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

432 Color Atlas of Ophthalm ology

Presentation

After th e prim ar y in fect ion result ing in ch ickenpox, th e virus rem ain s dorm an t w ith in th e body in th e t rigem in al an d dorsal root ganglia an d m ay becom e react i- vated to produce h erp es zoster. Th e risk of zoster in creases w ith age an d im m u n o- su ppression . System ic involvem en t m ay cause seriou s com plicat ion s.

Ocu lar involvem en t:

Acute conjun ct ivit is w ith or w ith out secon dar y bacterial in fect ion

Pseu doden drites an d corn eal u lcers

St rom al kerat it is

Uveit is, ret in it is, opt ic n eu rit is, oph th alm oplegia

Management

Man agem en t is in coordin at ion w ith th e n eu rologist , in fect ious disease specialist , an d derm atologist . Treat m en t is based on th e severit y, age, an d im m un e state. An - t iviral m edicat ion s are th ough t to decrease th e du rat ion of sym ptom s an d th e likelih ood of posth erpet ic n euralgia w h en star ted w ith in 2 days of th e on set of rash . Medicat ion s available in clu de acyclovir, valacyclovir, pen ciclovir, an d fam ciclovir.

Rheumatoid Arthritis

Rh eu m atoid arth rit is (RA) is an au toim m un e system ic disease ch aracterized by asym m et rical, dest r uct ive, deform ing, in flam m ator y polyar th ropathy, in associa- t ion w ith a spect ru m of ext raart icu lar m an ifest at ion s. Associat ion of HLA-DW 4/ DR4 occu rs in w h ite person s.

Presentation

Wom en are m ore frequ en tly affected th an m en (3:1). RA is com m on ly seen in th e fou r th decade. Fever, m alaise, w eigh t loss, m orn ing st iffn ess, join t pain an d in - flam m at ion , an d lim itat ion of m ovem en ts are th e m ain com plain ts. Uln ar devia- t ion an d sw an n eck deform it y, bursal effusion , Baker cyst an d h allu x valgus are seen . Ext raocular fin dings in clude superficial rh eum atoid n odu le, pericardit is an d pleural effusion , Caplan syn drom e, an d Felt y syn drom e.

Oph th alm ic m an ifestat ion s in clude keratoconju n ct ivit is sicca, sclerit is, periph - eral u lcerat ive kerat it is, an d acqu ired superior oblique ten don sh eath syn drom e.

Management

Laborator y invest igat ion s in clu de rh eu m at ic factor (RF), an t in uclear an t ibodies (ANA), CBC, an d rh eum atology con su ltat ion . Non steroidal an t iin flam m ator y drugs (NSAIDs), gold salts, D-pen cillam in e, hydroxych loroquin e, sulp h asalazin e, steroids, an d cytotoxic agen ts are th e drugs u sed .

15 Ocular Manifestations of Systemic Disease 433

Systemic Lupus Erythematosis

System ic lu pus er yth em atosu s (SLE) is an au toim m un e, n on – organ -specific con - n ect ive t issu e disease ch aracterized by n u m erous an t ibodies an d circulat ing im - m u n e com plexes, w h ich m ediate w idespread vascu lit is an d t issu e dam age.

Presentation

Predom in an tly affects you ng w om en (9:1), frequ en tly in th e th ird or four th de-

cade. Com m on presen t ing sym ptom s in clu de fever, fat igu e, an d w eigh t loss.

Oph th alm ic feat u res in clude m adarosis, keratoconju n ct ivit is sicca, periph eral ulcerat ive kerat it is, sclerit is, ret in al vascu lit is, an d opt ic n eu ropathy.

Ext raocular m an ifest at ion s in clude th e follow ing:

Mucocutaneous: Bu t terfly facial rash , discoid rash , vasculit is, alopecia, oral ulcerat ion , Rayn aud ph en om en on

Muscu loskeletal: Ar th rit is, m yosit is, ten don it is

Renal: Glom eru lon ep h rit is

Cardiovascular: Pericardit is, en docardit is, m yocardit is, ar terial an d ven ous occlu sion s.

Pulm onary: Pleu risy, atelectasis

Hem atopoiet ic: An em ia, th rom bocytopen ia, lym ph open ia, leu copen ia

Ret iculoendothelial: Splen om egaly, lym ph aden opathy

Neurological: Polyn eurit is, cran ial-n er ve palsies, spin al cord lesion s, epilepsy

Management

Laborator y invest igat ion s in clude CBC, ANA, dou ble st ran ded/single st ran ded (DS/SS) DNA, SSA/SSB (Sjögren syn drom e), er yth rocyte sedim en tat ion rate (ESR), an t iph osph olipid an t ibody, lupu s an t icoagulan t , an d a rh eu m atologist’s opin ion . Th erapies in clu de an t im alarials, NSAIDs, steroids, an d cytotoxic agen t s.

Sjögren Syndrome

Sjögren syn drom e (SS) is ch aracterized by autoim m u n e in flam m at ion an d dest ru ct ion of lacrim al an d salivar y glan ds.

Prim ary SS: W h en it exist s in isolat ion

Secondary SS: W h en it is associated w ith oth er disease such as RA an d SLE

Presentation

Prim ar y SS affect s w om en m ore com m on ly th an m en . It presen ts in adu lt life w ith grit t in ess of th e eyes an d dr yn ess of th e m outh . En largem en t of th e salivar y glan ds w ith dim in ish ed salivar y flow rate an d a dr y fissu red tongue, dr y n asal passages, dim in ish ed vagin al secret ion s, Rayn au d ph en om en on , an d cut an eou s vascu lit is are th e feat u res of th e disorder.

Th e m ain ophthalm ic feat ures in clude keratoconjun ctivitis sicca and Adie pupil.

434 Color Atlas of Ophthalm ology

Management

Laborator y tests in clude CBC, rh eu m atoid factor, ANA, SSA/SSB, cr yoglobulin s, circulat ing im m u n e com plexes, gam m aglobu lin s, an d an t ithyroid an t ibodies. Diagn osis can be con firm ed by salivar y glan d biopsy. Im por tan t th erapies in clude system ic steroids an d cytotoxic agen ts.

Lyme Disease

Lym e disease is an in fect ion cau sed by a spiroch ete, Borrelia burgdorferi, t ran sm itted th rough th e bite of a deer t ick, Ixodes dam m ini. It is m ost com m on ly rep or ted from th e n or th eastern Un ited St ates.

Presentation

Follow ing are th e m an ifestat ion s of Lym e disease.

Early st age

Presen t s several days after th e bite

Er yth em a ch ron icu m m igran s

Con st it u t ion al sym ptom s, lym ph aden opathy

Neu rological an d cardiac com plicat ion s m ay follow w ith in 3 to 4 w eeks of th e in it ial m an ifest at ion .

Late stage

Ch ron ic ar th rit is of th e large join ts

Polyn europathy, ch ron ic acroderm at it is

Oph th alm ic m an ifest at ion s in clu de th e follow ing:

Ph otoph obia, pain , periocular edem a, conjun ct ivit is

Kerat it is, an terior uveit is, in term ediate uveit is, opt ic n eu rit is, n eu roret in it is, ocu lar m otor n er ve palsies

Periph eral m u lt ifocal ch oroidit is, ret in al periph lebit is

Management

Ch eck for a h istor y of t ick bite, exposure, an d th e ch aracterist ic rash . Perform a th orough derm atological, n eu rological, an d ocu lar exam in at ion . Im m un ofluorescen ce an d ELISA m ust be perform ed . Western blot is con firm ator y. Th erapies in - clude oral doxycyclin e an d in t raven ou s an t ibiot ics.

Leprosy

Leprosy is a ch ron ic gran ulom atou s in fect ion cau sed by an in t racellu lar acid -fast bacillu s, Mycobacterium leprae.

Presentation

Leprom atou s leprosy is a gen eralized, m ult isystem in fect ion .

Sk in: W h en it exists in isolat ion

Nose: W h en it is associated w ith oth er disease such as RA an d SLE

15 Ocular Manifestations of Systemic Disease 435

Neurological: Sen sor y n europathy, au ton om ic n eu ropathy, an d m otor n eu - ropathy

Tu bercu loid leprosy is rest ricted to th e skin an d periph eral n er ves.

Sk in: An n u lar, an esth et ic, hypopigm en ted lesion s w ith raised edges

Nerves: Th icken ing of cut an eou s sen sor y n er ves

Oph th alm ic feat ures in clude th e follow ing:

Madarosis, lagoph th alm os due to seven th -n er ve palsy, n eu rot ropic kerat it is du e to t rigem in al n er ve involvem en t

An terior uveit is (gran u lom atous)

Management

Dapson e is th e drug of ch oice. Rifam picin an d clofazim in e con t ributes in m ult idrug th erapy. Clofazim in e is n o longer available th rough m ost U.S. ph arm acies. Requ est s for clofazim in e to t reat leprosy sh ould be directed to th e Nat ion al Han sen’s Disease program (a division of th e U.S. Dept . of Health an d Hum an Ser vices), w h ich h olds th e IND for th is in dicat ion . It can th us be obtain ed for u se in leprosy.

Leukemia

Leu kem ias are a grou p of n eoplast ic disorders ch aracterized by abn orm al p roliferat ion of w h ite blood cells. Ocular involvem en t is m ore com m on ly seen in th e acute th an in th e ch ron ic form .

Presentation

Acute leukem ia presen ts w ith con st it u t ion al sym ptom s, in clu ding fever, lym ph - aden opathy, h epatosplen om egaly, epistaxis, an d easy bruisabilit y. Cen t ral n er vou s system (CNS) involvem en t an d secon dar y in fect ion s are th e m ain con cern (Fig. 15.2). Ocular m an ifestat ion s in clude th e follow ing:

Fig . 15.2 Color fundus photograph showing superficial and deep retinal hemorrhages, and a preretinal boat-shaped hem - orrhage associated with leukemia. (Courtesy of Stephen W. Wong,

MD, Philadelphia, PA)

436 Color Atlas of Ophthalm ology

Ret in opathy, in clu ding flam e-sh aped h em orrh ages, Roth spot s, cot ton -w ool spot s, periph eral ret in al n eovascu larizat ion leu kem ic pigm en t epith eliop athy, ch aracterized by a leopard sp ot fun du s

Orbit al involvem en t

Iris th icken ing, irit is, an d pseudohypopyon

Subconjun ct ival h em orrh age, hyph em a

Opt ic n europathy

Management

Bon e m arrow aspirat ion , biopsy w ith im m un ocytogen ic m arkers, com pu ted tom o- grap h ic (CT) scan , an d lu m bar pu n ct u re are required for con firm ing th e diagn osis an d exten t of th e disease. System ic ch em oth erapy, gen eral support ive m easu res, an d ocu lar radioth erapy are th e m ain st ays of th e t reat m en t .

Lymphoma

Lym ph om a is a t ype of solid h em atological n eoplasm origin at ing from th e lym - ph ocytes. Prim ar y in t raocular an d cen t ral n er vous system lym p h om a is a h igh ly m align an t , diffuse, large B-cell lym ph om a (Figs. 15.3 an d 15.4).

Presentation

In t raocu lar lym p h om as gen erally affect elderly pat ien t s an d are u n ilateral (20%) or bilateral (80%). Tw o t ypes are recogn ized: vit reoret in al (n ot associated w ith system ic involvem en t) an d uveal (associated w ith system ic involvem en t).

Ocu lar feat ures

Ch ron ic an terior uveit is

In term ediate uveit is

Fig . 15.3 Color fundus photograph of a patient with intraocular lymphoma showing hemorrhagic retinal

vasculitis. (Courtesy of Debra A. Goldstein, MD, Chicago, IL)

15 Ocular Manifestations of Systemic Disease 437

A B

Fig . 15.4 (A) Color and (B) red-free fundus photographs of a patient with central nervous system lymphoma that resulted in central retinal artery occlusion secondary to

optic nerve compression. (Courtesy of Lawrence J. Ulanski, MD, Chicago, IL)

Posterior segm en t

Mu lt ifocal, large, yellow ish , subret in al pigm en t epith elium (RPE) in filt rates.

Diffuse ret in al in filt rates, vascu lar sh eath ing, an d occlu sion

CNS feat ures

Solit ar y or m u lt iple in t racran ial n odu les

Diffuse m en ingeal or periven t ricular lesion s

Localized in t radu ral spin al m asses

Management

Diagn osis of ocular lym ph om a is con firm ed w ith aqu eou s an d vit reou s biopsy an d cytology. CT, m agn et ic reson an ce im aging (MRI), an d lu m bar p un ct ure h elp in ascer t ain ing th e diagn osis of CNS lym ph om a. Lym ph om a is t reated w ith h igh -dose extern al beam radioth erapy to th e eyes, w h ole-brain radioth erapy, system ic or in - t rath ecal ch em oth erapy, an d in t rath ecal m eth ot rexate.

Pregnancy

Pregn an cy is associated w ith m atern al h orm on al, m et abolic, h em atological, cardiovascu lar, an d im m u n ologic alterat ion s th at can affect th e ocular t issues.

Presentation

Ret in al an d ch oroidal disorders arising in pregn an cy

Preeclam psia an d eclam psia

Ret in opathy—focal or gen eralized ret in al ar teriolar n arrow ing

Ch oroidopathy—serou s ret in al det ach m en t s or yellow RPE lesion s

Cor t ical blin dn ess

Ret in al ar terial an d ven ou s occlusion s

Cen t ral serou s ch orioret in opathy

Dissem in ated in t ravascu lar coagulop athy (DIC)

Th rom bot ic th rom bocytopen ic purpu ra (TTP)

438 Color Atlas of Ophthalm ology

Am n iot ic flu id em bolism

Uveal m elan om a

Worsen ed preexist ing con dit ion s

Diabet ic ret in op athy (progression can be dim in ish ed by bet ter m etabolic con - t rol before pregn an cy)

Diabet ic m acu lar edem a (pregn an t w om en w ith diabet ic m acular edem a sh ould n ot be t reated during pregn an cy becau se of th e h igh rate of spon tan eous im - provem en t post par t um )

Management

Treat m en t is aim ed at th e path ological st ate an d is don e in coordin at ion w ith th e pat ien t’s obstet rician . A close w atch m ust be kept on th e ret in opath ies because spon tan eous regression can occur w ith t im e. Proliferat ive diabet ic ret in opathy m ay be required to be t reated to preven t vit reous h em orrh age during labor.

Albinism

Albin ism is a gen et ically determ in ed h eterogen eou s group of disorders involving hypop igm en tat ion of th e eyes or skin due to a deficien cy of t yrosin ase, w h ich m e- diates th e conversion of t yrosin e to m elan in (Fig. 15.5).

A B

Fig. 15.5 (A) Albinotic patient. (B) Transillumination seen through the iris in the same patient.

15 Ocular Manifestations of Systemic Disease 439

Presentation

Oculocuta neous Albinism

Tyrosinase-negat ive: Th ese albin os are in capable of syn th esizing m elan in an d h ave blon d h air an d ver y pale skin .

Iris is diaph an ou s an d t ran slu cen t , giving rise to a “pin k-eyed” appearan ce.

Fu n dus

Lack of pigm en t w ith con spicu ou sly large ch oroidal vessels

Hypoplasia of vessels form ing th e perim acu lar arcades

Foveal an d opt ic n er ve hypoplasia m ay be presen t .

Refract ive errors are com m on .

Nyst agm us

Th e ch iasm h as a decreased n um ber of u n crossed n er ved fibers.

Tyrosinase-posit ive: Th ese albin os can syn th esize variable am oun ts of m elan in

an d var y in com plexion from ver y fair to alm ost n orm al.

Iris m ay be blu e or dark brow n .

Fu n dus sh ow s variables hypopigm en t at ion .

Visual acuit y is u su ally im paired ow ing to foveal hypoplasia.

Associated syndrom es: Ch édiak-Higash i an d Herm an sky-Pu dlak syn drom es can be associated w ith oculocut an eou s albin ism .

Ocula r Albinism

Th e eyes are predom in an tly affected, w ith less eviden t skin an d h air involvem en t . In h eritan ce is XL or less com m on ly AR. Fem ale carriers are asym ptom at ic an d h ave n orm al vision , alth ough th ey m ay sh ow part ial iris t ran slu cen cy, m acu lar st ip - pling, an d scat tered areas of depigm en tat ion an d gran u larit y in th e m idperip h er y. Affected m ales m an ifest hypopigm en ted iris an d fun du s.

Management

Tyrosin ase test , elect roret in ography, h em atological tests are h elpfu l in th e diagn o- sis. Dark glasses, low vision aids, gen et ic coun seling are h elpfu l.

440 Color Atlas of Ophthalm ology

Marfan Syndrome

Marfan syn drom e is a w idespread disorder of con n ect ive t issu es associated w ith m u tat ion of th e fibrillin gen e or ch rom osom e 15q. It is th e m ost com m on cause of h eritable ectopia len t is. Marfan syn drom e is au tosom al dom in an t , an d th e prevalen ce is 5/100,000 (Fig. 15.6).

Presentation

Oph th alm ic feat ures

Ectopia len t is, hypoplasia of dilator pu pillae, angle an om aly, m yopia, an d ret in al detach m en t

Len s dislocat ion occurs in ~80% of pat ien ts w ith Marfan syn drom e an d is usually bilateral, sym m et rical, an d su perotem poral.

Microsph eroph akia, keratocon u s, an d corn ea plan a

Muscu loskeletal feat u res

Tall, th in st at u re, scoliosis, in creased arm span

Arach n odact yly, m ild join t hyperm obilit y

Narrow an d h igh -arch ed palate

Cardiovascu lar feat ures

Dilat at ion of th e ascen ding aor ta leading to aor t ic in com peten ce an d h ear t failu re

Mit ral valve disease an d aor t ic dissect ion

Skin

St riae, fragilit y, an d easy bruising

Fig . 15.6 Long, thin, and slender fingers in a patient with Marfan syndrom e.