INFLAMMATIONS OF

THE ORBIT
DR POOJA PEETHAMBARAN
INTRODUCTION

• All inflammatory disease that affects some or all of the structures

contained within the orbit external to the ocular globe
• Inflammatory process may extend beyond the orbit ,in to the cavernous
sinus through the orbital apex.
• OID needs to be differentiated from neoplastic and infectious disease that
may have a similar presentation.
CLINICAL FEATURES

• PAIN is often a prominent complaint.

• EOM –diplopia
• Lacrimal gland- painful superolateral orbital swelling
• Generalized –proptosis ,chemosis ,periorbital swelling or lid swelling,
• Severe –blindness due to optic nerve compression.
• Most common infectious etiology.
• Onset can be acute to insidious depending up on etiology.
• A subacute clinical course is common with TAO and idiopathic orbital
inflammation ,but also occurs with systemic vasculitis .
RHINO ORBITAL MUCORMYCOSIS

• Mucormycosis are a group of invasive infections which are caused by

filamentous fungi of the order ,Mucorales of the mucoraceae family.
• Rhino orbital mucormycosis (ROM) is a rare disease with an overall
prevalence of 0.15% of the diabetic.
• high mortality rate of 30-70% .
• Aggressive fungal infection which is seen in immunocompromised hosts
• Risk factors –poorly controlled diabetes mellitus ,hematological
malignancies ,covid and a prolonged corticosteroid treatment.
• Death may occur within 2 weeks in untreated or unsuccessful cases.

They represent the third leading cause of invasive fungal infections

following aspergillus and candida
• Presentation –is with gradual onset facial and periorbital swelling,
diplopia, and visual loss.
• Signs-Ischemic infarction superimposed on septic necrosis is responsible
for black eschar which develops on palate ,nasal septum ,skin and eyelid
• Pathogenesis- infection is acquired by inhalation of spores giving rise to
upper respiratory infection which spreads to the sinuses and subsequently
to the orbit and brain.
• Invasion of blood vessels by hyphae results in occlusive vasculitis with
ischemic infarction of orbital tissue.
• Complication –retinal vascular occlusion ,multiple cranial nerve palsies.
TREATMENT

• Intravenous antifungal agents such as amphotericin

• Daily packing and irrigation of involved area with amphotericin
• Wide excision of devitalized and necrotic tissue.
• Correction of underlying metabolic defect.
• Exenteration required in unresponsive cases
IDIOPATHIC ORBITAL INFLAMMATORY DISEASE (IOID)

• It is a disorder characterized by non-neoplastic ,non infective ,space

occupying orbital lesions .
• Histopathology reveals pleomorphic inflammatory infiltrates .
• It was previously known as orbital Pseudotumour/nonspecific orbital
inflammation.
• The inflammatory process may involve any or all orbital tissue resulting in
myositis, dacryoadenitis ,orbital apicitis
• Third most common orbital disease after Graves's orbitopathy and
lymphoproliferative disease.
• UNILATERAL
COURSE

• Spontaneous remission after a few weeks without sequelae

• Severe prolonged inflammation eventually leading to progressive fibrosis
of orbital tissues , resulting in a frozen orbit.
• Associated with ptosis and visual impairment caused by optic nerve
involvement .
INVESTIGATIONS

• Brief history
• Complete ocular examination
• Orbital CT (axial and coronal )
• Blood investigation is generally required in persistent cases to confirm the
diagnosis
TREATMENT

• Systemic steroid –administered only after the diagnosis has been

confirmed .initially 60-80 mg/day and later tapered .
• Radiotherapy if no improvement after 2 weeks of adequate steroid therapy.
Even low dose (10Gy )produces remission.
• Antimetabolites –such as methotrexate or mycophenolate mofetil may be
necessary if there is resistance to steroid and radiotherapy
• Systemic infliximab effective in recurrent cases.
TOLOSA HUNT SYNDROME

• Rare idiopathic condition caused by non specific granulomatous

inflammation of the –
• Cavernous sinus
• Superior orbital fissure
• Orbital apex
• Relapsing /remitting acute orbital pain and paralysis of cranial nerve 3,4,6
and 7
• It is a diagnosis of exclusion .
• Prevalence –estimated annual incidence is one case per million per year.
• No information is available as to what triggers the inflammation process
in the region of the cavernous sinus /superior orbital fissure .
SYMPTOMS

• Unilateral
• Severe retro orbital /periorbital constant pain of acute onset
• Diplopia, visual loss, paresthesia along the forehead
SIGNS

• Proptosis
• 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.
• Gnawing pain may precede ophthalmoplegia.
• The international headache society include following criteria for Tolosa-
hunt syndrome .
• Episodes of unilateral orbital pain for an average of 8 weeks if left
untreated
• Associated paresis of the third ,forth, or sixth cranial nerves ,which may
coincide with onset of pain or follow it by a period of up to 2 weeks
• Pain that is relieved within 72hours of steroid therapy initiation .
• Exclusion of other conditions by neuroimaging and angiography .
BEFORE AND AFTER TREATMENT
• Corticosteroids are the treatment of choice
• Usually providing significant pain relief within 24-72 hours of therapy
initiation.
• Ophthalmoparesis usually requires weeks to months for resolution .
• For refractory cases ,azathioprine, methotrexate, or radiation therapy has
been employed.
ORBITAL MYOSITIS

• It is an idiopathic ,non specific inflammation of one or more extraocular

muscles.
• Considered a subtype of IOID
• Presentation –most commonly affects young adults in the third decade of
life, with a female predilection.
• Histology – chronic inflammatory cellular infiltrates
• Presents with unilateral orbital or periorbital pain, painful and restricted
eye movement ,Proptosis ,periorbital edema and hyperemia of the
conjunctiva .
• Classic appearance-unilateral thickening of one or two EOMs, often also
involving the surrounding fat, tendon, and myotendinous junction.
• Differential diagnosis -1.TAO 2.IgG Related disease 3.lymphoma 4.CCF
• Differentiation from thyroid disease –unilateral, infiltration of the
surrounding fat and early involvement of the superior oblique muscle .
• Course –acute non recurrent involvement which resolves spontaneously
within 6 weeks
• Chronic disease characterized by either a single episode persisting for
longer than 2 month or recurrent attacks.
TREATMENT

• NSAIDS adequate in mild diseases

• Systemic steroids are generally required and produce dramatic
improvement .
• Radiotherapy –effective ,if above treatment fails.
DACRYOADENITIS

• lacrimal gland involvement occurs in 25%of patients with IOID

• More commonly occurs in isolation, resolves spontaneously without
treatment .
• Etiology –
• m/c –inflammatory non –infectious
• Rare –Bacterial, usually due to S.aureus ,N. gonorrhoeae
• Viral –mumps, influenza ,infectious mononucleosis
• Typically occurs in children and young adults
• Signs –
• Swelling of the lateral aspects of the eye lid giving rise to a characteristic s-
shaped ptosis and slight downward and inward displacement.
• Classic –diffuse enlargement ,including orbital and palpebral lobes along
with inflammation of surrounding tissues .
• D/D-epithelial neoplasm –involves portion of the lacrimal gland ,orbital
lobe usually
• Treatment –if specific etiology is unclear treat the patient empirically with
systemic antibiotics
• Clinical response to antibiotics can guide further management and in
unresponsive cases steroid therapy can be started .
IGG4 RELATED DISEASE

• IgG4 related disease was first described in patients with autoimmune

pancreatitis who had high serum IgG4 levels.
• M=F, younger age group.
• Considered as a multi system disorder which involves pancreas, biliary
tree, salivary gland, kidney, lung, skin, and prostate .
• CNS manifestation are uncommon -hypertrophic pachymeningitis and
hypophysitis are frequent manifestations .
• In orbit ,it involves lacrimal gland >extraocular muscles>adipose tissue
• C/F –painless unilateral or bilateral periorbital swelling ,erythema due to
lacrimal gland involvement.
• EOM less common than TAO.
• Compressive optic neuropathy –less common
• Epiphora ,salivary gland enlargement.
DIAGNOSTIC CRITERIA –IGG4 ROD

• Imaging studies-enlargement of lacrimal gland ,trigeminal nerve or EOM

as well as masses, enlargement or hypertrophic lesions in various
ophthalmic lesions
• Histology -lymphoplasmacytoid infiltration and storiform fibrosis,ratio of
IgG4 /Igg 40% or above or more than 50 IgG 4 cells/HPF
• Blood test shows elevated serum IgG4 >135 mg/dl
TREATMENT

• Corticosteroid
• Relapse rate has been reported as high as 70%
• Treatment of relapse -6-10 weeks of steroid or rituximab.
• Relapse is seen also with rituximab after 6 months .
• Poor outcome with MMF, azathioprine and radiotherapy.
SARCOIDOSIS

• Idiopathic, multisystem inflammatory disorder characterized by

noncaseating granulomas.
• Ophthalmic disease occurs in 25 -50%of patients with sarcoidosis among
which anterior and posterior uveitis are common.
• Generally limited to the lacrimal gland .when extralacrimal gland
involvement is present ,it is almost always unilateral.
• Presentation is same as other OID like pain ,ophthalmoplegia ,proptosis, and
later diminished vision.
• Symptoms-wax and wane.
• Diagnosis is made with help of other organ involvement.
• ACE level doesn’t help without pulmonary involvement .
• Chest CT has been reported to be far more sensitive than chest radiography.
• Confirmation-pathologic evidence of noncaseating granulomatous
inflammation as well as the exclusion of other possible granulomatous
disease.
• treatment- corticosteroid
• Refractory-methotrexate, azathioprine and infliximab.
VASCULITIS

• Churg – Strauss syndrome

• Polyarteritis nodosa
• Giant cell arteritis
• WG has a special predilection for orbital tissue which is a small and
medium sized vessel vasculitis
• Orbital inflammation along with episcleritis , scleritis or uveitis is
common than retinal vasculitis
• Diffuse orbital involvement is common.
• Bilateral eye pain , proptosis , erythema and motility restriction are
common clinical features.
• Proptosis is a poor prognostic sign , with half of patients in 1 series
subsequently losing vision due to optic nerve ischemia
• ANCA can have a high positive predictive value for this disease in patients
with a symptom complex suggesting WG.
• Sensitivity and specificity for c – ANCA with antibodies directed toward
proteinase 3 (PR3)is between 80 and 100%.
• Since greater than 30% of patients with limited WG , in which the kidneys
are spared , will not have ANCA positivity, it should not be used to rule
out WG.
• Hence , confirmed diagnosis can be done on basis of biopsy.
• The diagnosis of WG is usually based on histological findings in a compatible
clinical setting
• The classic histopathological triad
1. Granulomatous formation
2. Tissue necrosis
3. Vasculitis involving arteries , capillaries and veins
• Nearly all patients who underwent orbital biopsy in 1 small series had evidence of
necrosis and granulomas
• Necrotizing vasculitis appears to be less common
• Remission – corticosteroid , Methotrexate , Cyclophosphamide ,
Rituximab
• Maintenance – Azathioprine , methotrexate , leflunomide , Rituximab
• More aggressive therapy is reserved for those with potential optic nerve
compromise with combination of high dose corticosteroid and
cyclophosphamide.
• Rituximab vs Azathioprine in maintenance therapy
• Thank you