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

9 Medical Retina 271

Fig . 9.6 (A) Angioid streaks—color fundus photo. (B) Angioid streaks—fundus fluorescein angiography.

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B

progressing to legal blin dn ess. Pseudoxanthom a elast icum is associated w ith peau d’orange an d m ot tled backgrou n d fun du s appearan ce. Focal m idp eriph eral fin e exu dates an d opt ic n er ve h ead dru sen are also associated . Loose skin folds an d plaqu elike lesion s can be seen in th e n eck (ch icken skin appearan ce), an d on th e flexor aspect s of th e join ts.

Differential Diagnosis

Myopic lacquer cracks, ch oroidal ru pt u re

Management

FFA is ver y h elpfu l in delin eat ing angioid st reaks. Fluorescein angiography (FA) reveals early hyperfluorescen ce du e to t ran sm ission defects th rough at roph ic RPE presen t n ext to th e st reak. Late-ph ase FFA sh ow s leakage at th e m argin of angioid st reaks from adjacen t ch oriocapillaries an d deep ch oroidal vessels. Any eviden ce of ch oroidal n eovascu lar m em bran e is also picked on FFA. In docyan in e green an - giography is superior to fu n dus flu orscein in diagn osing th e details of a ch oroidal n eovascular m em bran e. Laser ph otocoagulat ion for ju xtafoveal an d p arafoveal lesion s an d ph otodyn am ic th eor y (w ith in t ravit real t riam cin olon e aceton ide) for su bfoveal CNVM h ave been recom m en ded .

Skin biopsy an d cardiological w orkup for su spected Pseudoxanthom a elast icum , seru m alkalin e ph osph atase, an d calcium levels for su spected Paget disease an d h em oglobin elect roph oresis for su spected sickle cell an em ia are recom m en ded .

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Degenerative Myopia

Progressive m yopia of m ore th an –6D or eyes w ith an axial length greater th an 26 m m h ave st ru ct u ral alterat ion s of th e globe result ing in axial length en ing, especially in th e posterior pole, leading to st retch ing of th e ret in a, th in n ing of th e sclera, ch oroidal degen erat ion , an d poten t ial loss of vision .

Presentation

Decreased best-corrected visual acu it y an d progressive ch ange in p ow er are seen , usually beyon d th e fifth decade. Posterior segm en t fin dings in clu de vit reou s liquefact ion , m yopic crescen t , t ilted disk, posterior st aphylom a, RPE abn orm alit ies, patchy ch oroidal at rophy, Fu ch s spot (dark area cau sed by CNV), lat t ice degen era- t ion in th e periph er y, an d lacquer cracks. Risk for rh egm atogen ous ret in al det ach - m en t is in creased, especially w ith con cu rren t lat t ice degen erat ion (Fig. 9.7).

Ocu lar associat ion s in clude cat aract , prim ar y op en -angle glaucom a, p igm en tar y glaucom a, an d ret in opathy of prem at u rit y. System ic associat ion s in clu de Marfan syn drom e, Eh lers-Dan los syn drom e, an d St ickler syn drom e.

Differential Diagnosis

Ocu lar h istoplasm osis, gyrate at rophy, congen ital staphylom a, age-related ch oroidal at rophy, ARMD, an d angioid st reaks

Management

No t reat m en t w ill regress th e progression of staphylom a. An n u al to bian n u al careful exam in at ion of th e fu n dus is recom m en ded . Sym ptom at ic ret in al breaks sh ou ld be t reated w ith laser ph otocoagulat ion , cr yoth erapy, or scleral-bu ckling surger y.

Fig . 9.7 High myopia with chorioretinal degeneration and parapapillary atrophy.

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Differential Diagnosis

Macu lar h ole, differen t cau ses of CNV, ARMD, ch oroidal t um ors an d m et astasis, CME, an d ret in al detach m en t

Management

Th e Am sler grid sh ow s distorted lin es or closely crow ded lin es. FFA dem on st rates th e site of th e leak in a t ypical sm okest ack pat tern . OCT sh ow s a hyporeflect ive flu id accu m ulat ion bet w een th e n eurosen sor y ret in a an d th e RPE layer. A deposi- t ion of lipofuscin m ay be associated w ith CSCR, con fu sing it w ith vitelliform dys- t rophy. OCT con firm s th e diagn osis by dem on st rat ing th e lipofuscin deposited below RPE. ICG angiography m ay h elp in som e cases. ERG sh ow s a deficit in both eyes in a pat ien t w ith u n ilateral CSCR poin t ing tow ard a system ic path ology.

Progn osis is gen erally good, w ith 85%of th e p at ien ts gain ing back 20/20 vision in 6 to 8 w eeks. Laser ph otocoagu lat ion of th e leaking area sh or ten s th e disease cou rse. It is don e on ly w h en th e leak is n oted 300 µm from th e cen ter of th e fovea an d w h en th e disease process th reaten s to be ch ron ic. Laser is an opt ion in cases w h ere th ere is a persisten t fluid level for m ore th an 4 m on th s, recurren ce in an eye w ith visual deficit in th e oth er eye, presen ce of visu al deficit s in th e opposite eye from previou s episodes of CSCR, an d occup at ion al n eed requiring prom pt recover y of vision .

Plasm in ogen act ivator in h ibitor 1 h as been fou n d to h ave associat ion w ith CSCR. Car valh o-Recch ia et al an d Haim ovici et al h ave derived a sign ifican t relat ion sh ip bet w een pat ien ts on exogen ous steroids an d CSCR. ICG angiography repeatedly dem on st rates both m ult ifocal ch oroidal hyperperm eabilit y an d hypoflu orescen t areas suggest ive of focal ch oroidal vascular com prom ise in a pat ien t w ith CSCR. Recen tly PDT h as been used to t reat su ch areas of hyperperm eabilit y in a case w ith CSCR.

Cystoid Macular Edema

CME is th e accu m u lat ion of fluid (edem a) in th e in t racellu lar spaces of ou ter plexiform an d in n er n uclear layers of th e ret in a, cau sing Mü ller cell degen erat ion w ith in t racellular vacu olat ion . CME w ith a postcat aract su rger y cau se is called Ir vin e- Gass syn drom e. It is th e m ost com m on t ype. Oth er causes can be uveit is, diabet ic m acu lopathy, AMD, ret in al vein occlu sion , ret in it is pigm en tosa (RP), vit reom acu - lar t ract ion syn drom e, topical epin eph rin e, CNV, ret in al vascu lit is, ret in al telangiect asias, in t raocu lar t um ors, n icot in ic acid, an d juven ile ret in osch isis.

Th ere are m any hypoth eses to describe th e m ech an ism leading to CME; h ow - ever, th e m ost acceptable is th e on e involving in t raocular in flam m at ion , w h ich occurs w h en CME com plicates uveit is or t ract ion /distor t ion of th e iris. In flam m ator y m ediators gen erated from th e arach idon ic acid m et abolism , such as th e prostaglan din s an d leukot rien es, h ave been im plicated in th e p ath ogen esis (Fig. 9.9A,B).

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A

Presentation

Pat ien t presen ts w ith gradu al loss of vision or m et am orph opsia an d a h istor y of a cer t ain p recipit at ing factor. Biom icroscopy reveals ch aracterist ic cyst ic in tercellu - lar sp aces in th e m acular region w ith dim in ish ed foveal ligh t reflex an d associated foveal th icken ing. Eviden ce of in t raocular in flam m at ion like ciliar y flush , vit reou s cells, n er ve fiber layer edem a, or papilloedem a m ay be presen t .

In Ir vin e- Gass syn drom e, sign s of com plicated cat aract su rger y m ay be p resen t , su ch as vit reous t ract ion , vit reou s loss, polym erase ch ain react ion , exch ange of in t raocular len s (IOL), secon dar y IOL, old age, post–pen et rat ing keratoplast y, post–YAG capsu lotom y, topical epin eph rin e, or latan oprost .

Differential Diagnosis

Ret in al telangiect asias, juven ile ret in osch isis, ph ototoxic injur y, Berlin edem a, CNV, an d foveal cyst s

Management

FFA sh ow s an early an d m idph ase leak from th e ch oriocap illaries surroun ding fovea. In th e late ph ase a t ypical petaloid or sp oke-w h eel pat tern of pooling of dye is eviden t . An associated con dit ion can also be p icked up in FFA. OCT reveals th e cys- t ic spaces presen t in th e layers of ret in a. It can also be u sed as a m on itoring device becau se it can m ain t ain a serial foveal th ickn ess record of a pat ien t .

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Sp on t an eou s resolut ion occu rs in 75%of postcataract pat ien t s w ith in a span of 6 w eeks. Cor t icosteroids h elp by in h ibit ing th e en zym e ph osph olipase an d th u s con t rolling th e in flam m at ion in post uveit ic cau ses. It can be adm in istered by all routes, su ch as topical, oral, posterior su bten on , an d in t ravit real. Non steroidal an - t iinflam m ator y drugs (NSAIDs) in h ibit th e en zym e cyclooxygen ase an d can also be u sed in th e preven t ion of CME. NSAIDs, especially ketorolac an d flu rbiprofen , can be adm in istered topically for 3 to 4 m on th s. Carbon ic an hydrase in h ibitors su ch as acetam ide are h elpfu l in con t rolling CME in pat ien ts of RP. Prolonged CME due to BRVO respon ds w ell to laser ph otocoagu lat ion .

Th e role of p ars plan a vit rectom y is in dicated in CME related to uveit is. It is h elpfu l in rem oving vit reou s st ran ds, in flam m ator y m ediators, ret ain ed len s frag- m en ts, an d m alposit ion ed IOLs as w ell as for greater pen et rat ion of topical an d oral cort icosteroids an d su ppressing th e an t igen -specific im m un e respon se. Th erapies such as an t im etabolites, cyclodiath erm y, cr yoth erapy, an d ph otocoagu lat ion h ave been t ried w ith lim ited success. Th e adven t of n ew m odes of steroid deliver y su ch as posterior subten on depot an d in t ravit real inject ion of steroids are bet ter op - t ion s available. Various st u dies (e.g., Italian diclofen ac st udy group ) h ave repor ted th e su periorit y of ketorolac over oth er topical agen ts in t reat ing CME.

Age -Related Macular Degeneration

Exudative Age -Related Macular Degeneration

In 1995, th e In tern at ion al ARM Epidem iologic St udy Grou p redefin ed age-related m acu lar degen erat ion (ARMD). Pat ien t s w ith m in im al to m oderate n on exudat ive age-related ch anges at th e m acula w ere classified as age-related m acu lopathy (ARM). Advan ced at rophy or th e presen ce of CNV m em bran es w as classified as ARMD. Th e con dit ion is seen m ore com m on ly in w om en th an in m en an d is diag- n osed in in dividuals over th e age of 50. Th e use of tobacco, obesit y, an d gen et ic factors are believed to be st rongly associated w ith th e con dit ion (Fig. 9.10A,B,C,D).

Presentation

Th e pat ien t u sually presen t s w ith a gradual, pain less loss of vision associated w ith delayed dark adapt at ion , severe m et am orph opsia, an d field loss. Vision loss is m ore rapid in exudat ive ARMD. A su dden loss of vision can be associated w ith su bret in al h em orrh age or pigm en t epith elium det ach m en t . Th e Am sler grid an d frequ en t slit-lam p biom icroscopy are th e best w ays to m on itor th e con dit ion (Fig. 9.10E,F,G,H).

Differential Diagnosis

Non exu dat ive ARMD, angioid st reaks, ch oroidal rupt u re, an d oth er causes leading to CNV m em bran e

Management

FFA is an im p or tan t tool in diagn osis an d classificat ion (subfoveal, ju xtafoveal, an d ext rafoveal) of CNV. ICG angiography provides a m ore specific diagn osis by pinpoin t ing th e feeder vessel in th e ch oroidal vasculat ure. OCT is u sed as a tool to follow u p after ph otodyn am ic th erapy (PDT).

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A B

C D

E F

G H

Fig . 9.10 (A) Dry age -related macular degeneration (ARMD)—color fundus photo.

(B) Dry ARMD—FFA late phase. (C) Dry ARMD, same patient, other eye—color fundus photo. (D) Dry ARMD, same patient, other eye—FFA late phase. (E) Wet ARMD—CNVM— color fundus photo. (F) Wet ARMD—CNVM—midphase FFA. (G) Wet ARMD—CNVM—late phase FFA. (H) Wet ARMD, same patient, other eye—venous phase FFA.

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Th erm al laser p h otocoagu lat ion an d feeder vessel ph otocoagulat ion can be u sed to t reat all kin ds of CNV m em bran es.

Tran spupillar y th erm oth erapy form s a t reat m en t m odalit y for occult CNV m em bran es.

PDT using ph otosen sit izers (verteporfin , rost aporfin , m otexafin lu tet ium , talaporfin sodium , ATX-S10) is w idely used to t reat classic CNV. Target-receptor PDT an d com bin at ion PDT h ave also been t ried .

An t iangiogen ic agen t s su ch as vascular en doth elial grow th factor (VEGF) in h ibitors (pegaptan ib sodium , ran ibizum ab, bevacizum ab, VEGF t rap) form th e latest adjuvan t in th e t reat m en t of CNV m em bran es.

Steroids like t riam cin olon e aceton ide, an ecor t ave acetate, an d im plan table cor- t icosteroids h ave also been t ried .

Sm all in terfering RNA th erapy, pigm en t epith elium – derived factor in ducer, an d m icrost ruct ure m odu lat ion are som e of th e t reat m en t m odalit ies u n der t rial.

Subm acu lar surgeries, m acu lar t ran slocat ion , an d RPE t ran splan tat ion are th e su rgical opt ion s at variou s st ages of developm en t .

Stargardt Disease and Fundus Flavimaculatus

At roph ic m acu lar dyst rophy w ith flecks (i.e., St argardt disease) is th e m ost com - m on t ype of juven ile m acu lar degen erat ion . Fu n dus flavim aculat us (FF) is a varian t of St argardt disease w ith a sign ifican t differen ce in clin ical presen t at ion an d progn osis. Alm ost all cases are determ in ed as autosom al recessive in th e pedigree ch ar t . Rarely, cases h ave been iden t ified as au tosom al dom in an t . Th e gen e locu s lies on ABC4R on 1p21–22. FF is also an autosom al recessive disease.

Presentation

Th ere is con siderable con fu sion related to th e term s Stargardt disease an d fundus flavim aculat us. FF w as first described as a flecked fu n dus w ith yellow -w h ite irregular lesion s th at exten ded to th e equ ator. St argardt disease occu rred as flecks in th e posterior pole early in life w ith a p rogressive m acu lar dyst rophy. It is n ow believed th at th ese t w o con dit ion s are t w o differen t allelic m an ifest at ion s of th e sam e disease.

Th e sym ptom s are m ain ly bilateral, presen t ing in th e age grou p from 6 to 20 years. Th ere is associated color vision defect an d ph otop h obia. Disappearan ce of th e foveal reflex form s th e first sign on oph th alm oscopy. Later, a gran u lar m ot tling of th e m acula appears. An oval “sn ail-slim e” or “beaten bron ze” appearan ce of th e fovea is th e h allm ark of Stargardt disease. Th e flecks m ay exten d to th e m idperiph - er y an d m ay give a salt-an d -pepper app earan ce in th e region . Th e disk an d vessels are gen erally n orm al. FF is usu ally diagn osed in ciden tally. Th e pat ien t can experien ce a reduced cen t ral visu al acu it y an d m etam orph opsia in longst an ding cases. FF is a bilateral disorder w ith ill-defin ed yellow ish w h ite flecks (“fish tail-like”) exten ding from th e posterior pole to th e m idperiph er y. Th e fu n du s h as a verm ilion t inge in m ost cases (Fig. 9.11A,B).

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Fig . 9.11 (A) Stargardt disease fundus flavimaculatus—color fundus photo. (B) Stargardt disease fundus flavimaculatus—fundus fluorescein angiography showing early hyperfluorescence.

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B

Differential Diagnosis

Fu n dus albipu n ctat us, ret in it is pu n ct a albescen s, fam ilial dru sen , p at tern dyst rophy, RP inversus, ARMD, h istosp ots

Management

FFA sh ow s th e m ost ch aracterist ic feat u re called silen t ch oroid . Th e eviden ce of flecks over th e con t rast ing dark ch oroid w ith a n orm al m acu la is a t radem ark an - giograph ic appearan ce of FF. ERG sh ow s a variable ph otopic resp on se an d n orm al scotopic respon se. Elect ro-olfactogram (EOG) is subn orm al. Deu teran an d t ritan defects are presen t . Progn osis is poor; h ow ever, visu al acuit y usu ally st abilizes at 6/60.

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Fig . 9.12 Flecked retina.

Fundus Albipunctatus

Fu n du s albip un ct at u s is congen it al st at ion ar y n igh t blin dn ess w ith autosom al recessive pat tern .

Presentation

Pat ien ts m ay be asym ptom at ic or h ave n igh t blin dn ess, u su ally st ar t ing in in fan cy. Num erou s w h ite-yellow dotlike lesion s occu r in th e m idperiph eral fun du s at th e level of th e RPE (Fig. 9.12).

Differential Diagnosis

Ret in it is p un ct ate albescen ce (progressive, beh aves like RP), birdsh ot ch orioret i- n it is, St argardt disease, fam ilial dru sen , Biet t i cr ystallin e ret in opathy, pat tern dyst rophy, RP inversu s, m acu lar degen erat ion , pseu doexfoliat ion , cr ystallin e ret i- n opathy, h istospots

Management

ERG sh ow s a prolonged t im e for dark adapt at ion to produ ce n orm al am plit u de du e to th e delay in regen erat ion of rh odopsin . Vision an d fields rem ain n orm al.

Best Disease : Vitelliform Macular Dystrophy

Best disease is an au tosom al dom in an t (variable p en et ran ce) degen erat ive m acu - lopathy w h erein a m u tat ion in th e best roph in gen e leads to lipofuscin accu m u la- t ion in RPE cells.