Ординатура / Офтальмология / Английские материалы / Comprehensive Ophthalmology_Khurana_2007

Fig. 6.3. Non-necrotizing anterior diffuse scleritis.

Fig. 6.4. Non-necrotizing anterior nodular scleritis.

3.Anterior necrotizing scleritis with inflammation.

It is an acute severe form of scleritis characterised by intense localised inflammation associated with areas of infarction due to vasculitis (Fig. 6.5). The affected necrosed area is thinned out and sclera becomes transparent and ectatic with uveal tissue shining through it. It is usually associated with anterior uveitis.

4.Anterior necrotizing scleritis without inflammation (scleromalacia perforans). This specific entity typically occurs in elderly females usually suffering from long-standing rheumatoid arthritis. It is characterised by development of yellowish patch of melting sclera (due to obliteration of arterial supply); which often together with the overlying episclera and conjunctiva completely separates from the surrounding normal sclera. This sequestrum of sclera becomes dead white in colour, which eventually absorbs leaving behind it a large punched out area of thin sclera through which the uveal tissue shines (Fig. 6.6). Spontaneous perforation is extremely rare.

5.Posterior scleritis. It is an inflammation involving the sclera behind the equator. The condition is frequently misdiagnosed. It is characterised by features of associated inflammation of adjacent structures, which include: exudative retinal detachment, macular oedema, proptosis and limitation of ocular movements.

Complications

These are quite common with necrotizing scleritis and include sclerosing keratitis, keratolysis, complicated cataract and secondary glaucoma.

Investigations

Following laboratory studies may be helpful in identifying associated systemic diseases or in establishing the nature of immunologic reaction:

1.TLC, DLC and ESR

2.Serum levels of complement (C3), immune complexes, rheumatoid factor, antinuclear antibodies and L.E cells for an immunological survey.

3.FTA - ABS, VDRL for syphilis.

4.Serum uric acid for gout.

5.Urine analysis.

6.Mantoux test.

7.X-rays of chest, paranasal sinuses, sacroiliac joint and orbit to rule out foreign body especially in patients with nodular scleritis.

Treatment

(A)Non-necrotising scleritis. It is treated by topical steroid eyedrops and systemic indomethacin 100 mg daily for a day and then 75 mg daily until inflammation resolves.

(B)Necrotising scleritis. It is treated by topical steroids and heavy doses of oral steroids tapered slowly. In non-responsive cases, immuno-suppressive agents like methotrexate or cyclophos-phamide may be required. Subconjunctival steroids are contraindicated because they may lead to scleral thinning and perforation.

BLUE SCLERA

It is an asymptomatic condition characterised by marked, generalised blue discolouration of sclera due

to thinning (Fig. 6.7). It is a typical association of osteogenesis imperfecta. Its other causes are Marfan's syndrome, Ehlers-Danlos syndrome, pseudoxanthoma elasticum, buphthalmos, high myopia and healed scleritis.

STAPHYLOMAS

Staphyloma refers to a localised bulging of weak and thin outer tunic of the eyeball (cornea or sclera), lined by uveal tissue which shines through the thinned out fibrous coat.

Types

Anatomically it can be divided into anterior, intercalary, ciliary, equatorial and posterior staphyloma (Fig. 6.8).

Fig. 6.7. Blue sclera.

1.Anterior staphyloma. (see page 122)

2.Intercalary staphyloma. It is the name given to the localised bulge in limbal area lined by root of iris (Figs. 6.8A and 6.9). It results due to ectasia of weak scar tissue formed at the limbus, following healing of a perforating injury or a peripheral corneal ulcer. There may be associated secondary angle closure glaucoma,

Fig. 6.9. Intercalary staphyloma.

Treatment consists of localised staphylectomy under heavy doses of oral steroids.

3.Ciliary staphyloma. As the name implies, it is the bulge of weak sclera lined by ciliary body. It occurs about 2-3 mm away from the limbus (Figs. 6.8B and 6.10). Its common causes are thinning of sclera following perforating injury, scleritis and absolute glaucoma.

4.Equatorial staphyloma. It results due to bulge of sclera lined by the choroid in the equatorial region (Fig. 6.8C). Its causes are scleritis and degeneration of sclera in pathological myopia. It occurs more commonly at the regions of sclera which are perforated by vortex veins.

5.Posterior staphyloma. It refers to bulge of weak sclera lined by the choroid behind the equator (Fig. 6.8D). Here again the common causes are pathological myopia, posterior scleritis and perforating injuries. It is diagnosed on ophthalmoscopy. The area is

Fig. 6.10. Ciliary staphyloma.

excavated with retinal vessels dipping in it (just like marked cupping of optic disc in glaucoma) (Fig. 6.11). Its floor is focussed with minus number lenses in ophthalmo-scope as compared to its margin.

Fig. 6.11. Fundus photograph showing excavation of retinal tissue in posterior staphyloma.

APPLIED ANATOMY

Uveal tissue constitutes the middle vascular coat of the eyeball. From anterior to posterior it can be divided into three parts, namely, iris, ciliary body and choroid. However, the entire uveal tract is developmentally, structurally and functionally one indivisible structure.

THE IRIS

Iris is the anterior most part of the uveal tract. It is a thin circular disc corresponding to the diaphragm of a camera. In its centre is an aperture of about 4-mm diameter called pupil which regulates the amount of light reaching the retina. At the periphery, the iris is attached to the middle of anterior surface of the ciliary body. It divides the space between the cornea and lens into anterior and posterior chambers.

Macroscopic appearance. Anterior surface of the iris can be divided into a ciliary zone and a pupillary zone by a zigzag line called collarette (Fig. 7.1).

1.Ciliary zone. It presents series of radial streaks due to underlying radial blood vessels and crypts which are depressions where superficial layer of

iris is missing. Crypts are arranged in two rows —the peripheral present near the iris root and the central present near the collarette.

2.Pupillary zone. This part of the iris lies between the collarette and pigmented pupillary frill and is relatively smooth and flat.

Microscopic structure (Fig. 7.2) . The iris consists of four layers which from anterior to posterior are :

Fig. 7.1. Macroscopic appearance of anterior surface of iris.

1.Anterior limiting layer. It is the anterior most condensed part of the stroma. It consists of melanocytes and fibroblasts. Previously this layer was called endothelial layer of iris which was a misnomer. This layer is deficient in the areas of crypts. The definitive colour of the iris depends on this layer. In blue iris this layer is thin and contains few pigment cells. While in brown iris it is thick and densely pigmented.

2.Iris stroma. It consists of loosely arranged collagenous network in which are embedded the sphincter pupillae muscle, dilator pupillae muscle, vessels, nerves, pigment cells and other cells which include lymphocytes, fibroblasts, macrophages and mast cells.

The sphincter pupillae muscle forms one millimetre broad circular band in the pupillary part of the iris. It is supplied by parasympathetic fibres through third nerve. It constricts the pupil.

The dilator pupillae muscle lies in the posterior part of stroma of the ciliary zone of iris. Its myofilaments are located in the outer part of the cells of anterior pigment epithelial layer. It is supplied by cervical sympathetic nerves and dilates the pupil.

3.Anterior epithelial layer. It is anterior continuation of the pigment epithelium of retina

and ciliary body. This layer gives rise to the dilator pupillae muscle.

4.The posterior pigmented epithelial layer. It is anterior continuation of the non-pigmented epithelium of ciliary body. At the pupillary margin it forms the pigmented frill and becomes continuous with the anterior pigmented epithelial layer.

CILIARY BODY

Ciliary body is forward continuation of the choroid at ora serrata. In cut-section, it is triangular in shape. The anterior side of the triangle forms the part of the angle of anterior and posterior chambers. In its middle the iris is attached. The outer side of the triangle lies against the sclera with a suprachoroidal space in between. The inner side of the triangle is divided into two parts. The anterior part (about 2 mm) having finger-like ciliary processes is called pars plicata and the posterior smooth part (about 4 mm) is called pars plana (Fig. 7.2).

Microscopic structure (Fig. 7.2). From without inwards ciliary body consists of following five layers:

1.Supraciliary lamina. It is the outermost condensed part of the stroma and consists of pigmented collagen fibres. Posteriorly, it is the continuation of suprachoroidal lamina and anteriorly it becomes continuous with the anterior limiting membrane of iris.

2.Stroma of the ciliary body. It consists of connective tissue of collagen and fibroblasts. Embedded in the stroma are ciliary muscle, vessels, nerves, pigment and other cells.

Ciliary muscle occupies most of the outer part of ciliary body. In cut section it is triangular in shape. It is a non-striated muscle having three parts: (i) the longitudinal or meridional fibres which help in aqueous outflow; (ii) the circular fibres which help in accommodation; and (iii) the radial or oblique fibres act in the same way as the longitudinal fibres. Ciliary muscle is supplied by parasympathetic fibres through the short ciliary nerves.

3.Layer of pigmented epithelium. It is the forward continuation of the retinal pigment epithelium. Anteriorly it is continuous with the anterior pigmented epithelium of the iris.

4.Layer of non-pigmented epithelium. It consists mainly of low columnar or cuboidal cells, and is the forward continuation of the sensory retina. It continues anteriorly as the posterior (internal) pigmented epithelium of the iris.

5.Internal limiting membrane. It is the forward continuation of the internal limiting membrane of the retina. It lines the non-pigmented epithelial layers.

Ciliary processes. These are finger-like projections from the pars plicata part of the ciliary body. These are about 70-80 in number. Each process is about 2-mm long and 0.5-mm in diameter. These are white in colour.

Structure. Each process is lined by two layers of epithelial cells. The core of the ciliary process contains blood vessels and loose connective tissue. These processes are the site of aqueous production.

Functions of ciliary body. (i) Formation of aqueous humour. (ii) Ciliary muscles help in accommodation.

CHOROID

Choroid is the posterior most part of the vascular coat of the eyeball. It extends from the optic disc to ora serrata. Its inner surface is smooth, brown and lies in contact with pigment epithelium of the retina. The outer surface is rough and lies in contact with the sclera.

Microscopic structure (Fig. 7.3). From without inwards choroid consists of following three layers:

Fig. 7.3. Microscopic structure of the choroid.

1.Suprachoroidal lamina. It is a thin membrane of condensed collagen fibres, melanocytes and fibroblasts. It is continuous anteriorly with the supraciliary lamina. The potential space between this membrane and sclera is called suprachoroidal space which contains long and short posterior ciliary arteries and nerves.

2.Stroma of the choroid. It consists of loose collagenous tissue with some elastic and reticulum fibres. It also contains pigment cells and plasma cells. Its main bulk is formed by vessels which are arranged in three layers. From without inwards these are: (i) layer of large vessels (Haller’s layer),

(ii)layer of medium vessels (Sattler’s layer) and

(iii)layer of choriocapillaris which nourishes the outer layers of the retina.

3.Basal lamina. It is also called Bruch’s membrane and lines the layer of choriocapillaris. It lies in approximation with pigment epithelium of the retina.

Blood supply of the uveal tract

Arterial supply. The uveal tract is supplied by three sets of arteries (Fig. 7.4):

1.Short posterior ciliary arteries. These arise as two trunks from the ophthalmic artery; each trunk divides into 10-20 branches which pierce the sclera around the optic nerve and supply the choroid in a segmental manner.

2.Long posterior ciliary arteries. These are two in number, nasal and temporal. These pierce the sclera obliquely on medial and lateral side of the optic nerve and run forward in the suprachoroidal space to reach the ciliary muscle, without giving any branch. At the anterior end of ciliary muscle these anastomose with each other and with the anterior ciliary arteries; and gives branches which supply the ciliary body.

3.Anterior ciliary arteries. These are derived from the muscular branches of ophthalmic artery. These are 7 in number; 2 each from arteries of superior rectus, inferior rectus, and medial rectus muscle and one from that of lateral rectus muscle. These arteries pass anteriorly in the episclera, give branches to sclera, limbus and conjunctiva; and ultimately pierce the sclera near the limbus to enter the ciliary muscle; where they anastomose with the two long posterior ciliary arteries to form the circulus arteriosus major, near the root of iris. Several branches arise from the circulus arteriosus major and supply the ciliary processes

(one branch for each process). Similarly, many branches from this major arterial circle run radially through the iris towards pupillary margin, where they anastomose with each other to form circulus arteriosus minor.

Venous drainage. A series of small veins which drain blood from the iris, ciliary body and choroid join to form the vortex veins. The vortex veins are four in number–superior temporal, inferior temporal, superior nasal and inferior nasal. They pierce the sclera behind the equator and drain into superior and inferior ophthalmic veins which in turn drain into the cavernous sinus.

CONGENITAL ANOMALIES OF

UVEAL TRACT

HETEROCHROMIA OF IRIS

It refers to variations in the iris colour and is a common congenital anomaly. In heterochromia iridium colour of one iris differs from the other. Sometimes, one sector of the iris may differ from the remainder of iris; such a condition is called heterochromia iridis. Congenital heterochromia must be differentiated from the acquired heterochromia seen in heterochromic cyclitis, siderosis and malignant melanoma of iris.

CORECTOPIA

It refers to abnormally eccentric placed pupil. Normally pupil is placed slightly nasal to the centre.

POLYCORIA

In this condition, there are more than one pupil.

CONGENITAL ANIRIDIA (IRIDREMIA)

It refers to congenital absence of iris. True aniridia, i.e., complete absence of the iris is extremely rare. Usually, a peripheral rim of iris is present and this condition is called ‘Clinical aniridia'. Zonules of the lens and ciliary processes are often visible. The condition is usually familial and may be associated with glaucoma due to angle anomalies.

PERSISTENT PUPILLARY MEMBRANE

It represents the remnants of the vascular sheath of the lens. It is characterised by stellate-shaped shreds of the pigmented tissue coming from anterior surface of the iris (attached at collarette) (Fig. 7.5). These float freely in the anterior chamber or may be attached to the anterior surface of the lens.

Fig. 7.5. Persistent pupillary membrane.

CONGENITAL COLOBOMA OF THE UVEAL TRACT

Congenital coloboma (absence of tissue) of iris (Fig. 7.6), ciliary body and choroid (Fig. 7.7) may be seen in association or independently. Coloboma may be typical or atypical.

Fig. 7.6. Typical coloboma of the iris.

Fig. 7.7. Coloboma of the choroid.

Typical coloboma is seen in the inferonasal quadrant and occurs due to defective closure of the embryonic fissure.

Atypical coloboma is occasionally found in other positions.

Complete coloboma extends from pupil to the optic

nerve, with a sector-shaped gap occupying about one-eighth of the circumference of the retina, choroid, ciliary body, iris, and causing a corresponding indentation of the lens where the zonular fibres are missing.

UVEITIS

GENERAL CONSIDERATIONS

The term uveitis strictly means inflammation of the uveal tissue only. However, practically there is always some associated inflammation of the adjacent structures such as retina, vitreous, sclera and cornea. Due to close relationship of the anatomically distinct parts of the uveal tract, the inflammatory process usually tends to involve the uvea as a whole.

CLASSIFICATION

I. ANATOMICAL CLASSIFICATION

1.Anterior uveitis. It is inflammation of the uveal tissue from iris up to pars plicata of ciliary body. It may be subdivided into :

Iritis, in which inflammation predominantly affects the iris.

Iridocyctitis in which iris and pars plicata part of ciliary body are equally involved, and

Cyclitis, in which pars plicata part of ciliary body is predominantly affected.

2.Intermediate uveitis. It includes inflammation of the pars plana and peripheral part of the retina and underlying ‘choroid’. It is also called ‘pars planitis'.

3.Posterior uveitis. It refers to inflammation of the choroid (choroiditis). Always there is associated inflammation of retina and hence the term ‘chorioretinitis’ is used.

4.Panuveitis. It is inflammation of the whole uvea.

II. CLINICAL CLASSIFICATION

1.Acute uveitis. It has got a sudden symptomatic onset and the disease lasts for about six weeks to 3 months.

2.Chronic uveitis. It frequently has an insiduous and asymptomatic onset. It persists longer than 3 months to even years and is usually diagnosed when it causes defective vision.

III. PATHOLOGICAL CLASSIFICATION

1.Suppurative or purulent uveitis.

2.Non-suppurative uveitis. It has been further subdivided in two groups (Wood’s classification).

(i)Non-granulomatous uveitis, and

(ii)Granulomatous uveitis

IV. ETIOLOGICAL (DUKE ELDER'S)

CLASSIFICATION

1.Infective uveitis

2.Allergic uveitis

3.Toxic uveitis

4.Traumatic uveitis

5.Uveitis associated with non-infective systemic diseases

6.Idiopathic uveitis

ETIOLOGY OF UVEITIS

Despite a great deal of experimental research and many sophisticated methods of investigations, etiology and immunology of the uveitis is still largely not understood. Even today, the cause of many clinical conditions is disputed (remains presumptive) and in many others etiology is unknown. The etiological concepts of uveitis as proposed by Duke Elder, in general, are discussed here.

1. Infective uveitis. In this, inflammation of the uveal tissue is induced by invasion of the organisms. Uveal infections may be exogenous, secondary or endogenous.

i Exogenous infection wherein the infecting organisms directly gain entrance into the eye from outside. It can occur following penetrating injuries, perforation of corneal ulcer and postoperatively (after intraocular operations). Such infections usually result in an acute iridocyclitis of suppurative (purulent) nature, which soon turns into endophthalmitis or even panophthalmitis.

iiSecondary infection of the uvea occurs by spread of infection from neighbouring structures, e.g., acute purulent conjunctivitis. (pneumo-coccal and gonococcal), keratitis, scleritis, retinitis, orbital cellulitis and orbital thrombophlebitis.

iiiEndogenous infections are caused by the entrance of organisms from some source situated elsewhere in the body, by way of the bloodstream. Endogenous infections play important role in the inflammations of uvea.

Types of infectious uveitis. Depending upon the causative organisms, the infectious uveitis may be classified as follows:

i.Bacterial infections. These may be granulomatous e.g., tubercular, leprotic, syphilitic,

brucellosis or pyogenic such as streptococci, staphylococci, pneumococci and gonococcus.

ii.Viral infections associated with uveitis are herpes simplex, herpes zoster and cytomegalo inclusion virus (CMV).

iii.Fungal uveitis is rare and may accompany systemic aspergillosis, candidiasis and blastomycosis. It also includes presumed ocular histoplasmosis syndrome.

iv.Parasitic uveitis is known in toxoplasmosis, toxocariasis, onchocerciasis and amoebiasis.

v.Rickettsial uveitis may occur in scrub typhus and epidemic typhus.

2. Allergic (hypersensitivity linked) uveitis. Allergic uveitis is of the commonest occurrence in clinical practice. The complex subject of hypersensitivity linked inflammation of uveal tissue is still not clearly understood. It may be caused by the following ways:

i.Microbial allergy. In this, primary source of infection is somewhere else in the body and the escape of the organisms or their products into the bloodstream causes sensitisation of the uveal tissue with formation of antibodies. At a later date a renewal of infection in the original focus may again cause dissemination of the organisms or their products (antigens); which on meeting the sensitised uveal tissue excite an allergic inflammatory response.

Primary focus of infection can be a minute tubercular lesion in the lymph nodes or lungs. Once it used to be the most common cause of uveitis worldwide, but now it is rare. However, in developing countries like India tubercular infections still play an important role. Other sources of primary focus are streptococcal and other infections in the teeth, paranasal sinuses, tonsils, prostate, genitals and urinary tract.

ii.Anaphylactic uveitis. It is said to accompany the systemic anaphylactic reactions like serum sickness and angioneurotic oedema.

iii.Atopic uveitis. It occurs due to airborne allergens and inhalants, e.g., seasonal iritis due to pollens. A similar reaction to such materials as danders of cats, chicken feather, house dust, egg albumin and beef proteins has also been noted.

iv.Autoimmune uveitis. It is found in association with autoimmune disorders such as Still’s disease, rheumatoid arthritis, Wegener’s granulomatosis, systemic lupus erythematosus, Reiter’s disease and so on.

In phacoanaphytic endophthalmitis, lens proteins play role of autoantigens. Similarly, sympathetic ophthalmitis has been attributed to be an autoimmune reaction to uveal pigments, by some workers.

v. HLA-associated uveitis: Human leucocytic antigens (HLA) is the old name for the histocompatibility antigens. There are about 70 such antigens in human beings, on the basis of which an individual can be assigned to different HLA phenotypes. Recently, lot of stress is being laid on the role of HLA in uveitis, since a number of diseases associated with uveitis occur much more frequently in persons with certain specific HLAphenotype. A few examples of HLA-associated diseases with uveitis are as follows:

HLA-B27. Acute anterior uveitis associated with ankylosing spondylitis and also in Reiter’s syndrome.

HLA-B5: Uveitis in Behcet’s disease.

HLA-DR4 and DW15: Vogt Koyanagi Harada’s disease.

3. Toxic uveitis. Toxins responsible for uveitis can be endotoxins, endocular toxins or exogenous toxins.

i.Endotoxins, produced inside the body play a major role. These may be autotoxins or microbial toxins (produced by organisms involving the body tissues). Toxic uveitis seen in patients with acute pneumococcal or gonococcal conjunctivitis and in patients with fungal corneal ulcer is thought to be due to microbial toxins.

ii.Endocular toxins are produced from the ocular tissues. Uveitis seen in patients with blind eyes, long-standing retinal detachment and intraocular haemorrhages is said to be due to endocular toxins. Other examples are uveitis associated with intraocular tumours and phacotoxic uveitis.

iii.Exogenous toxins causing uveitis are irritant chemical substances of inorganic, animal or vegetative origin. Certain drugs producing uveitis (such as miotics and cytotoxic drugs) are other examples of exogenous toxins.

4. Traumatic uveitis. It is often seen in accidental or operative injuries to the uveal tissue. Different mechanisms which may produce uveitis following trauma include: