Congenital anomalies of uveal tract

• Heterochromia of iris: variations in iris colour– Heterochromia iridium: color of one iris differs from other– Heterochromia iridis: one sector of iris differs from other– Acquired seen in: heterochromia, cyclitis, siderosis and malignant

melanoma of iris

• Corectopia: abnormally eccentric placed pupil(normal pupil is slightly nasal to centre)

• Polycoria: more than one pupil• Congenital aniridia: congenital absence of iris; true absence

is very rare; usually a peripheral rim of iris is present and this is called clinical aniridia where zonules of lens and ciliary processes are visible; familial condtn

• Persistent pupillary membn: – represents the remanant of vascular sheath of lens; c/b

stellate shaped shreds of pigmented tissue coming from ant surface of iris; float freely in the ant chamber or attached to ant surface of lens

• Coloboma: absence of tissue of iris, ciliary body and choroid– Typical: seen in infero nasal quadrant and occurs d/t

defective closure of embryonic fissue– Atypical: found in other positions – Complete coloboma extends from pupil to optic nerve, with a

sector gap occupying about one eigth of circumference

Uveitis

• Inflammation of uveal tissue only; but often a/w inflammation of retina, vitreous, sclera and cornea; and d/t close association inflammation tends to involve the uvea as whole

• Classification Anterior uveitis Inflammation of uveal tissue from iris upto

pars plicata of ciliary body-Iritis, Iridocyclitis, Cyclitis

Intermediate uveitis Iris + pars plicata

Post. Uveitis Choroid and often retina; chorioretinis

Pan uveitis Inflammation of whole uvea

Acute uveitis Sudden symptomatic onset; last upto 6wks – 3 months

Chronic uveitis Insidious and asymptomatic; >3mo

Suppurative Pus

Non suppurative Non granulomatous and Granulomatous

Infective Allergic

Toxic Traumatic

Uveitis w/ noninfective systemic dzs Idiopathic

Etiology of uveitis

Infective uveitis • Modes:

– Exogenous infection: penetrating injuries, perforation of ulcer and ocular surgery

– Secondary infection: acute purulent conjunctivits, keratitis, scleritis, retinitis, orbital cellulitis and orbital thrombophlebitis

– Endogenous infection: through bloodstream

Types Comments

Bacterial infections Granulomatous; TB, syphilis, leprotic, brucellosis Pyogenic by staph, strep, pneumo., gonoco

Viral Herpes simplex, Herpes zooster and CMV

Fungal Aspergillosis, Candidiasis, Blastomycosis

Parasitic Toxocariasis, Onchocerciasis, amoebiasis

Rickettsial Scrub typhus and epidemic

Allergic • D/t microbial allergy• Anaphylactic uveitis • Atopic uveitis: airbone allergens • Autoimmune uveitis: Still’s dz, RA, Wegner’s granulomatosis, SLE,

Reiter’s dz• Phacoanaphylactic • Sympathetic • HLA associated

– HLA-B27= a/w ankylosing spondylitis and Reiter’s synd. – HLA-B5: Behcet’s dz– HLA-DR4 and DW15: Vogt kayanagi harada’s dz

Toxic• Endotoxins: autotoxins or microbial toxins • Endocular toxins: from ocular tissues; blind eyes, retinal hhg and

RD • Exogenous toxins: inorganic, animal or vegetative origin

Traumatic -d/t accidental or operative injuries to uveal tissues by

direct mechanical effects, irritative effects, microbial invasion, chemical effects, sympathetic ophthalmia

Uveitis a/w noninfectious systemic dz: a/w sacroidosis, PAN, RA, DM, Gout, Sclerosis, Psoriasis, Lichen planus, Erythema nodosum

Idiopathic

Pathology

• Suppurative uveitis: – d/t infection by organisms like staph, strep, pseudomonas, pneumo.,

gonococcus, purulent inflamm of the uvea is the part of endo or panophthalmitis

– c/b outpouring of purulent exudate and infiltration by PMN cells of uveal tissue, ant. Chamber, post chamber and vitreous as a result whole uveal tissue is thickened and necrotic filled with pus

• Non granulomatous uveitis – Occurs d/t physcial or toxic reactions often d/t hypersensitivity – Marked dilation and increased permeability of vessels, breakdown of

blood aq. Barrier with outpouring of fibrinous exudate and inflitration by plasma cells, lymphocytes and macrophages; usually diffuse

– Iris waterlogged, oedematous, muddy w blurring of crypts and furrow, mobility of iris is reduced, pupil same d/t toxic constriction and engorgement of vessels of iris

– Exudates and lymphocytes poured into ant chamber results into aq. Flare and Kp at the back of cornea

– Posterior synechia d/t exudate in post chamber – Cyclitic membrance at the back of lens in severe cases – Heals by necrotic point fibrosis> destruction

• Granulomatous uveitis:– d/t chronic inflammation by irritant forgein body,

hhg or necrotic tissue or non pyogenic organisms of less virulence like TB, Syphilis, Leptospirosis, Mycotic, Protozoal, Helminthic

– Also seen in sarcoidosis, sympathetic ophthalmia and Vogt kayanagi haradas dz

– Similar to granuloma formation else where but here the epithelioid and giant cells forms nodules like Koeppe nodules near pupillary margin and at the back of cornea called mutton KP

Anterior uveitis

• Clinical features:– Acute or chronic – Pain; dull aching throbbing sensation worse at night – Redness; circumcorneal congestion – Photophobia and blepharospasm; reflex mechanism– Lacrimation – Defective vision: slight blur to marked deterioration d/t

myopia, corneal haze, aq turbidity, pupillary block, complicated catarct, vitreous haze, cyclitic membrance, macular oedema, papillitis and secondary glaucoma

• Signs:– Lid edema– Circumcorneal congestion – Corneal signs:

• Corneal edema• Keratic precipitates:

– Mutton fat; d/t epitheloid & giant cells in granulomatous; usually few in number upto 15 which are greasy and waxy

– Smaller & medium Kps: d/t lymphocytes in non granulomatous, small discrete multiple may be hundres

– Red kps: with RBCs + inflammatory cells – Old kps: sign of healed uveitis; either of above shrink, fade, become pigmented

and irregular in shape

• Posterior corneal opacity

--Ant chamber signs: • Aq. Cells;

– early feature and should be counted with oblique slit lamp – -= o cells, +/-=1-5 cells, +1= 6-10cells, +2= 11-20 cells, +3= 21-50 cells, +4= over 50 celss

• Aq. Flare – d/t leakage of protein partices in aq humour; demonstrated on the slit lamp examn by a

point beam of light passed obliquely – In beam of light, they appear as a suspended and moving dust particles= Brownian

movement – Marked in granulomatous – 0= no aq flare, +1= just detectable, +2=moderate flare, +3=marked flare, +4= intense

flare • Hypopyon, Hyphaema, Changes in depth and shape of ant chamber, Changes in angle of ant

chamber

• Pupillary signs – Narrow pupil; d/t irriation of sphincter pupillae by toxins – Irregular pupil shape: d/t segmental post. Synechia when atropinised dilatation of

pupil results into festooned pupil– Ectropion pupillae; eversion of pupillary margin– Pupillary reaction: sluggish or absent – Occlusio pupillae: completely occludes by exudates

• Iris signs:– Loss of normal pattern– Changes in iris colour; muddy in color during active phase and

hyperpigmented & depigmented cells in healed stage – Iris nodules:

• Typically seen in granulomatous uveitis – Koeppe’s nodules; at pupillary bordermay initiate posterior synechia – Busacca’s nodules; near collarette, large but less common than Koeppe’s

nodules

– Posterior synechiae; segmental, annular(seclusio pupillae, iris bombe formn), total

– Neovascularisation(rubeosis iridis)

• Lenticular changes – Pigment dispersal on ant surface of lens – Exudates deposition – Complicated catarct; as a complication of persistent iridocyclitis, bread

cumb appearance of early posterior subcapsular opacities

• Changes in vitreous – Exudates and inflammatory cells in ant vitreous

Complications 1. Complicated cataract 2. Secondary glaucoma

I. Early glaucoma: d/t exudates clogging the trabecular meshworkII. Late glaucoma: d/t pupillary block formed by posterior synechia

3. Cyclitic membrane: d/t fibrosis of exudates behind the lens 4. Choroidits 5. Retinal complications: CME, Macular degeneration, RD, Secondary

periphlebitis 6. Papillitis 7. Band shaped keratopathy 8. Phthisis bulbi: final end stage of dz, ciliary body is disorganized and so

aq. Production is hamperedeye is soft, shrinks and eventually becomes a samll atrophic globe

DDx • Acute red eye

– Acute congestive glaucoma– Acute conjunctivitis

• Granulomatous vs Non granulomatous • Etiological ddx

Investigations: Blood; TLC, DLC, ESR, Blood sugar levels, Blood uric acid,

Serological test for syphilis, toxoplasmosis, histoplasmosis, test for ANA, RF, CRP

Urine Exam; for WBCs, pus cells, RBC and culture Stool exam: for cyst and ova in parasitic Radiological: for TB, sarcoidosisSkin test: tuberculin, K velm’s test, toxoplasmin test

TX of iridocyclitis

Specific tx: Find out the cause and tx is started but non specific is sufficient most of the time

TX of complication1. Inflammatory

glaucoma: 0.5% timolol maleate eyedrops 2ce day and tablet acetazolamide 250mg thrice a day

2. Post inflammatory glaucoma: iridotomy

3. Complicated catarct: lens extraction

4. Retinal detachment: vitrectomy and line of mx of RD

5. Phthisis bulbi: Enucleation

Non specific tx

Physical measures:1. Hot

fomentation2. Dark goggles

Systemic therapy1. Corticosteroids: high

doses of prednisolone daily or alternate day therapy and tapered In 6-8 weeks

2. NSAIDs: phenylbutazone or oxyphenbutazone

3. Immunosuppressive: mtx, azathioprine, cyclosporine,

Local therapy1. Mydriatic

cycloplegic drugs: 1% atropine 2-3 times a day, 2% Hoomatropine/ 1% cyclopentolate 3-4 times a day continued for 2-3 weeks

---0.25ml of mydricain2. Corticosteroids; dexa,

betamethasoneeye drops 4-6 times a day or ointment at night or ant subtenon injection

1.2

Posterior uveitis

• Refers to inflammation of choroid & since outer layers of retina are in clost contact with choroid and also depend on it for nourishment, the resultant lesion is always a chorioretinitis

• Etiology and pathogenesis: same as ant. Uveitis

Clinical types

• Suppurative choroiditis: always a part of endophthalmitis

• Non suppurative choroiditis: granulomatous or non granulomatous – Diffuse choroiditis – Disseminated choroiditis – Circumscribed or focal choroiditis

• Central • Juxtacaecal e.g Jensen’s choroiditis • Ant peripheral choroiditis • Equatorial Choroidits

Signs and symptoms

• Symptoms: – Painless condition – Defective vision:d/t vitreous haze – Photopsia: subjective sensation of flashes d/t irriation

of rods and cones – Black spots floating in fornt of eyes: exudative clumps– Metamorphosia: distorted image perception – Micropsia – Macropsia – Positive scotoma

• Signs:– Vitreous opacities: middle or posterior part; fine

coarse, stringy or snowball opacities – Features of patch of choroiditis

• Active stage: pale yellow dirty white raise area with ill defined edges

• In atrophic or healed stage: sharply defined and delineated; area shows white scleara below the atrophic choroid and black pigmented clumps at periphery of lesion

• Complications: – Extension of inflammation to ant. Uvea– Complicated cataract – Vitreous degeneration – Macular oedema– Secondary periphlebitis – RD

• Tx. Broadly on the lines of ant. Uveitis

Endophthalmitis

• Defined as inflammation of the inner structures of eyeball i.e uveal tissue and retina a/w pouring of exudates in the vitreous cavity, ant. Chamber and posterior chamber

• Infective and Non infective endophthalmitis

Infective endophthalmitis

• Modes of transmission:– Exogenous; penetrating, perforating injuries, perforation

of corneal ulcer and intraocular operationss– Endogenous; blood stream– Secondary; extension from orbital cellulitis,

thrombophlebitis and infected corneal ulcers • Causative organisms – Bacteria: Staph, Strept, Pseudomonas, Pneumococci and

Corynebacterium also propionibacterium and actinomyces – Fungi: asperigllus, fusarium, candida, etc

Non infective endophthalmitis

• Refers to inflammation of inner structurs d/t certain toxins or toxic substances – Post operative sterile endophthalmitis : d.t chemical

adherent to IOL, or chemicals adherent to instruments

– Post traumatic sterile endophthalmitis : toxic rxn to retained intraocular foregin body e.g pure copper

– Intraocular tumour necrosis: masquerade syndrome– Phacoanaphylactic endophthalmitis: lens protein is the

cause in Morgagnain cataract

Clinical picture• Symptoms: occurs within 7 days of operation and is c/b ocular pain

severe, redness, lacrimation, photophobia and marked loss of vision

• Signs:– Lids: swollen and red– Conjunctiva: chemosis and marked circumcorneal congestion– Cornea: edematous, cloudy and ring infiltration – Edges of wound: yellow and necrotic and wound may gape– Ant. Chamber: hypopyon, soon full of pus– Iris: edematous and muddy– Pupil: yellow reflex d/t purulent exudates in vitreous – Vitreous exudate: late stage yellowish white mass is seen through fixed

dilated pupil; amaurotic cat’s eye reflex– IOP: raised in early stages but in severe cases falls d/t destruction of clilary

process

Tx

• Antibiotic therapy • Steroid therapy • Supportive therapy • Vitrectomy

Antibiotic therapy

Intravitreal antibiotics and diagnostic tapThis forms the main stay of treatment of acute bacterial endophthalmitis1. It is performed

transconjunctivally under topical anesthesia from the area of pars plana, vitreous tap is made f/b intravitreal injection

2. Combination of 2 antibiotics;

First choice: Vancomycin + ceftazidime

Second choice: Vancomycin + Amikacin

Third choice: Vancomycin + gentamycin

Topical antibiotic therapy1. Used frequently

3omins to 1 hourly 2. 2 drugs are prefered

• Vancomycin or cefazoline

• Amkikacin or tobramycin

Subconjunctival injection1. First

choice:Vancomycin + ceftazidime

2. Second choice: Vancomycin + cefuroxime

Systemic therapy1. Ciprofloxacin IV fb oral doses 2. Vancomycin IV and ceftazidime IV 3. Cefazoline and amikacin IV

• Steroid therapy– Limit the inflammation and is used after 24-48 hrs of

extensive use of antibiotics – IVit Dexamethasone– Subconjunctival inj. Of dexa– Topical dexa– Systemic steroids: oral steroids after 24 hrs by

prednisolone high doses

• Supportive therapy– Cycloplegics – Antiglaucoma drugs

• Vitrectomy: if the px doesn’t improve with the above therapy for 48-72 hrs or when the px presents with severe infection with VA= PL

Panophthalmitis

• Intense purulent inflammation of the whole eyeball including the tenons capsule

• Usually begins either as purulent ant or post uveitis endophthalmitispanophthalmitis

• Etiology– Acute bacterial infection – Mode of infection and causative organisms as same

as endophthalmitis

s/s

• Symptoms: – Severe ocular pain and headache – Complete loss of vision– Profuse watering – Purulent discharge – Marked redness and swelling of eyes and – Associated with constitutional symptoms like fever

and malaise

• Signs: – Lids: swollen and hyperemic – Eyeball: sllightly proptosed, ocular movements are painful and

limited – Conjunctiva: marked chemosis and ciliary as well as

conjunctival congestion– Cornea: cloudy and oedematous – Ant chamber: full of pus – Vision: completely lost and perception of light is absent – IOP: markedly raised – Globe perforation occur at limubus and pus comes out, IOP falls

then

• Complications:– Orbital cellulitis – Cavernous sinsus thrombosis – Meningtitis or encephalitis

• Tx. – Little hope of saving such eye and the pain and toxemia

demands for its removal • Anti inflammatory and analgesic should be started

immediately • Broad spectrum antibiotics to prevent further spread • Evisceration