in 8-20 %
 Resistant and recurrent disease occasionally occurs despite
appropriate management.

6. Introduction
 An evaluation of 65 patients with IOID at Massachusetts Eye and Ear
Infirmary revealed the subtypes
- isolated dacroadenitis 32%
- isolated myositis 29%
- combined dacroadenitis and myositis 8%
- Orbital apex syndrome 9%
- Other 22%
 IOID can also appear as a diffuse orbital inflammation
 may on occasion spread intracranial, cause bony orbital destruction,
and rarely, extend into adjacent sinus
cavities.

7. Sub-classification

8. Ocular and Systemic Manifestations
 Typically acute – but can be insidious
 Most common features of this process are pain and proptosis.
 Chemosis and conjunctival injection
 Eyelid edema and ptosis.
 Diplopia and painful disturbances of ocular motility
 Visual loss is unusual but may be associated with
intraocular inflammation as well as perineuritis of the optic nerve
 Constitutional symptoms

9. Acute Anterior Ocular-Orbital Inflammation
 inflammation of the globe, in particular, the sclera and the Tenon
capsule, and the surrounding orbital tissues.
 acute or subacute
 decreased vision either due to uveitis, with or without an associated
exudative retinal detachment, or papillitis and perineuritis.
 diffuse and nodular choroidal swelling, thickened sclera, and retinal
folds,
 WG can be associated with similar finding
 Hypertrophy and hyperplasia of the RPE may also be present - may
mimic involvement of the choroid with melanoma, metastatic
disease, or lymphoma.

10. Acute Anterior Ocular-Orbital Inflammation

11. Diffuse Idiopathic Orbital Inflammation
 similar symptoms but more severe than those seen with anterior IOI
 proptosis, and soft tissue inflammatory signs - more severe than in
anterior IOI
 Associated with diplopia and limitation of extraocular motility
 All tissues of the orbit are involved, including the fat, muscles, globe,
and optic nerve
 Rarely bone erosion and extend into the adjacent sinus cavities.
 Neoplastic processes, sarcoidosis, and orbital cellulitis are part of the
differential diagnosis.

12. Diffuse Idiopathic Orbital Inflammation

13.  Lymphoma Vs inflammatory disease (IOID) – DWI MRI.
 The densely packed cells in lymphoma inhibit the
nonrandom motion of water, causing lymphoma to appear
bright on DWI, with associated reduction in apparent
diffusion coefficient (ADC)

14. Focal Idiopathic Orbital Inflammation
 present as solitary neoplastic processes.
 subacute in nature, as opposed to anterior orbital inflammation.
 proptosis with or without signs of acute inflammation.
 Diplopia may be present if there is also muscle involvement or
due to surrounding edema
 Neoplastic disease again represents the most important
diagnosis to exclude when considering these findings.

15. Orbital Apex and Superior Orbital Fissure
Inflammatory Syndrome
 Idiopathic inflammation involving the orbital apex is associated with
marked restriction of ocular motility as well as pain and minimal
proptosis.
 The pain is present behind the globe and is boring and persistent
 little evidence of obvious inflammatory signs – edema, injection..
 Decreased vision and an afferent pupillary defect are frequently present
- perineuritis or compression of the nerve by the surrounding edema.
 CT - diffuse inflammatory process in the posterior orbit with extension
into the adjacent orbital fat, EOM, ON, or posteriorily SoF.

16. Orbital Apex and Superior Orbital Fissure
Inflammatory Syndrome

17. Tolosa-Hunt syndrome
 specific form of apical idiopathic orbital inflammation involving
the superior orbital fissure and the cavernous sinus.
 Acute onset - progresses rapidly, recurrent course.
 Usually unilateral, it may be bilateral.
 Involvement of Optic nerve and, the III, IV, and VI CN and the V1.
 headache and ophthalmoplegia with pain described as constant,
deep, and boring.
 Compromise of the superior orbital vein will result in edema of
the eyelids and chemosis.

18. Tolosa-Hunt syndrome – cont’d
 sympathetic innervation to the pupil may also be affected,
resulting in pupillary dilatation and decreased pupillary motility.
 While generally diffuse in nature, this syndrome has been
reported to occur in association with a discrete orbital mass
 DDx - Trauma with or without hemorrhage, aneurysms and
tumors within the cavernous sinus, parasellar neoplasms,
metastatic disease, meningioma, and spread of adjacent sinus
neoplasia or infection.
 MRI, Orbital venography, fine needle biopsy

19. Tolosa-Hunt syndrome – cont’d

20. Orbital Myositis
 Non-infectious inflammatory condition primarily affecting
the extraocular muscles.
 Sudden onset of Orbital /periorbital pain
painful and restricted ocular motility,
diplopia,
proptosis
conjunctival injection
eyelid edema and ptosis
 Commonly single muscle involvement
 Forced duction testing is usually positive.

21. Orbital Myositis – cont’d
 Most common muscles to be involved in this process are the
superior and medial rectus muscles.
 Classic appearance of EOM myositis -- A unilateral thickening of
one or two EOMs, also involving the surrounding fat, tendon, and
myotendinous junction.
 Most important DDX – thyroid orbitopathy and
metastatic spread to extraocular muscle
IgG4 related disease
orbital cellulitis
lymphoma
low-flow carotid cavernous fistula (CCF)

22. Orbital Myositis – cont’d

23. Orbital Myositis – cont’d
 In patients with normal thyroid profile, these findings may
suggest IgG4-RD and a serum IgG4 level may be considered
 Metastases and lymphoma may also mimic myositis and are
seen as a focal mass with increased signal intensity in the EOM’s.
 Patients with low-flow carotid cavernous fistula (CCF) may also
share features with myositis, as the venous congestion may
appear on CT and MRI as inflamed EOM.

24. Dacroadenitis
 acute onset of pain localized to the supratemporal region
 Examination - lid edema, warmth, and erythema, but
there is no induration to suggest cellulitis.
 A characteristic S-shaped deformity with ptosis of the upper lid
 lacrimal gland may be palpable and exquisitely sensitive to touch.
 enlarged and erythematous palpebral lobe of the lacrimal gland is
easily visualized in the supratemporal conjunctival fornix.
 Lateral conjunctival chemosis and injection

25. Dacroadenitis – cont’d
 Involvement of the adjacent lateral rectus muscle results in
painful ophthalmoparesis and diplopia.
 Proptosis and decreased visual acuity are usually absent
 Bilateral involvement should suggest the presence of a systemic
disease such as sarcoidosis, lymphoma, Sjögren syndrome,
malnutrition, metabolic derangement such as diabetes mellitus
and cystic fibrosis, or drugs such as thiouracil

26. Dacroadenitis – cont’d
 neoplastic processes Vs inflammation
 An epithelial neoplasm will typically only involve a portion of the
lacrimal gland, usually the orbital lobe.
- well-circumscribed and round to oval in shape.
 Inflammatory process -- “almond-shaped” appearance of
the gland with a tapered posterior margin of the gland
- associated with inflammation of the surrounding soft tissues

27. Dacroadenitis – cont’d
 B-scan - lacrimal enlargement with a homogeneous acoustic
appearance.
 CT
 enlarged gland appears elongated and molded to
surrounding structures.
 Infiltration of the surrounding tissues is common with
involvement of Tenon’s capsule and the lateral rectus
muscle.
 There is no erosion of adjacent bone.

28. Pathology and Pathogenesis
 Differ depending on the degree of inflammatory and
fibrovascular response
 Histologic findings in the acute form of the disease consist of a
hypocellular polymorphous infiltrate –diffuse and multifocal
composed of mature lymphocytes, plasma cells, macrophages,
and polymorphonuclear leukocytes.
 In contrast, lymphoid neoplasms are characterized by a unifocal
hypercellular monomorphous infiltrate with scant stroma
● Atypical findings - eosinophilia, granulomatous inflammation,
vasculitis, and marked desmoplasia

29. Pathology and Pathogenesis
 Uncommonly, granulomatous inflammation with multinucleated
giant cells and noncaseating granulomas may occur and can
mimic sarcoidosis.
 Cases of idiopathic granulomatous orbital inflammation without
evidence of systemic disease are believed to be a
subclassification of orbital pseudotumor.
● subacute and chronic forms of idiopathic pseudotumor,
increasing amounts of fibrovascular stroma and relatively less
inflammatory cellular infiltrate are present.

30. Pathology and Pathogenesis
 Some cases of orbital inflammation are primarily sclerotic in
nature and may present insidiously without passing through a
prior acute inflammatory phase.
 Histopathologically, these lesions are characterized by a scant
inflammatory cell infiltrate and dense desmoplastic stroma.
 Some investigators classify these lesions as a form of
orbital pseudotumor.

31. Work-up of patients
 diagnosis of exclusion.
 A meticulous history and physical examination
 Medical evaluation and laboratory testing are tailored to the
clinical presentation and differential diagnostic possibilities
 CBC, ESR, TFT, TSI, anti-dsDNA, ACE, ANA, ANCA, VDRL/RPR, CSF
 CXR – TB, sarcoidosis, vasculitis, WG

32. Work-up of patients
 CT - provides good delineation of orbital anatomy
- associated sinus disease or bony erosion,
- Presence of calcium in a lesion
- Contrast enhancement
.
 MRI - more specific information concerning pathologic processes
- fat suppression technique increase specificity and sensitivity
- Suspected intracranial spread,
- orbital apex and cavernous sinus pathology
- hypointense to fat on T1-weighted images and
- isointense or minimally hyperintense to fat on T2-weighted
 Other imaging – PET, gadolinium scanning, orbital venography

33. Work-up of patients
 most important diagnostic aids - a trial of high-dose
corticosteroids.
An 80-mg daily oral dose for a week will usually result in significant
improvement or complete resolution of the inflammatory process
If this does not occur -- orbital biopsy should be performed in order
to obtain a tissue diagnosis.

34.  not without risk
 ?may exacerbate the condition, if biopsy is performed during
the acute phase of pseudotumor.
 biopsy when appropriate and possible.
 Adequate sample (1 cm3), from a representative location.
 Commonly Incisional
 isolated lacrimal lesion suggestive of a primary epithelial tumor
may require Excisional biopsy.
Issue of orbital biopsy?

35. Issue of orbital biopsy?
Instances in which biopsy should strongly be considered
 lesions recurrent or refractory to treatment
 Chronicity
 presence of a discrete mass on CT or MRI - particularly when
accompanied by bone involvement or extraorbital extension
 history of carcinoma
 isolated lacrimal enlargement

36. Treatment
 Since IOID is a diagnosis of exclusion, be ready to question the
diagnosis and biopsy as appropriate.
 primary goal is rapid and effective suppression of the
inflammatory process.
 Corticosteroids are the current mainstay of therapy
 Inadequate treatment regimens are believed to result in higher
rates of recurrence and increased visual morbidity from
cicatrization of orbital tissues.
 Therefore, prompt administration of systemic
corticosteroids in dosages of sufficient strength and
duration is important.

37. Treatment
 Initial dose of oral prednisone, 60–100 mg/day in divided doses
 1.0–1.5 mg kg/day may be used in both children and adults.
 High-dose corticosteroids should be continued for 2–3 weeks to
suppress the inflammation adequately.
 slowly taper over the next few weeks - reducing the prednisone by
5–10 mg/week will prevent rebound.
 A 3 day course of high dose IV steroids (1 g/day,
methylprednisolone) followed by an oral steroid taper.
 Other modalities include low dose radiation, Immunosuppressive
and biologic agents.

38. ● Duane's ophthalmology
● BCSC_2019-2020, Sec 7, fundamentals & principles of
ophthalmology
● Albert and Jakobiec's Principles and Practice of
Ophthalmology
● Yanoff & Duker: Ophthalmology, 3rd ed.
● Eyewiki
References

39. Thanks!