Ординатура / Офтальмология / Английские материалы / Ocular Pathology_6th edition_Yanoff, Sassani_2009

2.Ocular manifestations, most commonly a follicular conjunctivitis, usually occur in association with infectious mononucleosis.

3.X-linked lymphoproliferative syndrome (XLP), one of the reactive histiocytic disorders, is a disease in which a primary EBV infection results in a fatal outcome from infectious mononucleosis, aplastic anemia, malignant lymphoma, and hypogammaglobulinemia in most male patients who have the XLP gene.

Epithelial keratitis, episcleritis, iritis, uveitis, dacryoadenitis, cranial nerve palsies, Sjögren’s syndrome, and Parinaud’s oculoglandular syndrome (see p. 232 in Chapter 7) may also occur. Also, the Fas (also known as Apo1 and CD95) antigen, a cell surface receptor involved in apoptotic cell death, may be defective (through a deletion) in XLP, resulting in incomplete elimination of peripheral autoreactive cells.

4.Histologically, a chronic nongranulomatous inflammation is seen.

C.SSPE (Fig. 3.7)

1.SSPE, caused by measles slow virus infection, is a chronic, progressive disease of the central nervous system in children and young adults, which produces an intracellular infection of brain, retina, and lymphoid tissue.

2.The disease usually emerges 5 to 7 years after the child has had an uneventful measles (rubeola) infection.

SSPE has been reported in a young (20 years of age) male intravenous drug abuser.

a.Patients have high titers of measles antibody in their serum and cerebrospinal fluid.

b.Measles antigen can be demonstrated in brain tissue by immunofluorescence.

3.The ocular findings consist mainly of macular degeneration, optic atrophy, and peripheral retinochoroidal lesions.

The ophthalmologic signs and symptoms may antedate those of the central nervous system by as long as 2 years.

4.Histologically, the neural retina is necrotic, is infiltrated by lymphocytes, and shows conglomerations of multinucleated cells. Intranuclear inclusion bodies in retinal cells can be seen with light and electron microscopy.

II.Local ocular (nonsystemic) syndromes such as pars planitis and FHI may cause a nonsuppurative, chronic nongranulomatous uveitis.

A.Pars planitis (intermediate uveitis, peripheral uveitis, chronic cyclitis)

1.Pars planitis is a chronic process, usually of children and young adults, that consists of vitreous opacities, exudation, and organization of the vitreous base

(“snowbanking”) in the region of the pars plana, and neural retinal edema, especially of the posterior pole (cystoid macular edema).

A 36-kD protein (p-36) is elevated in the blood of many patients with active pars planitis. A pars planitis-like picture may be seen in cat-scratch disease. A significant association also exists between pars planitis and serum HLA-DR15 (HLA-DR15 specificity has been associated with other entities such as multiple sclerosis, idiopathic optic neuritis, and narcolepsy).

Relative sparing of the anterior chamber occurs.

Cataract and cystoid macular edema are common complications. Uncommon complications include band keratopathy, glaucoma, neural retinal detachment, retinoschisis, vitreous hemorrhage, and neural retinal hemorrhage. A link may exist between pars planitis and multiple sclerosis, especially when retinal periphlebitis is present at the time of diagnosis of pars planitis (multiple sclerosis develops in perhaps 15% of patients with pars planitis when they are followed for at least 8 years).

2.Histologically, a chronic nongranulomatous inflammation of the vitreous base, retinal perivasculitis, and microcystoid degeneration of the macular retina are seen.

a.The snowbank noted clinically corresponds microscopically to a loose fibrovascular layer in a condensed vitreous, containing occasional fibrocyte-like cells and scattered mononuclear inflammatory cells adjacent to a hyperplastic, nonpigmented pars plana epithelium.

b.The layer appears continuous with similar preretinal fibroglial membranes.

B.Fuchs’ heterochromic iridocyclitis (FHI) (Figs 3.8 and

3.9)

1.FHI, a condition of unknown cause (although persistence of the rubella virus has been implicated as the cause) consists of a unilateral, chronic, mild iridocyclitis with characteristic, translucent, stellate,

relatively unchanging keratic precipitates; heterochromia iridum with the involved iris becoming the lighter iris; and cataract and glaucoma development in the hypochromic eye.

The hypochromia of the involved eye is caused by iris stromal atrophy with loss of stromal pigment (the atrophy and depigmentation are not limited to the iris but are also found in the surrounding ocular wall). The iris stromal atrophy may become so severe that the iris pigment epithelium can be observed directly. The result is a paradoxical heterochromia with the involved eye becoming the darker eye. The glaucoma is probably caused by a combination of neovascularization of the anterior-chamber angle, a trabeculitis, and possibly an associated atrophy of the uveal portion of the drainage angle.

2.Characteristically, in spite of the chronic uveitis and iris neovascularization, anterior and posterior synechiae do not occur (even though a cataract forms), and intraocular surgery is tolerated well.

A subgroup of FHI has an association, which may be causal, with toxoplasmic retinochoroiditis, toxocariasis, and herpetic ocular infections.

3.White, opalescent iris nodules may develop in black patients.

4.Histologically, a chronic nongranulomatous inflammatory reaction is seen in the iris, ciliary body, and trabecular meshwork.

Plasma cells and Russell bodies are prominent in the iris stroma. The Russell bodies may be seen clinically with the slit lamp as subtle iris crystals.

a.Inflammatory membranes are common over the anterior surface of the iris and anterior face of the ciliary body.

b.A fine neovascularization of the anterior surface of the iris and anterior-chamber angle may be present.

The iris neovascularization is quite fine and just within the anterior-border layer of the iris. Anterior-segment perfusion defects and iris vasculature leakage may be seen. Chronic anterior-segment ischemia, therefore, may play a role in the development of iris neovascularization. Fine, translucent, stellate keratic precipitates, observed clinically, have their counterpart in small clumps of mononuclear cells, lymphocytes, and macrophages on the posterior surface of the cornea.

c.The iris stroma and the pigment epithelium show atrophy with loss of pigment, especially in the stroma and the posterior layer of pigment epithelium.

III.Systemic syndromes such as Reiter’s syndrome, rheumatoid arthritis, and Crohn’s disease

A. Reiter’s syndrome

1.The classic triad of nonbacterial urethritis, conjunctivitis or iridocyclitis, and arthritis characterize Reiter’s syndrome.

2.HLA-B27 is positive in a high percentage of patients.

3.Bilateral mucopurulent conjunctivitis is present in most cases, whereas iridocyclitis tends to be seen only in recurrent cases.

Rarely, a keratoconjunctivitis occurs. Epithelial erosions and pleomorphic infiltrates in the anterior stroma characterize the keratitis.

4.Histologically, edema and a lymphocytic and neutrophilic inflammatory infiltrate are noted in the conjunctiva.

B.Rheumatoid arthritis

1.Ankylosing spondylitis has a 10% to 15% prevalence of uveitis, and Still’s disease has a 15% to 20% prevalence.

2.Juvenile rheumatoid arthritis ( JR) is the most common specific childhood entity associated with uveitis in children.

a.Risk factors for uveitis in children who have JR include female sex, pauciarticular onset of arthritis, circulating antinuclear antibodies, and HLA-

DW5 and HLA-DPw2 antigens.

b.Approximately 12% of patients with JR in whom uveitis develops eventually become blind.

C.Crohn’s disease

1.Crohn’s disease is an idiopathic, chronic, inflammatory bowel disease that shows frequent extra-bowel inflammation in the eyes, eyelids, orbits, lungs, joints, and skin.

A Mollicute-like organism (i.e., a noncultivatable, cell wall-deficient bacterial pathogen) may cause

Crohn’s disease and the uveitis.

Bacteria can be classified as Firmicutes (Gram-positive), Gracilicutes (Gram-negative), or Mollicutes (lack a cell wall, enclosed only by a plasma membrane, and stain poorly with biologic stains). Obligate intracellular Mollicutes, previously called mycoplasma-like organisms, are prokaryotic (unicellular), have no cell wall or distinct nucleus, and are the smallest prokaryote capable of self-replication.

2.Ocular findings include, most commonly, acute episcleritis, scleritis, acute anterior uveitis, and marginal keratitis; less commonly, conjunctivitis, orbital inflammation, optic neuritis, ischemic optic neuropathy, and retinal vasculitis.

Similar ocular findings can occur in ulcerative colitis.

3.Histologically, a granulomatous process is most common, but a chronic nongranulomatous inflammation may also be found.

SEQUELAE OF UVEITIS,

ENDOPHTHALMITIS, AND

PANOPHTHALMITIS

Cornea

I.Corneal endothelial degeneration or glaucoma, or both, may result in chronic stromal and epithelial edema and ultimately in bullous keratopathy (Fig. 3.10).

A.Pannus degenerativus may follow bullous keratopathy.

B.Keratic precipitates of mononuclear cells (mainly lymphocytes and plasma cells) along with pigment (see Figs 3.5B and 3.9B) may be found on the endothelium.

II.Ruptured corneal bullae may become infected secondarily, leading to a corneal ulcer.

Corneal ulceration may lead to perforation. Perforation and the resultant abrupt decrease in intraocular pressure may cause a ciliary artery to rupture, producing an expulsive intraocular hemorrhage.

III.Band keratopathy (i.e., calcium deposition; see Figs 8.23 to 8.25) is common beneath the corneal epithelium in

chronically inflamed eyes, especially in children who have

Still’s disease.

IV. Corneal vascularization (see Fig. 8.18)

Anterior Chamber

I. Products of inflammation or hemorrhage may become organized, resulting in cicatrization.

II.The cicatrization or iris neovascularization may obliterate the angle of the anterior chamber.

Fig. 3.10 Corneal edema. The cornea is edematous and shows large bullous formation. The corneal edema and lymphocytes, plasma cells, and pigment on the posterior corneal surface, forming fine keratic precipitates, are secondary to chronic nongranulomatous uveitis.

Iris

I.The iris may undergo atrophy and necrosis with loss of dilator muscle, stroma, and even sphincter muscle and pigment epithelium.

II.Chronic anterior uveitis may induce peripheral anterior synechiae formation (see Fig. 3.5C).

III.Neovascularization of the anterior surface of the iris (rubeosis iridis or red iris, as seen clinically) may cause secondary anterior-chamber angle synechiae and secondary angle closure.

Shrinkage of the fibrovascular membrane on the ante-

rior iris surface may evert the pupillary border of the iris, termed an ectropion uveae (Fig. 3.11; see Fig. 15.5).

IV. Inflammatory and fibrous iris membranes may attach the pupillary margin of the iris to an underlying lens, to a lens implant or lens capsule in pseudophakic eyes, or to the anterior surface of the vitreous in aphakic eyes, resulting in an immobile pupil, seclusio pupillae (Fig. 3.12).

T e sameh membrane may grow over the pupil and cover or occlude the area completely, called occlusio pupillae (see Fig. 3.12).

The same membrane that binds the pupil down to surrounding structures usually grows across the pupil, so that seclusio pupillae and occlusio pupillae are often found together. Also, shrinkage of the membrane between iris and lens may cause the pupillary border to become inverted, termed entropion uveae.

V.Total (i.e., 360°) posterior synechiae cause a complete pupillary block, preventing aqueous flow into the anterior chamber.

A.The pressure builds up in the posterior chamber, bowing the iris forward (iris bombé; see Fig. 3.12).

B.An iris bombé forces the anterior peripheral iris to touch the peripheral posterior cornea, resulting in peripheral anterior synechiae and, if aqueous secretion is adequate, secondary closed-angle glaucoma.

Such eyes often have reduced aqueous flow and hypotony may result even in the face of a completely closed angle.

Lens

I.Intraocular inflammation frequently induces the lens epithelium to migrate posteriorly. The presence of the aberrant cells under the posterior lens capsule produces a posterior subcapsular cataract.

Although posterior subcapsular cataract can be induced by intraocular inflammation anywhere in the eye, it most likely is secondary to posterior inflammation (choroiditis).

II.An anterior subcapsular cataract frequently results from an anterior uveitis (e.g., iritis or iridocyclitis), especially when posterior synechiae are present.

Ciliary Body

I.With chronic intraocular inflammation, the ciliary processes or crests tend to become flattened and attenuated

and their cores fibrosed (hyalinized).

II.The ciliary epithelium (nonpigmented, pigmented, or both) may proliferate, sometimes to a marked degree (i.e.,

massive proliferation of ciliary epithelium).

III.Intraocular inflammation may organize and fibrose behind the lens or lens implant (or behind the pupil in an aphakic eye) between portions of the pars plicata of the ciliary body.

Such a fibrous membrane spanning the retrolental space and often incorporating proliferated ciliary epithelium and vitreous base is called a cyclitic membrane (see Fig. 3.12C;

Fig. 3.13).

When a cyclitic membrane shrinks, the vitreous base, ciliary body pars plana, and peripheral neural retina are drawn inward to cause

A B

ps

pas

pas

l

cm

h

dr

Fig. 3.12 Seclusion and occlusion of pupil. A, A membrane has grown across the pupil (occlusion of the pupil) and has adhered to the underlying lens, preventing the pupil from moving (seclusion of the pupil). B, Aqueous in the posterior chamber has bowed the iris forward (iris bombé), resulting in peripheral anterior synechiae. C, Histologic section of another case shows iris bombé, posterior synechiae (ps) of the iris to the anterior surface of the lens, a cyclitic membrane (cm), and a neural retinal detachment (dr). All are the result of long-standing chronic uveitis (pas, peripheral, anterior synechia; l, lens; h, hemorrhage under retina). (A and B, Courtesy of Dr. GOH Naumann.)

a total ciliary body and neural retinal detachment (see Fig. 3.13). Ciliary body degeneration diminishes aqueous production and leads to hypotony.

Vitreous Compartment

I.Newly formed blood vessels from the neural retina or optic disc, or both, may grow into the vitreous compartment of the eye.

A.They usually grow between the vitreous and internal surface of the neural retina, along the posterior surface of a detached vitreous, or into Cloquet’s canal.

B.The newly formed blood vessels almost never grow into the formed vitreous.

II.The vitreous body may collapse (i.e., become detached posteriorly).

III.Inflammatory products in the vitreous body may induce organization of the vitreous. Fibrous membranes, including a cyclitic membrane and anterior vitreal organization, usually result.

Fig. 3.13 Shrinkage of a cyclitic membrane, a fibrous membrane that spans the retrolental space and incorporates proliferated ciliary epithelium and vitreous base, has caused a neural retinal detachment. Massive ciliary body edema (“detachment”), posterior synechiae of iris to lens, and iris bombé are also present.

Choroid

I. As an aftermath of choroiditis, the choroid may show focal or di use areas of atrophy or scarring.

II.Retinochoroiditis or chorioretinitis may destroy Bruch’s membrane and retinal pigment epithelium, the choroid and retina may become fused by fibrosis, and a chorioretinal scar or adhesion results.

Chorioretinal adhesions may result without choroidal involvement. This occurs when proliferated retinal pigment epithelium adheres overlying neural retina to the underlying choroid.

Retina

I.Inflammation anywhere in the eye, even in the cornea, frequently causes a neural retinal perivasculitis with lymphocytes surrounding the blood vessels. If extensive, the perivasculitis can be noted clinically as vascular sheathing.

Permanent vascular sheathing results from organization and cicatrization of a perivascular inflammatory infiltrate or from involution of the blood vessels and thickening of their walls.

II.Intraocular inflammation, especially involving the peripheral neural retina or ciliary body, may be accompanied by

fluid in the macular retina (i.e., cystoid macular edema).

III. Retinochoroiditis or chorioretinitis may result in chorioretinal scarring.

IV. The neural retina may become detached secondary to subneural retinal exudation or hemorrhage or to organization and formation of vitreal fibrous membranes or a cyclitic membrane.

V.The retinal pigment epithelium is a very reactive tissue and may undergo massive hyperplasia after inflammation. It

may also show alternating areas of mild hyperplasia and atrophy, or it may be associated with intraocular ossification.

Glaucoma

I.Glaucoma may result from inflammatory cells and debris clogging an open anterior-chamber angle; from peripheral

anterior synechiae and secondary angle closure; from posterior synechiae, pupillary block, iris bombé, and secondary angle closure; or from trabecular damage (inflammation, i.e., trabeculitis and scarring).

II.The proper combination of factors must be present for glaucoma to develop; for example, peripheral anterior synechiae may be present, but if the inflammation damages the capacity for the ciliary body to secrete aqueous, glaucoma does not develop; in fact, hypotony may result.

When hypotony or a normal intraocular pressure is present in an eye with iris bombé and complete closure of the anterior-

chamber angle, aqueous is not being secreted. Intraocular surgery in such an eye often hastens the development of phthisis bulbi.

END STAGE OF DIFFUSE

OCULAR DISEASES

I.Atrophy without shrinkage

A.This refers to atrophy of intraocular structures such as the retina and uvea in a normal-size or even enlarged eye (e.g., buphthalmos).

B.The best example is the di use atrophy with long-

standing glaucoma.

II.Atrophy with shrinkage (atrophia bulbi; Fig. 3.14)

A.This refers to atrophy of intraocular structures, which remain recognizable, plus atrophy of the globe so that it is smaller than normal.

B.The best example is chronic, long-standing uveitis

(especially when it starts in childhood) that goes on to hypotony in the presence of an anterior-chamber angle closed by peripheral anterior synechiae.

Clinically, the eye is soft and partially collapsed. The pull of the horizontal and vertical rectus muscles causes the shrunken eye to appear cuboid (“squared-off”) instead of spherical when viewed with the lids widely separated. A soft, squaredoff atrophic eye when seen clinically is called a phthisical eye or a phthisis bulbi. Histologically, however, the eye does not usually show phthisis bulbi (see later), but rather atrophia bulbi.

III.Atrophy with shrinkage and disorganization (phthisis bulbi; see Fig. 3.14)

A.This refers to a markedly thickened sclera and atrophy of intraocular structures su ciently profound to make them unrecognizable.

B.The best example is an unchecked purulent endophthal-

mitis that results in destruction of all the intraocular structures and widespread intraocular scarring and shrinkage.

IV. Intraocular ossification

A.This is common in atrophia bulbi (see Fig. 3.14, and Fig. 18.11) and phthisis bulbi.

B.The bone, which forms without cartilage, seems to require pigment epithelium for its formation, either as an inducer or from actual metaplasia.

A fatty marrow is often present in the bone. In younger patients (<20 years of age), the marrow usually possesses hematopoietic elements.

V.Calcium, often as calcium oxalate, may be deposited in a band keratopathy, a cataractous lens, intraocular bone, sclera, a gliotic neural retina, or optic nerve.

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INTRODUCTION

Chronic granulomatous inflammation is a proliferative inflammation characterized by a cellular infiltrate of epithelioid cells

[and sometimes inflammatory giant cells, lymphocytes, plasma cells, polymorphonuclear leukocytes (PMNs), and eosinophils; see p. 12 in Chapter 1].

POSTTRAUMATIC

Sympathetic Uveitis (Sympathetic Ophthalmia,

Sympathetic Ophthalmitis)

I.Sympathetic uveitis (Figs 4.1 and 4.2) is a bilateral, di use, granulomatous, T-cell-mediated uveitis that occurs from 2 weeks to many years after penetrating or perforating ocular injury and is associated with traumatic uveal incarceration or prolapse.

A.Although the uveitis may start as early as 5 days or as late as 50 years after injury, well over 90% of cases occur after 2 weeks but within 1 year. Most of these (80%) occur within 3 weeks to 3 months postinjury.

B.Removal of the injured eye before sympathetic uveitis occurs usually completely protects against inflammation developing in the noninjured eye.* Once the inflammation starts, however, removal of the injured (“exciting”) eye probably has little e ect on the course of the disease, especially after 3 to 6 months.

*Rarely, sympathetic uveitis has been reported to have developed in the sympathizing eye after the injured eye has been enucleated.

Evidence exists that early enucleation of the exciting eye can favorably affect visual prognosis, especially early enucleation, within the first 3 to 6 months. Sympathetic uveitis has been reported in nontraumatized eyes in a few isolated cases. However, unless the whole eye is serially sectioned and carefully examined for evidence of perforation, the clinician can never be sure that some long-forgotten penetrating ocular wound is not present. A diagnosis of sympathetic uveitis in the absence of an ocular injury should be viewed with marked skepticism.

II.Blurred vision and photophobia in the noninjured (sympathizing) eye are usually the first symptoms. Vision and photophobia worsen concurrently in the injured (exciting) eye, and a granulomatous uveitis develops, especially mutton-fat keratic precipitates (see Fig. 4.1A), which are collections of epithelioid cells plus lymphocytes, macrophages, inflammatory multinucleated giant cells, or pigment on the posterior surface of the cornea.

Glaucoma may develop owing to blockage of the angle by cellular debris or peripheral anterior synechiae, or hypotony may occur from decreased aqueous output by the inflamed ciliary body.

III.The cause appears to be a delayed-type hypersensitivity reaction of the uvea to antigens localized on the retinal pigment epithelium or on uveal melanocytes.

A.The lymphocytic infiltrate consists almost exclusively of T lymphocytes.

B.B cells found in some cases, usually of long duration, may represent the end stage of the disease.

Phacoanaphylactic endophthalmitis (PE) was found in approximately 25% of patients who had sympathetic uveitis in cases submitted to the Armed Forces Institute of Pathology before