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Anatomy and diseases of the uvea

Uvea is the vascular coat of eye ball and lies between the sclera and retina. Uvea is composed of three parts i.e. iris, ciliary body and choroid. These three portions are intimately connected and a disease of one part also affects the other portions though not necessarily to the same degree.


1) Iris:
Iris is a delicate membrane placed in the anterior part of the eye ball and perforated in the center i.e. pupil. It arises from the middle of anterior surface of ciliary body. It is slightly pushed forward by the lens which gives it the appearance of a truncated cone, the apex of which has been cut off.


Anterior surface has two zones: 1) Papillary zone: is flat and has a dark border at the papillary margin , known as papillary ruff. Junction of papillary and ciliary zone is marked by a smooth ridge known as collaret. 2) Ciliary zone: towards the ciliary border has ciliary crypts. Histologically iris has got 5 layers (from before backward). 1- Endothelium: flat nucleated cells absent over the crypts probably to allow free movement of the aqueous in and out of the iris.

2- Vessel layer: consist of blood vessels lying in collagen fibers, chromotophores etc., 3- Muscular layer: two muscles: a) Sphincter pupillae- plain muscle developed from neuroectoderm- supplied by parasympathetic fibers coming through the 3rd nerve (relayed in ciliary ganglion). b) Dilator pupillae- also arise from neuroectoderm and supplied by cervical sympathetic fibers reaching the eye through long ciliary nerves (not relayed in ciliary ganglion).

4- Pigmented epithelium- two layers. The two layers are loosely attached to each other and there is a potential space between the 2 layers. In iridocyclitis there is adhesion of the iris to the lens. When a mydriatic is applied the posterior layer of pigment epithelium gets partly detached. This produces pigment deposition and pigmented patches on the anterior lens capsule (broken posterior synechiae).

5- Internal limiting membrane- fine and homogenous structure, not consistently present. Function of the iris: 1- Regulates the entry of light into the eye by changing the size of papillary aperture. 2- Cuts away the peripheral aberrations. 3-Absorption of aqueous also takes place from its surface.

2) Ciliary body: Ciliary body is a ring shaped structure placed more or less sagitally and extends from the ora-serrata to the scleral spur. It consists of ciliary processes and ciliary muscle. On longitudinal section it is triangular. Its anterior surface or base is shortest. Iris is attached to the base. The outer side of triangle is adjacent to sclera and is formed by ciliary muscle.

Ciliary body

Ciliary body

The inner surface is directed towards the cavity of the eye ball and is divided into two portions anterior (pars plicata ) and posterior (pars plana). Ciliary muscle consists of: 1. Meridional fibers. 2. Circular fibers. 3. Radiating fibers.

Meridional fibers on contraction pull the suprachoroidea forwards and release the suspensory ligament allowing the lens to become more convex as in accommodation. The pars plicata has about 70 ciliary processes. They secrete aqueous. Functions of ciliary body: 1. Brings about accommodation. 2. Formation of aqueous. 3. Helps in drainage of aqueous at the angle of anterior chamber.

3) Choroid: Choroid is the analogue of pia-arachnoid of the brain and serves the same purpose of supplying nutrition to the neural portion of eye i.e. retina. Choroid is composed of five portions. 1. The outer most is SUPRACHOROIDEA a potential space between the choroid and sclera. This is lined by endothelium and traversed by fibrous trabeculae.

This space is utilized for the treatment of aphakic glaucoma in the operation of cyclodialysis. Deeper to it are three vascular layers. 2. Layer of LARGE BLOOD VESSELS is outer most. 3. Next comes MEDIUM SIZED BLOOD VESSELES. 4. and SMALL BLOOD VESSELES or CHOROIDO-CAPILLARIES.

Layer of choriocapillaries is the most important. It serves to provide nutrition to the outer layers of retina. The choriocapillaries are much wider than the capillaries elsewhere. Their diameter varies from 10 to 30 microns. 5. The innermost layer is avascular known as MEMBRANE of BRUCHS. This is composed of elastic and cuticular lamina and pigment epithelium of retina is intimately attached to it.

Classification: A) Depending upon the site of inflammation: 1) Anterior uveities - iritis. - cyclitis. - iridocyclitis. As iris and ciliary body are continuous, iritis seldom occurs without some inflammation of the ciliary body and vice versa.




The terms iritis and cyclitis are used depending on which structure is clinically more affected. 2) Posterior uveities- choroidities. 3) Pan uveities: Iris, ciliary body and choroid are inflamed. B) Uveities may be: -acute. -sub acute. -chronic.



C) Clinico pathological classification:

1) Onset: insidious, (1

Non Granulomatous

Acute onset of severe eye relatively white. inflammation (red eye). 2) Course: chronic. Short. 3) KPs: medium and large Small KPs. and often mutton fat. 4) Slight aqueous flare. 5) Nodules on iris present Intense aqueous flare. Koeppe's nodules are Absent. seen on the pupillary margin. Busacca's nodules are seen near coflarette.

6) Dense broad based posterior synechiae.

Fine synechiae.

7) Tends to affect entire uvea.

8) Heavy vitreous exudate or veils common.

Tends to affect anterior uvea.

Fine punctate opacities in the vitreous.

9) Etiology: direct organismal infection.

10)Complications: more


Complications: few.


In most cases it remains obscure.

1) Infection:

a. Exogenous- the organisms reach the eye from outside. i) Perforating injury. ii) Perforating corneal ulcer. iii)Intraocular operation. This usually leads to suppurative iridocyclitis, endophthalmitis and even panophthalmitis. b. Secondary- due to spread from one or other of the ocular tissue- corneal ulcer, scleritis.

c. Endogenous: Organisms primarily lodged in some other organ of the body reach the eye through the blood stream. i) Viral- herpes simplex, herpes zoster, measles, mumps, rubella. ii)Bacterial: tuberculosis- syphilis, leprosy, gonorrhea, brucellosis. iii) Fungal: histoplamosis, asperigillosis, candida albicans, actionomycosis. iv) Protozoa: Toxoplasmosis. v) Nematodes: ankylostomiasis, filariasis.

2) Allergy:

a. Bacteria: i) T.B. usually of the lungs, lymph nodes. ii) Streptococci- teeth, tonsils, sinuses, urogenital tract. Primarily source of infection exists at these sites. At one time the infection was generalized by the escape of organisms into the blood stream when the ocular tissue- uvea had become sensitized to them. At a later date further dissemination of the organisms or their proteins meeting the sensitized uveal tissue excites an allergic response.

b. Lens proteins- phako- anaphylactic reaction. They have also a toxic action on iris. c. Uveal pigment- sympathetic ophthalmitis.
3) Constitutional disorders:

Diabetes mellitus, gout, rheumatoid arthritis (adult and juvenile), ankylosing spondylitis.
4) Trauma:

a. Blunt. b. Sympathetic ophthalmitis.

5) Idiopathic:

a. Sarcoidosis. b. Vogt- Koyanagi- Harada's disease. c. Behcet's disease.

6) Miscellaneous:

Intraocular haemorrhage, intraocular tumour.


Inflammation of the iris and ciliary body has the same characteristic as other vascular connective tissue.


1. dilatation of the blood vessels. 2. exudation of protein rich fluid into the tissue space with leucocytes or lymphocytes.
1) Pupil: small and reacts sluggishly to

light owing to a. hyperemia of the radially disposed vessels of the iris. b. exudate contains toxic substances which irritate the sphincter pupillae (this muscle in more powerful than dilator pupillae).

2) Delicate patterns of iris: crypts-

become blurred and indistinct (muddy iris).

3) Colour of iris: undergoes change-

brown iris becomes grayish.

4) Exudate in AC:

a. Aqueous flare. b. Sometimes hypopyon.

5) Exudate in posterior chamber:

induces adhesion between posterior surface of iris and the lens (posterior synechiae).

6) Exudate in vitreous: vitreous haze. Symptoms:

1) Pain the eye ball: It is dull aching, worse at night, it may be referred to the forehead along the 1st division (ophthalmic) of the trigeminal. It is due to: a. The iris has a rich nerve supply; the nerve endings are stimulated by a high concentration of toxic substances. b. Spasm of ciliary muscle. c. Secondary glaucoma.

2) Diminition of vision due to:

a. Spasm of ciliary muscle- pseudo myopia. b. turbidity of aqueous, vitreous. c. exudate in papillary area. d. choroidities when associated in panuveitis. e. Secondary glaucoma. f. complicated cataract. g. cyclitic membrane. h. retinal detachment.
3) Photophobia, redness, watering.


1) Oedema lids in severe cases with watery discharge. 2) Ciliary and conjunctival congestion. 3) Cornea: May show oedema, KPs- These are small accumulations of cells derived from the uveal tract upon the back of the cornea. They are found in iridocyclitis, and choroidities. In iridocyclitis the nutrition of the corneal endothelium becomes affected so that the cells become sticky and may desquamate in places.

There the cells derived from the uvea tend to stick, forming KPs. The KPs are scattered over a triangular area of the lower part of the cornea (triangle of Arlt) an arrangement due to convection currents in the aqueous and gravitation of the cells towards the bottom of the AC.
Morphological types:

KPs may be fine, coarse, snowball type or mutton fat (these are large greasy plaques with regular outline). KPs may either be fresh (grayish white with regular outline) or old (pigmented brown and serrated margins).


1)In exudative type of inflammation the KPs are composed mainly of lymphocytes and plasma cells. 2)Granulomatous type of uveities- mutton fat KPs are composed of epithelioid and histiocytic mononuclear phagocytes.

KPs may be seen with naked eye- mutton fat KPs. Frequently the KPs cannot be seen even with corneal loupe.

The detection of these KPs with slit lamp is of great value: 1) In diagnosing a quiet uveitis, sympathetic ophthalmitis. 2)KPs are also useful in prophylactic treatment of sympathetic ophthalmitis. 3)KPs indicate whether the uveitis is active or healed.

a- Deep (due to iris being plastered on the lens) b-cells in AC c- aqueous flare

The aqueous humour is almost optically empty. If a spot light of slit lamp be used the presence of colloidal particles such as proteins produces sufficient dispersion of light (Tyndall phenomenon) to make it visible. This is known as aqueous flare and it is present in iridocyclitis due to increased permeability of the vessels to proteins.

5) pupil: Is small, irregular (due to posterior synechiae) and reacts sluggishly to light. This irregularity is exaggerated when pupil is dilated with mydriatic- festooned pupil (resembles a wreath such as is placed on a war memorial). Posterior synechiae may be: a) filiform seen in non granulomatous uveitis. b) broad based posterior synechiae seen in granulomatous uveitis. c) annular or ring synechiae or seclusion pupillae.

In severe cases of plastic iridocyclitis or after recurrent attacks the whole circle of the papillary margin may become tied down to the anterior lens capsule. It is of great danger to the eye since if unrelieved it inevitably leads to secondary glaucoma- the aqueous unable to pass forwards into the anterior chamber, collects behind the iris which becomes bowed forward like a sail, a condition which is called iris bombe. Regarded from in front, the anterior chamber is seen to be funnel shaped, deepest in the centre and shallowest at the periphery.

The filteration angle is then obliterated by the apposition of the iris to the cornea of the periphery where eventually adhesion may from (peripheral anterior synechiae). The drainage of aqueous is obstructed and the tension rises. d) Total posterior synechiae: whole of the posterior surface of the iris is plastered to the lens. The AC is deep uniformly.

OCCLUSIO PUPILLAE (blocked pupil):

In severe cases of irido cyclitis the exudates may cover or may organize across the entire papillary area which becomes ultimately filled by a film of opaque fibrous tissue- the condition is called occlusio pupillae occlusion pupillae and seclusion pupillae often occur together. Occlusion pupillae may occur with total posterior synechiae.

6) IRIS: a- "Muddy iris" delicate pattern or iris crypts become blurred and indistinct. b- nodules- these may be of 2 types: i) pseudo nodules: These consists of accumulations of lymphocytes and epitheloid cells which are deposited on the iris koeppes nodules near the papillary border and Busacca's nodules near the collarette.They disappear without fibrosis. ii)True nodules: These arise in the stroma of the iris and on healing leave behind a scar or an atrophic patch.


Tuberculous nodule 1.Colour Grey or yellow avascular capillaries pass over nodule.

Syphilitic nodule Yellowish-red avascular

Leprosy nodule Pale avascular

Sarcoid nodule

Ophthalmia nodosa

Reddish Pink vascular vascular capillaries pass over and in the nodule


Pupillary or

Pupillary or


Any part

Any part

ciliary region

ciliary region



Grow slowly

Grow relatively quickly

Grow slowly

Grow slowly

Grow slowly

Syphilitic nodule

C- Colour of iris: undergoes change brown iris becomes grayish. White patches of atrophy are seen in late stages. d- Rubeosis iridis: new vessels on iris in chronic iridocyclitis.
7) Lens:

Anterior surface of lens may show patches of pigment where posterior synechiae have been broken by mydriatic.

raised, normal or lowered.

9) CILIARY TENDERNESS: particularly when cyclitis is predominant. Healed iridocyclitis Active iridocyclitis

Diminished vision may be present.

1) symptoms: Pain, redness, photophobia, diminished vision

2)signs: a- ciliary and conjunctival congestion b-KPs grayish white, circular with regular margin. c- Aqueous flare present. d- Ciliary tenderness present.

Eye is white Pigmented KPs with crenated or serrated margin. Absent Absent

COMPLICATIONS: 1) Secondary glaucoma: hypertensive

iridocyclitis. This may be due to: i) clogging of trabecular meshwork by inflammatory exudates- plasmoid aqueous. ii) seclusio pupillae. iii) seclusio pupillae produces iris bombe which gives rise to peripheral anterior synechiae. iv) occlusion pupillae. v) increased capillary hydrostatic pressure leading to increased aqueous formation.

2) Complicated

cataract: Refer lens

3) Cornea:

a) a corneal opacity may result from persistent oedema. b) band shaped keratopathy particularly in cases of Stills disease. c) Retrocorneal membrane due to organization to exudates behind the cornea.
4) Cyclitic membrane:

It is a membrane formed in the anterior vitreous behind the lens due to organization of inflammatory exudates.


1-causes diminision of vision 2- causes traction retinal detachment. 3- one of the causes of pseudoglioma in children.
5) Retinal detachment:

a) due to contraction of cyclitic membrane. b) It may be exudative retinal detachment when iridocyclitis is associated with choroiditis.

Retinal detachment

6) Phthisis bulbi: a) damage to ciliary body occurs due to organization of exudates on the surface. b) contraction of cyclitic membrane may detach the ciliary body. These result in decreased production of aqueous leading to ocular hypotony and phthisis bulbi.

Phthisis bulbi

Differential diagnosis:
Acute conjunctivitis
1-Pain Foreign body or gritty sensation No pain

Acute Iridocyclitis Acute congestive glaucoma

Pain radiating along 1ST division (ophthalmic) Of 5th nerve Severe neuralgic pain radiating along all 3 divisions of 5th nerve associated with nausea and vomiting. Gross diminision sometimes reduced to just PL.


Not affected, may be slightly blurred.

Moderate diminision Watery (serous)



Watery (serous).


Conjunctival congestion

Ciliary and conjunctival congestion

Ciliary and conjunctival congestion


Clear sensation normal

Mild oedema sensation Normal KPs on back of cornea

Cloudy or steamy insensitive.


Normal size reaction Normal normal absent

Small, irregular sluggish reaction Low, normal or raised present

Large, vertically oval fixed. Markedly raised eye stony hard. Absent.

8-Intraocular pressure 9-Ciliary tenderness

Acute iridocyclitis should also be differentiated from other causes of red- corneal ulcer, scleritis etc.


1.General examination for evidence of leprosy, tuberculosis, syphilis, rheumatoid arthritis etc. 2.Urine albumin, sugar and microscopic examination for pus cells. 3.Stools- ova and cyst. 4.Tuberculosis Mantoux test, X-ray chest 5.Syphilis blood VDRL 6.Rheumatoid arthritis- Rose- waaler test. 7.Toxoplasmosis: HA test, dye test and ELISA test. 8.Thorough dental, ENT, and gynaecological check up to rule out septic foci


i. Cycloplegic mydriatic therapy: a) 1% atropine drops or ointment t.d.s. This is the most important drug in the treatment of iridocyclitis. It acts as follows: 1-puts the iris and ciliary body at rest. 2-diminishes congestion of iris and ciliary body. 3-prevents the formation of posterior synechiae or tends to break those already formed. 4-has mild anaesthetic action.

b) In cases where pupil does not dilate with atropine, subconjunctival injection of 0.3 cc mydricaine is given (this contains atropine, adrenaline and procaine and is a potent mydriatic). c) In cases of atropine allergy- 2% homatropine, 1% cyclopentolate, 0.5% hyoscine may be used.
ii) Cortisone drops or ointment 3-6 times a day. This is useful because of the allergic nature of the disease. In severe cases subconjunctival injection of Dexamethasone is given.

NB: Indiscriminate and long term use of corticosteroid may cause rise in intraocular pressure. iii) Hot fomentation t.d.s. iv) dark glasses.
II) General

1)Treatment of causative factor- T.B., (1 infected teeth etc., should be treated or eliminated. 2) Analgesics- paracetamol. 3) Systemic corticosteroid in severe cases of iridocyclitis or when associated with choroiditis.

III) Treatment of complications:

1) secondary glaucoma: a) local use of 1% atropine, corticosteroid, hot fomentation. b) Tab. Diamox 250 mg t.d.s. c) surgical- paracentesis. Peripheral iridectomy can be done to relieve the papillary block in cases of iris bombe. In cases with extensive peripheral anterior synechiae due to iris bombe, trabeculectomy may be required.

Earlier, Fuchs four dot iridotomy was done by passing Von Graefes knife across AC puncturing and counter puncturing both cornea and iris. This is now of historical interest only. 2) Complicated cataract: Refer lens chapter.

Etiology: I-Infection 1) Endogenous a. virus - Rubella. b. bacteria - TB, syphilis. c. fungi - histoplasmosis. d. protozoa - toxoplasmosis. e. Nematodes - ankylostomiasis, filariasis. 2) Exogenous : perforating injury; perforated corneal ulcer ect, these lead to suppurative choroiditis , endophthalmitis and even panophthalmitis. 3) Secondary : spread of inflammation from posterior scleritis II) Idiopathic: sarcoidosis ,Vogt koyanagi - haradas disease


Inflammation of choroids:

Suppurative choroiditis: this is acute type of (1 inflammation and usually spreads over whole of choroids, ultimately leads to panophthalmitis. 2) Non suppurative choroiditis: a) Granulomatous choroiditis : it is associated with presence causative organisms . b) Exudative choroditis : this is non specific plastic inflammatory reaction characterized with excessive exudation and poor cellular response . this is an allergic response of the sensitive tissue to the presence of bacterial toxins .

Fundus examination shows : 1) vitreous haze and vitreous opacities. 2) patches of choroiditis. These patches are yellowish or yellowish white with ill defined fluffy margin. 3) Later the exudates undergo resolution by fibrosis and the patches become white with well defined margin. Complication : 1) complicated cataract result from defective nourishment of the lens. 2) Exudative retinal detachment due to exudation of fluid between pigment epithelium and rods and cones .

3) optic neuritis : result when the inflammatory patch is close to optic disc . 4) consecutive optic atrophy : from the destruction of retinal ganglion cells .

Varieties of posterior uveitis :

It classified according to the situation and character of the patches: 1.Circumscribed choroidities. 2.Diffuse choroiditis. 3.Disseminated choroiditis. 4.Anterior choroiditis. 5.Central choroiditis. 6.juxta papillary choroiditis of Jensen.

Symptoms :
Visual disturbances are common: 1) visual acuity may become defective more markedly when: a- the lesion is in the central area near the posterior pole. b- cloudiness of vitreous is present. 2) metamorphpsia distortion of object may be seen . 3) micropsia and macropsia objects appear smaaler or bigger, due to separation or crowding of retinal elements respectively. 4) photopsiasubjective flashes of light, due to irritation or retinal elements by exudates.

5) positive scotoma: perception of black spot before the eye. 6) negative scotoma: hiatus in the field of vision ( patient is not aware of this ). specific type of posterior uveitis : Tuberculosis of the choroid occurs in acute milliary and chronic forms: 1) acute - in milliary tuberculosis there are multiple small spots scattered all over the fundus varying in size from pin point to 2mm diameter. 2) chronic tuberculosis - may result in: diffuse choroiditis.- disseminated choroiditis. - solitary or conglomerate tubercle.

Differences between active and healed choroiditis :

Active choroiditis Healed choroiditis

1-Vitreous haze is present. 2-Yellow patch with feathery margin. 3-Borders are not pigmented. 4-Produces passive scotoma due to inflammatory exudates in front of light sensitive element. 5-Underlying choroidal vessels obscured. 6- Often requires treatment.

Vitreous is clear. White patch with circumscribed margin. Borders are pigmented. Produce negative scotoma due to destruction of photoreceptors.
Large choroidal vessels. No treatment required.

Treatment of choroiditis: usually unsatisfactory as the retinal damage caused is permanent.

1) local: - 1% atropine . - posterior subtenon injection of corticosteroid. - hot fomintation .

2) systemic: - corticosteroid by mouth .

- specific treatment of cause syphilis Inj . Benzathine penicillin. - tuberculosis anti tubercular treatment.

congenital abnormalities of the uveal tract: persistent pupillary membrane - in the early foetal eye pupil is covered over by a delicate membrane known as pupillary membrane. conginital aniridia : failure of iris to develop. There is usually associated nystagmus and foveal hypoplasia. heterochromia iridis : part of the same iris usually a sector may differ in colour from the remainder . heterochromia iridum : one iris may have different colour from the other. behcets syndrome: it is an acute iridocyclitis seen in young adult associated with:- hypopyon, ulcerative lesion in mouth.

Congenital aniridia

Iris coloboma

heerfordts disease: it is a bilateral affection characterized by involvement of the entire uveal tract, the parotid gland and cranial nerve. Vogt koyanagi haradas syndrome Fuchs heterochromic iridocyclitis Sympathetic ophthalmitis Rubeosis iridis: it is characterized by the development of new and enlarged vessels on iris.and is seen in: i) diabetes mellitus ii)After central retinal vein occlusion iii)After central retinal artery occlusion In cases of diabetes mellitus with rubeosis and mature cataract iridectomy may have to be omitted or peripheral iridectomy done in an area where rubeosis is not present Complication:secondary glaucoma due to fibro vascular membrane blocking trabecular meshwork and due to recurrent hyphaema

Rubeosis iridis

Vogt koyanagi haradas syndrome

Nodules on the iris are seen in the following disease 1) Iridocyclitis: due to tuberculosis, syphilis, leprosy 2)Iris melanoma:usually seen in people between 40 and 50 years and presents as a solitary pigmented nodule on the lower half of iris feature that should arouse suspicion of malignancy include:
- ectropion uveae - pupillary distortion - neovascularization - raised IOP - increase in nodule size 3) Lisch nodules

4) Juvenile xanthogranuloma

Iris melanoma

Lisch nodules

Juvenile xanthogranuloma

Definition: An intense suppurative inflammation of the entire uveal tract which fills the eye ball with pus, extends to all the structures of the eye and ends in complete destruction of this organ. Etiology: Infection is by pyogenic organisms: 1) ps. Pyocyanea 2) pneumococcus 3) staph 4)strept 5) E. coli 6) gonococcus

i) Exogenous infection: 1) perforated corneal ulcer 2) penetrating wounds 3) intraocular operation ii) Endogenous infection Results from septic embolism of a retinal or a choroid vessels. Such cases occur in the course of infectious disease pneumonia, meningitis

symptoms: 1) the disease is usually start with fever, headache, vomiting 2) severe pain in the eye ball. 3) complete loss of sight.

Sign: it is usually unilateral 1) lids swollen and red. 2)congunctiva- intense ciliary and congunctiva congestion, chemosis of conjunctiva. 3) slight proptosis and limitation of movement of eyeball. 4) perforated corneal ulcer, and cloudy yellow ring infiltration occur.

5) AC filled with pus. 6) increased I.O.P . 7) in severe cases the pus bursts through the wall of the globe.

I- At early stage : 1) intensive treatment by antibiotics both local and systemic. 2) analgesics- tap.paracetamol. 3)hot fomentation.

II- As soon as it becomes evident that the eye cannot be saved evisceration is done. Frill excision: if the entire sclera is left. There is considerable pain, reaction and delayed healing. It is not considered advisable to enucleate in cases of panophthalmitis on account of the danger of causing meningitis. Prognosis: is always bad. However the condition does not cause sympathetic ophthalmitis.

Hetrochromia waardenhurgs

Iris prolapse

Iris hemangioma

Iris cyst

Iris nevus

Iris tumor

Iris neovascuralization

Herberts pits



Koeppe's nodule

Synechiae posterior

pseudo exfoliation