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

Margo CE, Barletta JP, Staman JA: Giant cell astrocytoma of the retina in tuberous sclerosis. Retina 13:155, 1993

Milot J, Michaud J, Lemieux N et al.: Persistent hyperplastic primary vitreous with retinal tumor in tuberous sclerosis: Report of a case including tumoral immunuhistochemistry and cytogenetic analyses. Ophthalmology 106:614, 1999

Shields JA, Eagle RC Jr, Shields CL et al.: Aggressive retinal astrocytomas in 4 patients with tuberous sclerosis complex. Arch Ophthalmol 123:856, 2005

von Slegtenhorst M, de Hoogt R, Hermans C et al.: Identification of the tuberous sclerosis gene TSC1 on chromosome 9q34. Science 277:805, 1997

Zimmer-Galler IE, Robertson DM: Long-term observation of retinal lesions in tuberous sclerosis. Am J Ophthalmol 119:318, 1995

Other Phakomatoses

Buckley RH: Immunodeficiency diseases. JAMA 268:2797, 1992

Carbonari M, Cherchi M, Paganelli R et al.: Relative increase in T cells expressing the gamma/delta rather than the alpha/beta receptor in ataxia-telangiectasia. N Engl J Med 322:73, 1990

Danis R, Appen RE: Optic atrophy and the Wyburn–Mason syndrome.

J Clin Neuroophthalmol 4:91, 1984

E ron L, Zakov ZN,Tomask RL: Neovascular glaucoma as a complication of the Wyburn–Mason syndrome. J Clin Neuroophthalmol 5:95, 1985

Farr AK, Shalev B, Crawford TO et al.: Ocular manifestations of ataxiatelangiectasia. Am J Ophthalmol 134:891, 2002

Font RL, Ferry AP: The phakomatoses. Int Ophthalmol Clin 12:1, 1972 Hopen G, Smith JL, Ho JT et al.: T he Wyburn–Mason syndrome: concomitant chiasmal and fundus vascular malformations. J Clin Neu-

roophthalmol 3:53, 1983

Mansour AM, Wells CG, Jampol LM et al.: Ocular complications of arteriovenous communication of the retina. Arch Ophthalmol 107:232, 1989

Shin GS, Demer JL: Retinal arteriovenous communications associated with features of the Sturge–Weber syndrome. Am J Ophthalmol 117:115, 1994

Wyburn-Mason R: Arteriovenous aneurysm of the midbrain and retina with facial naevus and mental changes. Brain 66:12, 1943

Chromosomal Trisomy Defects

Fowell SAM, Greenwald MJ, Prendiville JS et al.: Ocular findings of incontinentia pigmenti in a male infant with Klinefelter syndrome. J Pediatr Ophthalmol Strabismus 29:180, 1992

Frangoulis M, Taylor D: Corneal opacities: A diagnostic feature of the trisomy 8 mosaic syndrome. Br J Ophthalmol 67:619, 1983

Guterman C, Abboud E, Mets MB: Microphthalmos with cyst and Edward’s syndrome. Am J Ophthalmol 109:228, 1990

Hoepner J, Yano M: Craniosynostosis and syndactylism (Apert’s syndrome) associated with a trisomy 21 mosaic. J Pediatr Ophthalmol 8:107, 1971

Hoepner J, Yano M: Ocular anomalies in trisomy 13–15: An analysis of 13 eyes with two new findings. Am J Ophthalmol 74:729, 1972 Jacoby B, Reed JW, Cashwell LF: Malignant glaucoma in a patient with

Down’s syndrome and corneal hydrops. Am J Ophthalmol 110:434, 1990

Lueder GT: Clinical ocular abnormalities in infants with trisomy 13. Am J Ophthalmol 141:1057, 2006

McDermid HE, Duncan AMV, Brasch KR et al.: Characterization of the supernumerary chromosome in cat eye syndrome. Science 232:646, 1986

Robb RM, Marchevsky A: Pathology of the lens in Down’s syndrome.

Arch Ophthalmol 96:1039, 1978

Seiberth V, Kachel W, Knorz MC et al.: Ophthalmic findings in partial monosomy 4p (Wolf syndrome) in combination with partial trisomy 10p. Am J Ophthalmol 117:411, 1994

Traboulsi EI, Levine E, Mets MB et al.: Infantile glaucoma in Down’s syndrome (trisomy 21). Am J Ophthalmol 105:389, 1988

Yano M, Font RL, Zimmerman LE: Intraocular cartilage in a microphthalmic eye of an otherwise healthy girl. Arch Ophthalmol 81:238, 1969

Yano M, Frayer WC, Scheie HG: Ocular findings in a patient with 13–15 trisomy. Arch Ophthalmol 70:372, 1963

Triploidy and Chromosomal

Deletion Abnormalities

Bonetta L, Kuehn SE, Huang A et al.: Wilms tumor locus on 11p13 defined by multiple CpG island-associated transcripts. Science 250:994, 1990

Cameron JD, Yano M, Frayer WC: Turner’s syndrome and Coats’ disease. Am J Ophthalmol 78:852, 1974

Chen RM, Lupski JR, Greenberg F et al.: Ophthalmic manifestations of Emith–Magenis syndrome. Ophthalmology 103:1084, 1996

Chrousos GA, Ross JL, Chrousos G et al.: Ocular findings in Turner syndrome: A prospective study. Ophthalmology 91:926, 1984

Dowdy SF, Fasching CL, Araujo D et al.: Suppression of tumorigenicity in Wilms tumor by the p15.5–p14 region of chromosome 11. Science 254:293, 1991

Finger PT, Warren FA, Gelman YP et al.: Adult Wilms’ tumor metastatic to the choroids of the eye. Ophthalmology 109:2134, 2002

Fulton AB, Howard RO, Albert DM et al.: Ocular findings in triploidy.

Am J Ophthalmol 84:859, 1977

Chromosomal Deletion Defects

Gupta SK, deBecker I, Guernsey DL et al.: Polymerase chain-reaction based risk assessment for Wilms tumor in sporadic aniridia. Am J Ophthalmol 125:687, 1998

Gupta SK, deBecker I, Tremblay F et al.: Genotype/phenotype correlations in aniridia. Arch Ophthalmol 126:203, 1998

Huang A, Campbell CE, Bonetta L et al.: Tissue, developmental, and tumor-specific expression of divergent transcripts in Wilms tumor. Science 250:991, 1990

Küchle M, Kraus J, Rummelt C et al.: Synophthalmia and holoprosencephaly in chromosome 18p deletion defect. Arch Ophthalmol 109:136, 1991

Meyer DR, Selkin RP: Ophthalmic manifestations of the chromosome 11q deletion syndrome. Am J Ophthalmol 115:673, 1993

Rauscher FJ III, Morris JF,Tournay OE et al.: Wilms’ tumor locus zinc finger protein to the ERG-1 consensus sequence. Science 250:1259, 1990

Schechter RJ: Ocular findings in a newborn with cri du chat syndrome.

Ann Ophthalmol 10:339, 1978

Schwartz DE: Noonan’s syndrome associated with ocular abnormalities.

Am J Ophthalmol 73:955, 1972

Seiberth V, Kachel W, Knorz MC et al.: Ophthalmic findings in partial monosomy 4p (Wolf syndrome) in combination with partial trisomy 10p. Am J Ophthalmol 117:411, 1994

Wright LL, Schwartz MF, Schwartz S et al.: An unusual ocular finding associated with chromosome 1q deletion syndrome. Pediatrics 77:786, 1986

Yano M, Rorke LB, Niederer BS: Ocular and cerebral abnormalities in chromosome 18 deletion defect. Am J Ophthalmol 70:391, 1970

Mosaicism

Hoepner J, Yano M: Craniosynostosis and syndactylism (Apert’s syndrome) associated with a trisomy 21 mosaic. J Pediatr Ophthalmol 8:107, 1971

Yano M, Rorke LB: Ocular and central nervous system findings in tetraploid-diploid mosaicism. Am J Ophthalmol 75:1036, 1973

Infectious Embryopathy

Arnold JJ, McIntosh EDG, Martin FJ et al.: A fifty-year follow-up of ocular defects in congenital rubella: Late ocular manifestations. Aust N Z J Ophthalmol 22:1, 1994

Cotlier E: Congenital varicella cataract. Am J Ophthalmol 86:627, 1978 Gregg NM: Congenital cataract following German measles in the

mother. Trans Ophthalmol Soc Aust 3:35, 1941

Quentin CD, Reiber H: Fuchs heterochromi cyclitis: rubella virus antibodies and genome in aqueous humor. Am J Ophthalmol 138:45, 2004

Yano M, Scha er DB, Scheie HG: Rubella ocular syndrome: Clinical significance of viral and pathologic studies. Trans Am Acad Ophthalmol Otolaryngol 72:896, 1968

Drug Embryopathy

Bogdano B, Rorke LB, Yano M et al.: Brain and eye abnormalities: Possible sequelae to prenatal use of multiple drugs including LSD. Am J Dis Child 123:145, 1972

Chan CC, Fishman M, Egbert PR: Multiple ocular anomalies associated with maternal LSD ingestion. Arch Ophthalmol 96:282, 1978 Edward DP, Li J, Sawaguchi S et al.: Di use corneal clouding in siblings

with fetal alcohol syndrome. Am J Ophthalmol 115:484, 1993

Miller MT, Strömland K: Ocular motility in thalidomide embryopathy.

J Pediatr Ophthalmol Strabismus 28:47, 1991

Streissguth AP, Aase JM, Clarren SK et al.: Fetal alcohol syndrome in adolescents and adults. JAMA 265:1961, 1991

Other Congenital Anomalies

Boynton JR, Pheasant TR, Johnson BL et al.: Ocular findings in Kenny’s syndrome. Arch Ophthalmol 97:896, 1979

Brunquell PJ, Papale JH, Horton JC et al.: Sex-linked hereditary bilateral anophthalmos: Pathologic and radiologic correlation. Arch Ophthalmol 102:108, 1984

Chestler RJ, France TD: Ocular findings in CHARGE syndrome. Ophthalmology 95:1613, 1988

Cohen SM, Brown FR III, Martyn L et al.: Ocular histopathologic and biochemical studies of the cerebrohepatorenal syndrome (Zellweger’s syndrome) and its relationship to neonatal adrenoleukodystrophy. Am J Ophthalmol 96:488, 1983

Dobyns WB, Pagon RA, Armstrong D et al.: Diagnostic criteria for Walker–Warburg syndrome. Am J Med Genet 32:195, 1989

Dobyns WB, Reiner O, Carrozo R et al.: Lissencephaly: A human brain malformation associated with deletion of the LIS1 gene located at chromosome 17p13. JAMA 270:2838, 1993

Dooling EC, Richardson EP: Ophthalmoplegia and Ondine’s curse.

Arch Ophthalmol 95:1790, 1977

Ferreira RC, Heckenlively JR, Menkes JH et al.: Menkes disease: New ocular and electroretinographic findings. Ophthalmology 105:1076, 1998

Font RL, Marines HM, Cartwright J Jr et al.: Aicardi syndrome: A clinicopathologic case report including electron microscopic observations. Ophthalmology 98:1727, 1991

Fries PD, Katowitz JA: Congenital craniofacial anomalies of ophthalmic importance. Surv Ophthalmol 35:87, 1990

Frydman M, Kauschansky A, Leshem I et al.: Oculo-palato-cerebral dwarfism: A new syndrome. Clin Genet 27:414, 1985

Gasch AT, Caruso RC, Kaler SG et al.: Menkes’ syndrome. Ophthalmic findings. Ophthalmology 109:1477, 2002

Hayashi N, Geraghty MT, Green WR: Ocular histologic study of a patient with the T 8993-G point mutation in Leigh’s syndrome. Ophthalmology 40:197, 2000

Heggie P, Grossniklaus HE, Roessmann U et al.: Cerebro-ocular dys- plasia-muscular dystrophy syndrome: Report of two cases. Arch Ophthalmol 105:520, 1987

Howard MA, Thompson JT, Howard RO: Aplasia of the optic nerve.

Trans Am Ophthalmol Soc 91:267, 1993

Kass MA, Howard RO, Silverman JP: Russell–Silver dwarfism. Ann Ophthalmol 8:1337, 1976

Khairallah M, Messaoud R, Zaouali S et al.: Posterior segment changes associated with posterior microphthalmos. Ophthalmology 109:569, 2002

Kremer I, Lerman-Sagie T, Mukamel M et al.: Light and electron microscopic findings in Leigh’s disease. Ophthalmologica 199:106, 1989

Kretzer FL, Hittner HM, Mehta RS: Ocular manifestations of the Smith–Lemli–Opitz syndrome. Arch Ophthalmol 99:2000, 1981

Krohel GB, Wirth CR: Engelmann’s disease. Am J Ophthalmol 84:520, 1977

Lewis RA, Crowder WE, Eierman LA et al.: The Gardner syndrome: Significance of ocular features. Ophthalmology 91:916, 1984

Lichtig C, Ludatscher RM, Mandel H et al.: Muscle involvement in Walker–Warburg syndrome: Clinicopathologic features of four cases. J Clin Pathol 100:493, 1993

MacRae DW, Howard RO, Albert DM et al.: Ocular manifestations of the Meckel syndrome. Arch Ophthalmol 88:106, 1972

Marcus DM, Shore JW, Albert DM: Anophthalmia in the focal dermal hypoplasia syndrome. Arch Ophthalmol 108:96, 1990

Millay RH, Weleber RG, Heckenlively JR: Ophthalmologic and systemic manifestations of Alström’s disease. Am J Ophthalmol 102:482, 1986

Murata T, Ishibashi T, Ohnishi Y et al.: Cornea choristoma with microphthalmos. Arch Ophthalmol 109:1130, 1991

O’Rahilly R, Müller F: Interpretation of some median anomalies as illustrated by cyclopia and symmelia. Teratology 40:409, 1989

Reynolds JD, Johnson BL, Gloster S et al.: Glaucoma and Klippel– Traunay–Weber syndrome. Am J Ophthalmol 106:494, 1988

Rosenthal AR, Ryan SJ, Horowitz P: Ocular manifestations of dwarfism. Trans Am Acad Ophthalmol Otolaryngol 76:1500, 1972

Rotberg M, Klintworth GK, Crawford JB: Ocular vasodilation and angiogenesis in Potter’s syndrome. Am J Ophthalmol 97:16, 1984

Sassani JW, Yano M: Anophthalmos in an infant with multiple congenital anomalies. Am J Ophthalmol 83:43, 1977

Sedwick LA, Burde RM, Hodges FJ III: Leigh’s subacute necrotizing encephalomyelopathy manifesting as spasmus nutans. Arch Ophthalmol 102:1046, 1984

Stefani FH, Hausmann N, Lund O-E: Unilateral diplophthalmos. Am J Ophthalmol 112:581, 1991

Stewart DH, Streeten BW, Brockhurst RJ et al.: Abnormal scleral collagen in nanophthalmos: An ultrastructural study. Arch Ophthalmol 109:1017, 1991

Torczynski E, Jakobiec FA, Johnston MC et al.: Synophthalmia and cyclopia: A histopathologic, radiographic, and organogenetic analysis.

Doc Ophthalmol 44:311, 1977

Toussaint D, Danis P: Dystrophie maculaire dans une maladie de Menkes: Etude histogogique oculaire. J Fr Ophthalmol 1:457, 1978

I.Suppurative nongranulomatous inflammation.

A.This is an acute, nongranulomatous (no epithelioid or giant cells), purulent inflammatory reaction in which the predominant cell type is the polymorphonuclear leukocyte.

B.The reaction usually has an acute onset and is characterized by suppuration (i.e., the formation of pus).

This type of inflammation is usually secondary to infection with bacteria that cause a purulent (pus) inflammatory reaction, such as Staphylococcus aureus.

II.Nonsuppurative nongranulomatous inflammation.

A.This may be an acute (cellulitis secondary to Streptococcus hemolyticus) or chronic (the common type of uveitis) inflammation.

Streptococcal gangrene of the eyelids, caused by group A hemolytic Streptococcus (also called flesh-eating disease, necrotizing fasciitis, necrotizing erysipelas, and gangrenous erysipelas), although rare, appears to be increasing in prevalence.

B.The predominant cell type in acute inflammation is the polymorphonuclear leukocyte, and in chronic inflammation it is the lymphocyte and the plasma cell.

Nongranulomatous inflammation may have an acute, subacute, or chronic course.

Terminology

I.If a single tissue is involved, the inflammation is classified according to involved tissue (e.g., cornea—keratitis; retina—retinitis; vitreous—vitritis; optic nerve—optic neuritis; sclera—scleritis; and uvea—uveitis).

If more than one tissue is involved but not an adjacent cavity (a most unusual occurrence), then the inflammation is classified by the tissues involved with the site of primary involvement first (e.g., retinochoroiditis in toxoplasmosis and chorioretinitis in tuberculosis).

II.Endophthalmitis (Fig. 3.1) is an inflammation of one or more coats of the eye and adjacent cavities.

By this definition, a corneal ulcer with a hypopyon or an iritis with aqueous cells and flare would be an endophthalmitis, but most clinicians require a vitritis before calling an ocular inflammation an endophthalmitis.

III.Panophthalmitis (Fig. 3.2) is an inflammation of all three coats of the eye (and adjacent cavities); it often starts as an endophthalmitis that then involves the sclera and spreads to orbital structures.

Sources of Inflammation

I.Exogenous: sources originate outside the eye and body [e.g., local ocular physical injury (surgical trauma, penetrating and perforating nonsurgical trauma, radiant

energy); chemical injuries (acid and alkali); and allergic reactions to external antigens (conjunctivitis secondary to pollen)].

II.Endogenous: sources originate in the eye [e.g., inflammation secondary to cellular immunity (phacoanaphylactic endophthalmitis); spread from contiguous structures (the sinuses); hematogenous spread (virus, bacteria, fungus, foreign particle); and conditions of unknown cause

(sarcoidosis)].

plasma cells) than is necrosis of a retinoblastoma. Clinically, however, inflammation is seen more frequently in retinoblastoma than in melanoma. In fact, retinoblastoma may clinically simulate inflammation in approximately 8% of retinoblastoma eyes.

C.Inflammation of contiguous or nearby structures (e.g., orbital abscess or cellulitis, meningitis, or a nasal phycomycosis) may rarely spread into the eye.

SUPPURATIVE ENDOPHTHALMITIS AND PANOPHTHALMITIS

Clinical Features

I.Severe ocular congestion, chemosis, and haziness of the cornea, aqueous, and vitreous are characteristic. A purulent

exudate, frequently visible as a hypopyon, may be present in the anterior chamber.

II.Pain is prominent in both conditions, but especially in panophthalmitis.

III. Extension into orbital tissue often results in congestion

and edema of the lids and even exophthalmos.

IV. The cause may be infectious or noninfectious.

Classification

I.Exogenous

A.Infectious keratitis and corneal ulcers may cause a reflex sterile suppurative iridocyclitis and hypopyon.

B.Nonsurgical penetrating or perforating trauma (or, rarely, surgical) may lead to the presence of a contaminated or sterile intraocular foreign body, producing a suppurative inflammation.

C.Postoperative suppurative inflammation in the first day or two after surgery is usually purulent, fulminating (i.e., rapid), and caused by bacteria.

1.Delayed (e.g., a month or two after intraocular surgery) endophthalmitis suggests a fungal infection.

2.A bacterial infection is also a possible cause of delayed endophthalmitis, especially with less virulent bacteria such as Staphylococcus epidermidis and

Propionibacterium acnes (see p. 121 in Chapter 5).

II.Endogenous

A.Metastatic septic emboli, especially in children or debilitated persons, may occur in subacute bacterial endocarditis,meningococcemia,or other infections associated with a bacteremia, viremia, or fungemia.

B.Necrosis of an intraocular neoplasm, particularly retinoblastoma, may rarely result in a suppurative endophthalmitis or even panophthalmitis.

Histologically, necrosis of a malignant melanoma is more likely to induce an inflammatory reaction (usually lymphocytes and

Histology

Suppurative inflammation is characterized by polymorphonuclear leukocytic infiltration into the involved tissues (Figs 3.3 and 3.4). Marked tissue necrosis causes a suppurative or purulent exudate (pus).

Examples

I.Behçet’s disease (see Fig. 3.3) is an example of a chronic endogenous endophthalmitis.

A.It is a triple-symptom complex consisting of ocular inflammation (occurs in 70% to 80% of patients), oral ulceration (aphthous stomatitis), and genital ulceration

(conjunctival ulcers may also occur).

Eating English walnuts can exacerbate Behçet’s disease.

B.The disease is most common in men between the ages of 20 and 30 years, has a male predominance, and men have a more severe involvement with a greater risk of vision loss than women.

C.Arthritis or arthralgia, cutaneous lesions, thrombophlebitis, ulcerative colitis, encephalopathy, pancreatitis, central and peripheral neuropathy, vena caval obstruction, subungual infarctions, and malignant lymphomas may also be seen.

D.Plasminogen activator levels may be decreased.

E.A hypercoagulable or general vascular endothelial dysfunction is usually found.

F.S-antigen-responsive lymphocytes are increased in the peripheral blood during episodes of ocular inflammation.

G.To make the diagnosis of Behçet’s disease, patients must have at least three episodes of aphthous or herpetiform ulcerations in 12 months and two of the following four findings: recurrent genital ulceration; ocular signs (e.g., anterior or posterior uveitis, vitritis, or retinal vasculitis); skin lesions (e.g., erythema nodosum, pseudofolliculitis, or papulopustules); and positive pathergy test (sterile pustule developing within 24 to 48 hours at site of a cuticular needle puncture).

Plasma exchange, by removing immune complexes from the circulation, may be an alternative treatment for severe cases of Behçet’s disease.

C

H.The ocular inflammation is characterized by recurrent iridocyclitis and hypopyon (often motile), usually involving both eyes but not necessarily simultaneously.

1.In addition, macular edema, retinal pigmentary changes and periphlebitis, vitritis, periarteritis, and retinal and vitreal hemorrhages may occur (even when visual complaints are not present, fluorescein angiography shows leakage from superficial optic nerve capillaries and venules and peripheral retinal capillaries).

2.The presence of small patches of retinal whitening is characteristic.

3.Secondary posterior synechiae may lead to iris bombé, peripheral anterior synechiae, and secondary angle closure glaucoma.

4.Rarely, a bilateral immune corneal ring (Wessely ring) may occur.

I.Biopsy of mucocutaneous lesions shows vasculitis.

J.The serum may show variable increases in polyclonal immunoglobulins and anticytoplasmic antibodies. Serum and aqueous humor sialic acid levels are elevated during the active and remission phases of Behçet’s disease.

K.Human leukocyte antigen (HLA)-B51, which belongs to the HLA-B5, B35 cross-reacting group, is the most

strongly associated genetic marker on Behçet’s disease over many ethnic groups.

1.The gene HLA-B appears to be responsible for, and determines the susceptibility to, Behçet’s disease.

2.Factor V Leiden mutations are a risk factor for the development of Behçet’s disease.

L.Histologically, the main process appears to be a small or moderate-sized blood vessel vasculitis.

1.Retinal perivasculitis,vasculitis,hemorrhagic infarction, and detachment, along with a chronic nongranulomatous uveitis, may be seen.

2.An acute, suppurative inflammatory infiltrate with neutrophils occurs in the anterior chamber (hypopyon).

3.A secondary chronic nongranulomatous inflammatory infiltrate is frequently noted in adjacent tissues (see Fig. 3.4).

The choroidal infiltrate is predominantly CD4+ T lymphocyte, and some B lymphocytes and plasma cells.

4.Spontaneous rupture of the lens can cause phacoanoaphylactic endopthalmitis.

NONSUPPURATIVE, CHRONIC NONGRANULOMATOUS UVEITIS AND ENDOPHTHALMITIS

Clinical Features

I.Anterior involvement often causes a severe, acute, “plastic” or exudative recurrent iridocyclitis, whereas posterior involvement produces a choroiditis or chorioretinitis.

II.A chronic nongranulomatous uveitis of unknown cause characterizes the “garden-variety” type of uveitis.

Classification

I.Exogenous: the inflammation is usually secondary to trauma.

A.The most common type is the iridocyclitis (traumatic iridocyclitis) that follows many injuries to the eye, particularly blunt trauma or intraocular surgery.

B.Penetrating or perforating injuries may produce a sterile, chronic nongranulomatous inflammation, resulting from multiple, tiny foreign bodies, degenerating blood, necrotic uvea, and so forth.

II.Endogenous (Fig. 3.5)

A.Idiopathic inflammation (i.e., “garden-variety” anterior uveitis) is the most common form of endogenous uveitis. The cause is unknown but may be related to cellular immunity.

Rhegmatogenous retinal detachment may occur in about 3% of cases of uveitis. A close association exists with the HLA-B27 antigen (also found in the rheumatoid group of diseases). In addition, the anterior uveitis may follow, and be related to, infection with a variety of Gram-negative bacteria (e.g., Yersinia species and Chlamydia trachomatis) or with Mollicutes (see discussion of Crohn’s disease, later).

B.The inflammation may be associated with viral infections such as rubella and subacute sclerosing panencephalitis (SSPE); bacterial infections such as syphilis;

local ocular (nonsystemic) entities such as pars planitis,

Fuchs’ heterochromic iridocyclitis (FHI), uveal e usion (see p. 355 in Chapter 9), and glaucomatocyclitic crisis (Posner–Schlossman syndrome; see p. 645 in Chapter

16); and systemic diseases such as Reiter’s syndrome, Behçet’s disease (see earlier), Kawasaki’s disease (mucocutaneous lymph node syndrome), phacoanaphylactic endophthalmitis (the uvea usually shows a chronic, nongranulomatous uveitis; see p. 75 in Chapter 4), collagen vascular disease (including rheumatoid arthritis), Crohn’s disease (regional enteritis; see p. 67 in this chapter), ulcerative colitis, and Whipple’s disease (see p. 484 in Chapter 12); and atopy.

Examples

I.Viral infections such as herpes simplex and zoster, Epstein– Barr virus (EBV), SSPE, rubella (see p. 43 in Chapter 2), and rubeola may cause an endogenous nonsuppurative, chronic nongranulomatous uveitis.

A.Herpes simplex virus (HSV; Fig. 3.6)

1.HSV consists of a linear, double-stranded DNA packaged in an icosahedral capsid and covered by a lipid-containing membrane.

a.HSV-1 is usually responsible for initial infections in children and for most herpetic eye infections in all ages.

b.HSV-2, usually responsible for genital herpes, may rarely cause ocular disease in neonates (contamination at birth by mother’s genital herpes) or adults.

2.Neonatal HSV most commonly causes a nonfollicular conjunctivitis followed by keratitis.

a.Other ocular findings include retinochoroiditis (or chorioretinal scarring), iritis, cataracts, optic atrophy or neuritis, and microphthalmia.

b.The di erential diagnosis consists of the TORCH syndrome (toxoplasmosis, rubella, cytomegalovirus, and herpes simplex).

Lymphocytic choriomeningitis virus can cause a congenital chorioretinitis and can be added to the TORCH list.

3.Acquired HSV in children and adults is similar to that in neonates.

Children who have HSV keratitis may have bilateral involvement and are at risk for recurrent keratitis and amblyopia.

a.A mucocutaneous eruption is common.

b.HSV retinitis (a cause of the acute retinal necrosis syndrome) may occur in immunocompetent or immunodeficient people (e.g., in acquired immunodeficiency syndrome).

c.The most common ocular manifestation is keratitis (see p. 267 in Chapter 8).

4.Histologically, the infected area reveals both acute and chronic nongranulomatous inflammation.

a.Intranuclear inclusions (Cowdry type A) may be seen.

b.HSV can be detected by monoclonal antibodies, such as the avidin–biotin complex immunoperoxidase technique, and by in situ DNA hybridization method using viral genome segments.

B.Epstein-Barr virus (EBV)

1.The EBV, a B-lymphotrophic virus, accounts for most cases of infectious mononucleosis, is associated with Burkitt’s lymphoma (see p.579 in Chapter 14), and is detected in up to 50% of B-cell malignancies encountered in immunosuppressed patients.

EBV, after infecting the host’s cells, can hide from the immune system by producing a protein that inhibits its own synthesis, thereby minimizing the amount that appears on the cell surface.