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

10 Surgical Retina 321

Th e t ypical appearan ce of th e blood vessels h elps in th e diagn osis. Dilated ret in al vein s an d vascular tor t uosit y w ith teth ering an d st raigh ten ing of th e vessels are seen directed to th e cen ter of th e con t ract ing m em bran e. Foveal ectopia is a com - m on fin ding. A w ide range of oth er associated fin dings are n oted, such as pun ct ate h em orrh ages, m icroan eu r ysm s, profoun d vascu lar leakage an d ret in al edem a, soft exu dates, an d ret in al tear. Macu lar pucker u sually develops after ret in al det ach - m en ts. Th ey are opaqu e th ick m em bran es w ith associated fu ll-th ickn ess ret in al folds.

Differential Diagnosis

Prim ar y cau ses of ERM: idiopath ic. Secon dar y cau ses of ERM: postocular in flam - m at ion , uveit is, ret in al det ach m en t , ret in al vascu lar occlu sion s, m acu lar h oles, post in t raocular su rgeries, post ret in al laser, cr yopexy.

Management

Vit rectom y is th e on ly t reat m en t for epiret in al m em bran es. After com plet ing th e vit rectom y, posterior hyaloid th at is adh eren t to th e ret in a is rem oved . In t ravit real t riam cin olon e can be u sed to facilit ate th is step . Th en th e m em bran e is peeled off th e su rface of th e ret in a, p referably from th e cen ter to th e p eriph er y after fin ding an edge of th e m em bran e. Recen tly in t ravit real stain s h ave been described . Tr ypan blue an d in docyan in e green are used to stain th e epiret in al m em bran e an d in ter- n al lim it ing m em bran e, resp ect ively.

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Management

Th e n at ure of th e lesion can be ascert ain ed by eith er fin e-n eedle aspirat ion or excision biopsy. If th e t u m or is large it can be t reated w ith argon laser ph otocoagu - lat ion or by inject ing eth an ol in to th e lesion w h en it is recalcit ran t .

Iris Melanoma

Iris t u m ors con st it u te 8% of uveal t u m ors presen t ing in th e younger age grou p (40 to 50 years) w ith a sligh t fem ale predilect ion . Th ey u su ally h ave a bet ter prog- n osis th an oth er uveal m elan om as.

Presentation

Th e lesion can presen t as t ypical (spot), diffu sely grow ing (diffuse color ch ange), or t apioca m elan om a (m ult iple su rface n odu les). Lesion s are m ostly asym ptom - at ic, involving th e in ferior iris m ore com m on ly, bu t can presen t as a rise in IOP, hyph em a, or cat aract . Risk factor for m align an t ch ange is th e sam e as th at for iris n evus.

Differential Diagnosis

Iridocorn eal en doth elial (ICE) syn drom e, n evus, aden om a an d ciliar y body t u m or, iris cyst , in t raocular foreign body (IOFB), juven ile xan th ogran u lom a, an d leiom y- om a

Management

B-scan an d biopsy h elp to con firm th e diagn osis. Biopsy can be fin e n eedle asp ira- t ion cytology or in cision al biopsy, eith er th rough a corn eal or a lim bal approach . Th e lesion can be obser ved u n t il sym ptom at ic or over t m align an t ch anges occu r for w h ich excision (iridectom y or iridocyclectom y), proton -beam radioth erapy, or rarely en ucleat ion h ave to be u n der taken .

Th e progn osis is usu ally good w h en th e lesion is sm all w ith out ciliar y body or ext rascleral exten sion .

Juvenile Xanthogranuloma

Juven ile xan th ogran u lom a is a rare idiopath ic gran ulom atou s disease of ch ildh ood du e to proliferat ion of n on -Langerh an s h ist iocytes. It can presen t eith er as cu t an e- ous lesion s or iris lesion s. Iris in filt rat ion is th e m ost com m on ocu lar m an ifesta- t ion . It m ay also rarely involve th e orbit , eyelids, corn ea, episclera, ciliar y body, ch oroid, an d opt ic disk.

Presentation

Cu tan eous lesion s presen t as yellow ish papu les w ith spon t an eou s regression . Iris lesion s are eith er localized or diffu se. Th e pat ien t m ay presen t as h eteroch rom ia of th e iris. Th e lesion s are usu ally associated w ith spon t an eou s hyph em a. An terior uveit is an d glau com a are oth er less com m on presen t at ion s.

Differential Diagnosis

Ret in oblastom a, sarcoid, leukem ia, foreign body, m eduloepith eliom a

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Management

Treat m en t opt ion s in clude local cor t icosteroids, irradiat ion , an d a com bin at ion of both an d excision .

Leiomyoma (Uveal)

Leiom yom a is an ext rem ely rare ben ign lesion arising from th e sm ooth m uscle. Th is is ver y sim ilar to am elan ot ic m elan om a, bu t gen erally it is n ot con fin ed to th e in ferior iris on ly.

Presentation

Pat ien ts t ypically presen t w ith a localized, flat to m ildly elevated, ligh tly pigm en ted or n onpigm en ted, vascular lesion in th e area of th e iris sph in cter. Th e lesion m ay also occu r periph erally or in th e an terior ch am ber angle.

Differential Diagnosis

Am elan ot ic iris m elan om a, m et astat ic iris lesion s

Management

Fin e-n eedle aspirat ion cytology (FNAC) w ith elect ron m icroscopy an d im m un oh istoch em ist r y can diagn ose an d differen t iate th e con dit ion from oth ers. Th ere is n o kn ow n m et astat ic p oten t ial.

Leukemic Iris Nodules

Leu kem ic iris n odules are m ore com m on in ch ron ic leu kem ias rath er th an in acu te leukem ia. Iris in filt rat ion can presen t as iris n odules.

Presentation

Iris involvem en t m ay be u n ilateral or bilateral. It is a vascularized lesion . It m ay be associated w ith an terior uveit is w ith or w ith out hypopyon . In filt rat ion can also occur in to th e conju n ct iva, orbit , an d opt ic n er ve. Glau com a m ay be p resen t due to blockage in th e filt rat ion from th e t rabecu lar m esh w ork. Leu kem ic ret in opathy du e to eith er direct in filt rat ion or secon dar y to an em ia h as to be looked for.

Differential Diagnosis

Ret in oblastom a, uveit is, t rau m a

Management

Workup involving an on cologist is p referred . Treat m en t con sists of system ic ch e- m oth erapy w ith localized radioth erapy. Topical steroids cau se quick bu t tem p o- rar y resolu t ion . Cen t ral n er vou s system (CNS) evaluat ion is m ust .

Melanocytosis

Ocu lar m elan ocytosis is an un com m on , congen ital, prem align an t con dit ion . Nevu s of Ot a is th e com m on varian t follow ed by th e lim ited derm al an d ocu lar form s. Th is is m ore com m on in fem ales an d Asian s.

11 Ocular Neoplasms 325

Presentation

Melan ocytosis presen ts as un ilateral hyperpigm en tat ion of th e face w ith ipsilateral iris hyperch rom ia, iris m elan ocytosis, glau com a (10%), an d lid pigm en t at ion . Th e con dit ion m ay be associated w ith pigm en tat ion of th e ext raocular skin w ith ipsilateral uveal t ract an d m en ingeal involvem en t .

Differential Diagnosis

St u rge-Weber syn drom e, racial p igm en tat ion

Management

Becau se th e hyperpigm en ted area h as a predisposit ion to develop m align an t m ela- n om a, regular close follow -u p ever y 6 to 12 m on th s is requ ired . Any suspiciou s lesion sh ould be w orked up accordingly for m align an t m elan om a.

Brushfield Spots (Dow n Syndrome)

Br ush field spot s are seen in Dow n syn drom e. Th ey involve th e periph eral st rom a, can be m u lt iple, an d th ey occu r as m u lt iple, pale, ben ign lesion s involving th e periph eral iris st rom a. Th e lesion s appear as pale lesion s. Th ese can also be seen in n orm al in dividu als. Sarcoid n odu les, iris n evu s, or freckles m ust be ru led out . No t reat m en t is required .

Lisch Nodules (Neurofibromatosis)

Lisch n odules are u su ally associated w ith n eurofibrom atosis. Th ey are sm all an d m u lt iple. Th ey ap pear sim ilar to iris n evi, w h ich are ben ign an d bilateral. Th ey are presen t in n eurofibrom atosis 1 in all pat ien t s after age 16 years. Th ey con sist of a collect ion of glial cells an d m elan ocytes. Th ey n eed to be differen t iated from iris n evus. Clin ical docum en t at ion an d follow -u p are required .

Ciliary Body Tumors

Ciliary Body Melanoma

Ciliar y body m elan om as con st it u te ~12% of all m align an cies an d u sually appear bet w een th e ages of 50 an d 60 years.

Presentation

Clin ically th ey are asym ptom at ic, alth ough th ey can produce visual sym ptom s occasion ally. Th ey are seen as ciliar y body m ass w ith sen t in el vessels overlying. Th ey can exten d on to th e iris or globe. Len s sublu xat ion , angle-closu re glau com a, spon tan eous hyph em a, secon dar y cat aract , an d an terior uveit is are com m on associat ion s.

Ciliary body m elanom as are well detected by ultrasound and biopsy using FNAC.

Differential Diagnosis

Ciliar y body aden om a, ciliar y body cyst , uveal effusion syn drom e, m eduloepith e- liom a, leiom yom a, an d m et astat ic lesion s

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Management

Th e t u m ors are am en able to excision w h en sm all. Radioth erapy, eith er as brachyth erapy or p roton beam th erapy, is an oth er opt ion . En ucleat ion is don e if oth er m odalit ies are n ot possible. Progn osis is u su ally w orse.

Medulloepithelioma (of the Ciliary Epithelium)

Medulloepith eliom a arises from im m at u re epith elial cells of th e em br yon ic op - t ic cu p an d are rare, slow -grow ing t u m ors th at presen t in ch ildren you nger th an 10 years of age, w ith n o sexual predilect ion . On e th ird are ben ign an d t w o th irds are m align an t . Th ey can be eith er teratoid or n on teratoid . Th ey origin ate from th e n onpigm en ted ciliar y epith eliu m predom in an tly involving th e iris an d ret in a, th ough oth ers can also be involved . Metast asis is rare.

Presentation

Clin ically pat ien t s can presen t w ith red eye an d decreased vision or w ith an iris m ass lesion w ith a ch ange of color. Tu m or m ay also be seen at th e opt ic disk. Neovascu lar glaucom a, len s colobom a, or sublu xat ion an d cataract are th e com m on com plicat ion s.

Differential Diagnosis

Persisten t hyperplast ic prim ar y vit reou s (PHPV), pars plan it is, en doph th alm it is, congen it al glaucom a, ret in oblastom a, m align an t glau com a

Management

Ult rasoun d is useful in th e diagn osis. Sm all, w ell-defin ed lesion s of a ben ign n a- t u re are t reated by iridocyclectom y, an d en ucleat ion is reser ved for t u m ors n ot am en able to oth er t reat m en t m odalit ies.

Choroidal Tumors

Choroidal Nevus

Ch oroidal n evus is a ben ign m elan ocyt ic t u m or of th e ch oroid an d is fou n d in 5 to 30% of w h ite person s. Th ese are usually foun d in ciden t ally because th ey are asym ptom at ic an d are th e com m on est of all in t raocu lar m align an cies.

Presentation

Clin ically th ey are asym ptom at ic but can p resen t w ith a decrease in vision . Lesion s close to th e fovea can cau se vision loss. Th ey are sm all, gray-brow n , h om ogen eou s lesion s. Th e lesion m ay be associated w ith lip ofu scin deposit s an d dru sen , or w ith su bret in al flu id (SRF) is absen t . Th ese lesion s gain im port an ce in th e fact th at th ey h ave to be differen t iated from m align an t m elan om a. Malign an t t ran sit ion is h er-

11 Ocular Neoplasms 327

Fig . 11.1 Choroidal nevus, fundus photograph shows a pigmented, placoid, well-demarcated choroidal mass with overlying drusen located temporal to the fovea.

alded by th e presen ce of sym ptom at ic lesion s, w ith in creasing th ickn ess, lesion s w ith lipofu scin pigm en t an d SRF, an d an in crease in IOP (Fig. 11.1).

Differential Diagnosis

Ch oroidal m elan om a, congen it al hyper t rophy of ret in al pigm en t epith eliu m (RPE), com bin ed h am ar tom a, hyperplasia of RPE, subret in al h em orrh age, ch oroidal h em - angiom as, ch oroidal osteom a, an d m etast at ic t um or.

Management

Lesion m u st be invest igated w ith fu n dus fluorescein angiograp hy (FFA) an d B-scan to ru le ou t oth er lesion s, especially m align an t m elan om a. Lesion s sh ould be closely m on itored . Any ch anges in th e m orph ology of th e lesion s sh ou ld be alarm ing.

Choroidal Melanoma

Ch oroidal m elan om as con st it u te ~80%of uveal m elan om as presen t ing aroun d 50 to 60 years of age. Th ey are classified based on th e size of th e t um or as sm all (<10 m m ), m ediu m (10 to 15 m m ), an d large (>15).

Presentation

Th ey can presen t as a decrease in vision or field loss, or flash es of ligh t m ay be th e presen t ing feat ures, bu t th ey are oth er w ise asym ptom at ic. Th ey can presen t as an elevated su b-RPE m ass an d can be am elan ot ic. Lipofu scin deposit s, exu dat ive ret in al det ach m en t , an d Bruch m em bran e ru pt ure w ith vit reous h em orrh age are t ypical feat u res of th e lesion . Cert ain t u m ors are diffu se rath er th an being n odular. Pat ien ts at t im es m ay com plain of blurr y vision , visual-field loss, floaters, ph otop - sia, an d pain . Oth er associated fin dings can be ret in al detach m en t , angle-closu re

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glaucom a, rubeosis iridis, vit reous an d subret in al h em orrh ages, an d len s su blu x- at ion s (Fig. 11.2).

Differential Diagnosis

Ch oroidal n evus, congen ital hyper t rophy of RPE, com bin ed h am ar tom a, hyperplasia of RPE, subret in al h em orrh age, ch oroidal h em angiom as, ch oroidal osteom a, an d m et astat ic t u m or

A

B

11 Ocular Neoplasms 329

Management

Ch oroidal m elan om as can usu ally be p icked u p by u lt rasoun d as acoust ically h ollow, low in tern al reflect ivit y solid t u m ors associated w ith ch oroidal excavat ion . Ext raglobular exten sion is to be ru led ou t by com pu ted tom ography/m agn et ic reson an ce im aging (CT/MRI) along w ith a system ic w orkup for m et astasis. FNAC is con t rain dicated .

Treat m en t guidelin es are given by ocu lar m elan om a st u dy. Tran s-pu pillar y th er- m oth erapy can be t ried w h en th e lesion is aw ay from th e disk an d th e m acu la an d w h en th e lesion is pigm en ted . Radioth erapy eith er as plaqu e or as proton beam irradiat ion . Local resect ion can be used w h en th e t u m or is sm all an d th ere is n o m etast asis. In advan ced cases orbital exen terat ion is th e opt ion .

Choroidal Metastasis

Ch oroidal m etast asis is th e m ost com m on form of in t raocu lar m align an cy in adu lts, w ith m ore th an 85%of m etast at ic uveal n eoplasm s being ch oroidal. It usually m e- tast asizes from breast can cer in w om en an d lung can cer in m en . Less com m on sites of origin in clude th e prost ate, th e kidn ey, th e thyroid, an d th e gast roin test in al t ract . Lym ph om as an d leukem ias m ay also m etast asize to th e eye an d th e orbit . Metastases are usu ally bilateral, m u lt iple lesion s.

Presentation

Most pat ien t s are asym ptom at ic or m ay com plain of flash es, floaters, or m et am orph opsia. Ch oroidal m etastases are u sually seen as solid, flat , plaqu elike, m ot tled, yellow -brow n lesion s w ith or w ith ou t associated serou s ret in al det ach m en t (Fig. 11.3).

Differential Diagnosis

Ch oroidal h em angiom a, ch oroidal osteom a, exophyt ic ret in al cap illar y h em an - giom a, oth er m etast at ic carcin om as, posterior sclerit is, h em orrh agic RPE det ach - m en t , an d am elan ot ic ch oroidal m elan om a

A B

Fig . 11.3 (A) Choroidal metastasis, tallow pink smooth, dome -shaped juxtapapillary choroidal mass. (B) Choroidal m etastasis. Fundus fluorescein angiography (FFA) shows hypofluorescence in the arterial and early venous phases.

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Management

A com bin ed approach in con su ltat ion w ith th e m edical on cologist an d radiat ion th erapist is requ ired . Th ough ch em oth erapy m ay be in dicated in m any cases, radiat ion th erapy in th e form of extern al beam radioth erapy is usually th e m ore defin it ive t reat m en t . Early t reat m en t offers th e best h ope for preser ving vision . Surger y is rarely required . If th e prim ar y is n ot readily iden t ifiable, a biopsy m ay be required prior to t reat m en t .

Cavernous Hemangioma of the Choroid

Cavern ou s h em angiom a is a ben ign vascular h am artom a. Lesion s can be localized or diffuse.

Presentation

Cavern ou s h em angiom a is congen it al an d asym ptom at ic u n t il adulth ood, at w h ich t im e it can presen t w ith loss of vision . It can presen t eith er as an isolated, circum - scribed lesion w ith ou t system ic associat ion or as a diffu se form along w ith oth er system or ocu lar abn orm alit ies (St urge-Weber syn drom e). Circum scribed lesion s are seen as an elevated, orange-red, ch oroidal m ass. Th e diffuse form is seen as a deep red ch oroid, especially in th e posterior pole com pared w ith n orm al. Oth er feat ures in clu de fibrous ch ange of RPE, tor t u ou s ret in al vessels, cyst ic ch ange, or serou s det ach m en t of ret in a (Fig. 11.4).

Differential Diagnosis

Ch oroidal n evus, congen ital hyper t rophy of RPE, com bin ed h am artom a, hyperplasia of RPE, su bret in al h em orrh age, ch oroidal m elan om a, ch oroidal osteom a, an d m etast at ic t u m or

Management

Ult rason ography is th e com m on invest igat ion an d sh ow s h igh in tern al reflect ivit y. Fu n dus flu orescein angiography (FFA) sh ow s early hyperflu orescen ce due to in t ralesion al vessels an d later hyperfluorescen ce of th e w h ole lesion . In docyan in e green (ICG) h elps in delin eat ing th e lesion bet ter th an FFA. MRI h elps in diffuse form . Brain an d system ic w orkup to r ule ou t h em angiom a in oth er sites is essen - t ial. Histopath ology reveals cavern ou s vascular ch an n els (n orm al en doth elial cells an d su ppor t ing sept a) an d capillaries like vessels in th e diffuse form .

Fig . 11.4 Choroidal hemangiom a, dome -shaped, juxtapapillary, red- dish-orange choroidal mass.