Ординатура / Офтальмология / Учебные материалы / Color Atlas of Ophthalmology The Quick-Reference Manual for Diagnosis and Treatment

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Fig . 6.1 (A) Arterial phase fluorescein angiogram (FA) demonstrating active toxoplasma chorioretinitis adjacent to a classic chorioretinal scar. (B) Arteriovenous phase FA demonstrating the classic old pigmented lesion with hypofluorescence at the center and hyperfluorescence at the margins of the lesion. (C) Late -phase FA showing juxtapapillary multiple hyperfluorescent areas corresponding to new, active lesions. (D) Active lesions, areas of hyperfluorescence, are usually seen in the vicinit y of old scars, as evidenced here. (Courtesy of J. Fernando Arevalo, Venezuela)

Adult toxoplasm osis: Th ere is react ivat ion of in flam m at ion at th e edges of a preexist ing scar; th is is called a satellite lesion . Vit rit is is severe an d th e m argin s of th e lesion are yellow an d blurred . Th ere is a hypersen sit ivit y react ion to th e t roph ozoites th at are released from th e cysts.

Th e follow ing term s h ave been used to describe th e lesion :

Searchlight in fog: Yellow lesion s seen th rough th e vit reous h aze

Grapevines: Of th e m em bran es an d cells of vit rit is

W et snow : St icky vit reou s exudates in vit rit is

Differential Diagnosis

In ch ildren , differen t ials in clude congen it al colobom as, cytom egaloviru s in clusion ch orioret in it is, h erp es sim plex ch orioret in it is, toxocariasis, ret in oblastom a, an d cerebral t rau m a.

In adu lt s, differen t ials in clu de t u berculous ch orioret in it is, can didiasis, an d h is- t ioplasm osis.

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Management

An t ibiot ics in th e form of Bact rim DS (dou ble st rength ), clin dam ycin , or pyrim eth - am in e w ith folin ic acid are given for 6 w eeks in conjun ct ion w ith an in tern ist . Oral steroids m ay be in dicated w h en th e disease spreads close to th e opt ic n er ve or m acu la.

Presumed Ocular Histoplasmosis

Histoplasm a capsulat um causes th e ch aracterist ic presu m ed ocu lar h istop lasm osis syn drom e (POHS). Im m un ocom prom ised pat ien t s are affected by th e presen ce of an isolated gran u lom a or en doph th alm it is.

POHS t ypically affects you ng people an d is respon sible for bilateral visu al loss. Th e ch aracterist ic feat u res of th e syn drom e are th e appearan ce of isolated disciform m acular lesion s an d scars th at are w ell circu m scribed . Th ese are quiet lesion s w ith ou t th e presen ce of act ive in flam m at ion an d th e presen ce of peripapillar y pigm en t at rophy (Fig. 6.2).

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Fig. 6.2 (A) Disseminated choroiditis (histo spots), maculopathy, and peripapillary chorioretinal degenerative changes in a patient with the presumed ocular histoplasmosis syndrome. (B) Disseminated choroiditis (histo spots), maculopathy, and peripapillary chorioretinal degenerative changes in another patient with the presumed ocular histoplasmosis syndrome. (C) Fluorescein angiography of the patient in Fig. 6.2B, revealed serosanguinous retinal detachment with faint pigment halo in right macula and choroidal neovascularization. (D) Fluorescein angiography of patient in Fig. 6.2B, revealed hypofluorescence after photodynamic therapy with verteporfin. (Courtesy of Steve Bloom, MD).

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Management

Follow up for developm en t of ch oroidal n eovascu lar m em bran e an d t reat accord - ingly.

White Dot Syndrome

Multifocal Choroiditis

Mu lt ifocal ch oroidit is is a rare disorder involving idiopath ic in flam m at ion of th e ch oroid an d ret in al pigm en t epith eliu m (RPE).

Presentation

Presen t at ion is u sually bilateral, w ith sym ptom s such as ph otoph obia, blu rred vision , eye pain , an d decreased visual acu it y. Th e pat ien t m ay also h ave m etam orph opsia, floaters, scotom as, an d ph otopsia. Th ere m ay be sign s an d sym ptom s of an terior uveit is.

Mu lt iple, sm all, yellow /gray-w h ite spot s m ay be seen along w ith cystoid m acu - lar edem a, ch oroidal n eovascular m em bran e, an d subret in al fibrosis.

Management

Cor t icosteroids are h elpfu l in resolving th e lesion s.

Multiple Evanescent White Dot Syndrome

Mu lt iple evan escen t w h ite dot syn drom e (MEW DS) is an acute bu t ben ign , rare, un ilateral disease of u n kn ow n et iology involving th e RPE an d ch oroidal capillaries. It u su ally affect s young fem ales. It can rarely be bilateral (Fig. 6.3).

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Fig. 6.3 Multiple evanescent white dot syndromes (MEWDS). (A) Fundus examination revealed sm all and large spots scat ted through the fundus in the left eye. (B) Fluorescein angiography showed punctate hyperfluorescence. (Courtesy of Antonio Ciardella, MD). (Continued on page 204)

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Fig . 6.3 (Continued) Multiple evanescent white dot syndromes (MEWDS). (C) Indocyanine green videoangiography demonstrated small and large hypofluorescent spots. A diagnosis of MEWDS was made. (D) One month later the spots in the fundus had disappeared, and visual acuit y recovered. (Courtesy of Antonio Ciardella, MD).

Presentation

Mu lt iple w h ite dot s at th e level of th e RPE an d opt ic n er ve h ead sw elling m ay be seen . Th e m acula often h as a gran u lar appearan ce. Visual-field defects in th e form of an en larged blin d spot an d paracen t ral an d cen t ral scotom as m ay also be seen . Visual loss, even if sign ifican t in it ially, alm ost alw ays ret urn s to n orm al later. Th e pat ien t m ay also h ave ph otopsia.

Management

Fu n du s fluorescein angiography sh ow s focal areas of early pu n ct ate hyperfluorescen ce th at corresp on d to th e w h ite dots. Th e ch anges on in do-cyan in e green angiography m ain tain longer th an fu n dus ph oto an d FFA. Th e progn osis for visu al acuit y in MEW DS is ver y good, an d it resolves on its ow n .

Acute Multifocal Posterior Placoid Pigment Epitheliopathy

Th is con dit ion closely resem bles MEW DS. It m ay be associated w ith HLA-B7.

Presentation

Sim ilar to MEW DS, it can also presen t w ith a viral predrom e follow ed by t ran sien t visu al loss in young to m iddle-aged adu lt s. It is u su ally bilateral. Ret in al lesion s con sist of m u lt iple yellow -w h ite placoid lesion s, w h ich are larger th an in MEW DS, involving th e RPE (Fig. 6.4).

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Fig. 6.5 (A) Fundus photograph and (B) hyperfluorescence on fluorescein angiography of a patient with serpiginous choroiditis demonstrating choroidal neovascularization. (Courtesy of J. Fernando Arevalo, Venezuela)

Presentation

Serpigin ou s ch oroidit is is seen m ost com m on ly in adu lts in th e fou rth to sixth decade of life. Blu rred vision is th e first sym ptom . Vit reou s varies from clear to m ildly cellular. A serpigin ou s or geograph ic (m aplike) pat tern of scars m ay presen t in th e posterior fun du s. Edges of th ese lesion s m ay be act ive, w ith a yellow -gray an d edem atou s appearan ce. As act ive lesion s becom e at ioph ied over w eeks to m on th s, n ew lesion s can occur elsew h ere or con t iguously in a sn ail-like pat tern . Scotom as m ay be seen . FFA sh ow s early hypoflu orescen ce w ith late hyp erflu orescen ce of lesion s during act ive disease (Figs. 6.5 an d 6.6).

Management

Oral steroids along w ith im m u n osu ppressan t s

Birdshot Retinochoroidopathy (Vitiliginous Chorioretinitis)

Th is is a cau se of ch ron ic p osterior uveit is, w ith a fem ale predilect ion . It is also associated w ith HLA-A29.

Presentation

Mu lt iple sm all w h ite/yellow spot s are seen scat tered about th e posterior pole in th e deep ret in a an d ch oroid . Vit rit is an d m acular edem a w ith or w ith ou t epiret in al m em bran e form at ion m ay be seen . Disk edem a an d opt ic n er ve in flam m at ion w ith peripapillar y at rophy m ay be seen (Fig. 6.7).

Management

Th ough it gen erally ru n s a ben ign course, it is poten t ially blin ding secon dar y to m acu lar in flam m at ion an d perm an en t dam age. Hen ce, in cases w ith sign ifican t in flam m at ion or vision -affect ing m acular edem a, aggressive t reat m en t sh ou ld be given .

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Fig . 6.6 Serpiginous choroiditis. (A) Color fundus photo. (B) Fluorescein angiography shows early blockage. (C) As the angiogram proceeds, the active margins progressively becom e hyperfluorescent (D) and spread toward the center of the lesion as it absorbs dye from the choriocapillaris. (Courtesy of J. Fernando Arevalo, Venezuela)

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Fig . 6.7 Birdshot retinochoroidopathy. (A) Color fundus photo of right and (B) left eye.

(C) Early fluorescein angiography with choroidal infiltration and m inim al retinal pigm ent epithelium atrophy. The spots are hypofluorescent. (D) The lesions become m ildly hyperfluorescent in the late phases of the study as dye from the choriocapillaris stains the extrachoroidal vascular space. (Courtesy of J. Fernando Arevalo, Venezuela)

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Cytomegalovirus (CMV) Retinitis

Presentation

Congenital CMV: Most com m on ly seen at th e posterior pole w ith overlying vit reous h aze an d pigm en t clum ps. Th ere can be m ild ch orioret in it is or a severe n ecrot izing ch orioret in it is. Th e lesion s are m u lt iple, an d hyperplast ic m acular scarring can be seen . Th e vision loss can be secon dar y to opt ic n er ve abn orm alit y.

Im m unosuppressed CMV ret init is: Ret in al in farcts m an ifest ing as cot ton w ool spot s, ret in al h em orrh ages, ret in al n ecrosis, vit reou s h aze, an d ret in al vasculit is are seen (Figs. 6.8 an d 6.9).

Management

CMV ret in it is is t reated w ith in t raven ou s gan ciclovir (5 m g/kg ever y 12 h ou rs) or in t raven ous foscarn et (60 m g/kg ever y 8 h ours) or cidofovir. In t ravit real inject ion s m ay also be given .

Fig . 6.8 Cytomegalovirus retinitis.

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Fig. 6.9 Cytomegalovirus (CMV) retinitis in an AIDS patient. (A) Early indocyanine green angiography (ICG-V) frame shows the onset of fluorescence. Engorged leaking choroidal vessels (white arrows) indicating inflammatory choroidal vasculopathy. (B) Maximum fluorescence on ICG-V of leaking choroidal vessels (white arrow). (Courtesy of J. Fernando Arevalo, Venezuela)

Acute Retinal Necrosis Syndrome

Presentation

Th is syn drom e is a form of severe posterior uveit is th at is cau sed by varicella zoster virus an d h erp es sim plex viru s t ypes 1 an d 2. It is defin ed by th e follow ing ver y defin ite clin ical ch aracterist ics: presen ce of focal, defin ed areas of ret in al n ecrosis in th e perip h eral ret in a out side th e vascular arcades, rapid p rogression to con flu - en t circu m feren t ial n ecrosis, occlusive vasculopathy, m arked vit rit is, an d irit is.

Oth er feat ures are opt ic at rophy, sclerit is, an d p ain .

Management

System ic an t ivirals su ch as acyclovir an d valacyclovir are given in it ially follow ed by oral steroids. An t icoagu lan t th erapy m ay be requ ired . Prophylact ic laser ph otocoagulat ion or pars plan a vit rectom y m ay be required .

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Vogt-Koyanagi-Harada Syndrome

Presentation

Headach e, n au sea

Bilateral vit rit is, ch oroidit is, an d m ult iple oval det ach m en t s of th e ret in a th at are exu dat ive

Ret in al an d peripapillar y n eovascu larizat ion s

Mu t ton fat kerat ic precipit ates

Papillar y n odules an d sh allow an terior ch am bers

Differen t ial diagn osis: sym path et ic oph th alm ia, sarcoidosis, acu te posterior m u lt ifocal placoid pigm en t epith eliopathy

Associated fin dings in clude alopecia, vit iligo, poliosis, an d h earing loss (Figs. 6.10 an d 6.11).

Management

Treat aggressively w ith cycloplegics; top ical, periocular, an d system ic steroids; an d/or im m u n osu ppressive agen t s.

Fig. 6.10 Occlusion (arrows) of choroidal vessels in a patient with Vogt-Koyanagi-Harada syndrome. (Courtesy of J. Fernando Arevalo, Venezuela)