Orbital pseudotumor

Orbital pseudotumor

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 M ost common features of this process are pain and proptosis . C hemosis and conjunctival injection E yelid 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 . A cute onset - progresses rapidly, recurrent course . U sually unilateral , it may be bilateral . I nvolvement 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 N on-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 M ost 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 D iffer 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. I ssue 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 I nitial 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 !