Ординатура / Офтальмология / Учебные материалы / Color Atlas of Ophthalmology The Quick-Reference Manual for Diagnosis and Treatment
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5 Cornea 191
Management
Th e bioprosth et ic graft con sists of a biological an d a prosth et ic p ar t . Th e biological par t is fash ion ed from a cadaveric w h ole globe (corn ea an d sclera), an d th e prosth et ic par t con sists of an an iridia IOL (iris, pu pil, an d IOL). Tw o par t ial-th ickn ess, lim bal-based scleral flaps of 3-m m size are created 180 degrees apart on th e don or sclera. Tw o st raigh t sclerotom ies are th en m ade w ith an 18-gauge n eedle 1.5 m m from th e lim bu s un der th e exist ing scleral flaps. A corn eo-scleral rim is th en cu t th e en t ire length of th e sclera, in clu ding in th e scleral flaps. A cyclodialysis is th en in duced to separate th e uveal t issue from th e dissected corn eoscleral but ton . Th e corn eoscleral graft is placed con cave (i.e., en doth elial side up ), an d th e an iridia IOL h apt ics are extern alized th rough th e sclerotom y un der th e scleral flaps u sing a 23-gauge forceps. Th e biosyn th et ic assem bly th u s con sist s of a don or corn ea an d sclera an d an ar t ificial iris an d len s (an iridia IOL). In th e recipien t eye, th e staphylom atous corn ea is excised . Un derlying cat aract is m an aged w ith len sectom y an d vit rectom y. Th e biosyn th et ic graft is th en placed on th e h ost an d sut u red . Th e IOL h apt ics are th en t ucked in to a scleral p ocket created at th e edge of th e scleral flaps, an d th e flap is th en glu ed dow n w ith t issu e glu e (Tisseel, Baxter, Deerfield, IL). Th e conju n ct iva is th en closed by gluing.
Enlarged Corneal Nerves
Presentation
Prom in en t corn eal n er ves m ay be seen radiat ing cen t rally from th e corn eal periph er y.
Differential Diagnosis
Mu lt ip le en docrin e n eop lasia t ype IIb, icthyosis, Refsu m disease, leprosy, keratocon us, Fuch s en doth elial dyst rophy, osteogen esis im p erfect a, ocu lar pem ph igu s, n eurofibrom atosis, ph th isis bu lbi, posterior polym orp h ou s dyst rophy, h erpes sim - plex, h erp es zoster, p rim ar y am yloidosis
Management
Man agem en t is aim ed at th e un derlying con dit ion .
Corneal Neovascularization
Th e corn ea is t yp ically devoid of blood vessels; h ow ever, in con dit ion s of in fect ion , in flam m at ion , or ocu lar surface in sult , blood vessels can abn orm ally exten d in to th e corn ea. Th is is often referred to as corn eal n eovascularizat ion or pan n us. It is m ain ly seen in in flam m ator y corn eal diseases such as t rach om a.
Presentation
Pan n u s presen t s as a vascu lar ingrow th in to th e corn ea from th e lim bal vascu la- t u re. It can be superficial or deep (Fig. 5.44).
192 Color Atlas of Ophthalm ology
Fig . 5.44 A slit-lamp photograph demonstrating severe corneal neovascularization from diffuse stem cell deficiency.
Differential Diagnosis
Trach om a, leprosy, h erpes sim plex or h erpes zoster kerat it is, ph lycten u lar keratoconju n ct ivit is, toxic conjun ct ivit is, acn e rosacea, bu llou s keratopathy, m ollu scum con tagiosum , vern al conjun ct ivit is, keratoconju n ct ivit is sicca, con tact len s u se, in - clusion conju n ct ivit is (m icrop an n u s), su perior lim bic keratoconjun ct ivit is, con tact len s kerat it is, Fu ch s m argin al kerat it is, hypoparathyroidism , vitam in B deficien cy, an d pellagra.
Management
Man agem en t is aim ed at th e un derlying con dit ion .
Leukocornea
Leu kocorn ea m ay arise due to a w ide variet y of con dit ion s in clu ding in fect ion , in flam m at ion , an om alies, h eredit ar y con dit ion s, an d t rau m a.
Presentation
Opacificat ion of th e corn ea is eviden t , even w ith out th e slit-lam p exam in at ion .
Differential Diagnosis
Infect ions: Bacterial, fu ngal, h erpet ic u lcers, st rom al scarring, t rach om a, Acan - tham oeba
Inf lam m atory: aph akic an d pseudoph akic bullous keratopathy, Steven Joh n son s syn drom e, graft reject ion , ocular cicat ricial pem ph igoid
Congenital: An terior ch am ber cleavage syn drom es, sclerocorn ea, congen it al glaucom a, derm oid, am yloidosis
Hereditary: Congen it al h eredit ar y corn eal dyst rophy, Dow n syn drom e, Patau syn drom e, in born errors of m etabolism (m u copolysacch aridosis, Low e syn - drom e, m ucolipoidosis)
Traum a: Descem et tear (bir th t rau m a), th erm al inju r y, ch em ical burn s
5 Cornea 193
Management
Man agem en t con sist s m ain ly of doing a corn eal t ran sp lan tat ion in su itable cases. Any un derlying cause sh ould also be addressed .
Chemical Burn
See th e ch apter 1 sect ion on ch em ical exposu re bu rn s.
6 Intraocular Inflammation
Soosan Jacob, Dhivya Ashok Kum ar, Athiya Agarw al, and Am ar Agarw al
Acute Anterior Nongranulomatous Uveitis
HLA-B27-Associated Uveitis
Ankylosing Spondylitis
An kylosing spon dylit is (AS) is an ar th ropathy ch aracterized by back pain an d st iff- n ess after in act ivit y. HLA-B27 is fou n d in u p to 90%of pat ien ts w ith AS. Th e ch an ce th at an HLA-B27–posit ive pat ien t w ill develop spon dyloar th rit is or eye disease is 1 in 4. Not all HLA-B27–posit ive pat ien ts develop disease.
Sacroiliac x-ray film s sh ould be obt ain ed w h en in dicated by a suggest ive h istor y in a p at ien t w ith ocular disease con sisten t w ith HLA-B27 syn drom e. Sacroiliac x- ray film s sh ow sclerosis an d even t ual n arrow ing of th e join t space, ligam en tou s ossificat ion .
Complications
Bony deform it y, pu lm on ar y apical fibrosis, aort it is, aor t ic valvular in su fficien cy
Uveitis
Management
Topical steroids an d cycloplegics. Sacroiliac join t radiograph , HLA-B27 screen ing rh eu m atology con su ltat ion
Reiter Syndrome
Reiter syn drom e is ch aracterized by th e follow ing:
Non specific u reth rit is
Polyar th rit is
Conju n ct ival in flam m at ion , often accom pan ied by irit is
Presentation
Th is is com m on ly seen in young adult m ales (90%), w h ereas fem ales con st it u te on ly 10%. Arth rit is is t ypically asym m et ric an d in oligoart icular dist ribu t ion , in - volving kn ees, an kles, feet , an d w rist s. Sacroiliit is is presen t in as m any as 70%of pat ien t s.
Keratoderm a blen n orrh agicum (a scaly, er yth em atous disorder of th e palm s an d soles) an d circin ate balan it is (a persisten t , scaly, er yth em atous, circu m feren t ial rash of th e dist al pen is) m ay be foun d .
Less com m on fin dings are plan t ar fasciit is, Ach illes ten din it is, sacroiliit is, n ailbed pit t ing, p alate u lcers, an d tongu e ulcers.
Ocu lar involvem en t ranges from conju n ct ivit is (m u copur ulen t an d papillar y) to kerat it is (pu n ct ate an d subep ith elial) to an terior, n ongran ulom atou s in flam m a- t ion .
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6 Intraocular In
am m ation 195
Uveitis
Management
Topical steroids an d cycloplegics
HLA-B27 screen ing becau se it is presen t in 85 to 95%of pat ien t s
Rh eu m atology con sult at ion
Inflammatory Bowel Disease (IBD)
Ulcerat ive colit is (diffu se in flam m at ion of th e colon ic m u cosa) an d Croh n disease (gran ulom atous iliocolit is) are both associated w ith acute irit is.
Presentation
Bet w een 5 an d 12% of pat ien ts w ith u lcerat ive colit is, an d 2.4% of pat ien ts w ith Croh n disease develop acu te an terior uveit is (AAU). Of pat ien t s w ith in flam m a- tor y bow el disease, 20%m ay h ave sacroiliit is, an d of th ese pat ien t s, 60%are HLAB27 posit ive. Ocular involvem en t m ay occu r first an d m ay in clu de an terior uveit is, conju n ct ivit is, kerat it is, ep isclerit is, sclerit is, ext raocu lar m u scle palsies, opt ic n europathy, ret in al vascu lit is, n eu roret in it is, an d orbital in flam m at ion . System ic sym ptom s in clu de bloody diarrh ea, cram py abdom in al pain , skin rash , ar th ralgia, er yth em a n odosu m , pyoderm a gangren osum , sacroiliit is, ren al ston es, an d h epatobiliar y abn orm alit ies.
Uveitis
Management
Topical steroids an d cycloplegics. Treat in conjun ct ion w ith an in tern ist .
Psoriatic Arthritis
Ocular involvem ent: Nongran u lom atou s, an terior in flam m at ion , n odular episclerit is, kerat it is, keratoconjun ct ivit is sicca
Uveit is is n ot associated w ith psoriasis w ith ou t arth rit is.
System ic: Er yth em atous, hyperkeratot ic rash , n ail pit t ing, an d distal in terp h a- langeal join t ar th rit is
Uveitis
Management
Topical steroids, cycloplegics, im m un osup pressives. Treat in conju n ct ion w ith an in tern ist .
196 Color Atlas of Ophthalm ology
Glaucoma-Related Uveitis
Posner-Schlossman Syndrome
Presentation
Man ifests as u n ilateral m ild acu te irit is sym ptom s th at in clu de discom for t , blurred vision , or h aloes. Sign s in clu de m arkedly elevated in t raocular pressure, corn eal edem a, fin e keriat ic p recipit ates, low -grade, an d sligh tly dilated pu pil.
Posn er-Sch lossm an syn drom e m ay be associated w ith HLA-B54 gen e locus.
Management
Topical steroids an d in t raocular p ressure–low ering agen ts
Uveitis-Glaucoma-Hyphema Syndrome
Irritat ion of th e iris root by th e w arped foot plates of poorly m ade, rigid, an terior ch am ber in t raocular len s im plan t s cau ses th e uveit is-glaucom a-hyp h em a t riad .
Presentation
In creased in t raocular pressu re, an terior ch am ber in flam m at ion , an d hyph em a for- m at ion in th e presen ce of an in t raocular len s
Management
Topical steroids, cycloplegics, an d in t raocu lar pressure–low ering agen t s
Phacolytic Uveitis/Glaucoma
Th is involves an acu te in crease in in t raocu lar pressure cau sed by blockage of th e t rabecu lar m esh w ork by len s protein an d engorged m acroph ages.
Presentation
Low -grade anterior cham ber inflam m ation, increased intraocular pressure, lack of keratic precipitates, and synechiae. Aqueous tap m ay reveal swollen m acrophages.
Management
In t raocu lar pressu re redu ct ion w ith osm ot ic agen t s as w ell as topical m edicat ion s. Th e cat aract n eeds to be rem oved .
Chronic Nongranulomatous Uveitis
Juvenile Rheumatoid Arthritis–Associated Uveitis
Juven ile rh eum atoid ar th rit is (JRA) is a grou p of diseases w ith on set before 16 years of age.
6 Intraocular In
am m ation 197
Presentation
System ic onset: Usu ally seen in ch ildren u n der th e age of 5 years, of w h ich < 6%
presen t w ith uveit is; rash , fever, lym ph aden opathy, h epatosplen om egaly, pericardit is, an em ia, psoriasis; pat ien t s presen t ing w ith system ic on set accou n t for ~20%of all cases of JRA.
Polyart icular onset: Sh ow s involvem en t of five or m ore join ts in th e first 6 w eeks of th e disease. It con st it utes 40%of JRA cases overall bu t on ly 7 to 14%of cases of JRA-associated iridocyclit is.
Panciart icular onset: Th is in cludes th e vast m ajorit y (80 to 90%) of pat ien ts w ith JRA w h o h ave uveit is.
Type 1: Girls un der age 5, posit ive for an t in u clear an t ibody (ANA). Ch ron ic iridocyclit is occurs in up to 25%of th ese pat ien t s.
Type 2: Older boys, seronegative spondyloarthropathy (75%are HLA-B27 posi-
tive). Uveitis tends to be acute and recurrent rather than chronic as in t ype 1.
Ocular involvem ent: It usu ally occu rs w ith in 5 to 7 years of on set of arth rit is, but th e risk rem ain s in to adulth ood . Ocular in flam m at ion occu rs in 2 to 12%of all cases an d is u sually bilateral. Un ilateral cases often progress to bilateral w ith in
12 m on th s. Th e ocu lar an d join t disease act ivit y are n ot associated . It m ay be an t in u clear an t ibody test posit ive, rh eum atoid factor n egat ive.
Ch ildren are often asym ptom at ic w ith in sidious disease on set . Th ere m ay be m ild ocular pain , h eadach e, ph otoph obia, an d decreased vision . A w h ite eye w ith act ive an terior ch am ber in flam m at ion , kerat ic precipit ates, posterior syn ech ia, cataract form at ion , glau com a, an d ban d sh aped keratopathy m ay even be fou n d on th e first exam in at ion .
Management
Uveitis is treated w ith topical steroids and cycloplegics. System ic or periocular steroids are som etim es needed. Ethylenediam inetetraacetic acid (EDTA) chelation is done for band-shaped keratopathy. Rheum atology consultation is often necessar y.
Fuchs Heterochromic Iridocyclitis
Presentation
Presen t at ion is u sually u n ilateral; sym ptom s var y from n on e to m ild blu rring an d discom fort .
Signs
Diffuse iris st rom al at rophy w ith variable pigm en t epith elial layer at rophy
Sm all w h ite stellate kerat ic precip itates scat tered diffu sely over th e en t ire en -
doth elium
Cells presen t in th e an terior ch am ber as w ell as th e an terior vit reou s.
Syn ech iae alm ost n ever form .
Glaucom a an d cat aract s occu r.
Abn orm al vessels m ay bridge th e angle on gon ioscopy.
Fu n dus lesion s are absen t; som et im es toxoplasm a scars h ave been reported .
Management
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Cat aract su rger y an d in t raocular len s im plan t can be don e successfu lly. |
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Glaucom a is difficult to con t rol an d m ay n eed surger y. |
198 Color Atlas of Ophthalm ology
Granulomatous Uveitis
Syphilis
Presentation
Syph ilit ic involvem en t of th e uveal t ract can presen t in t w o w ays:
1. Congenital syphilit ic uveit is: Here th e involvem en t is bilateral, th e periph er y of th e ret in a is involved, an d occasion ally on e or t w o qu adran ts of th e posterior pole can be involved rarely. Th us th e vision is n ot affected . Th e t yp ical appearan ce is th at of a salt an d pepper fun du s du e to areas of p igm en t accum ulat ion in terspread w ith areas of pigm en t loss. An associated ocu lar feat ure is th e presen ce of bilateral in terst it ial kerat it is.
Secondary ret inal pigm ent degenerat ion m ay be seen , w h ich is a con dit ion th at is progressive an d is associated w ith con st rict ion of th e blood vessels of th e ret in a an d th e ch oroids in th e form of sclerosis. Th e opt ic disk is pale w ith sh arp ly defin ed borders. Pigm en t s dispersed are also sh arply dem arcated w ith a st ar sh ape or bony corpu scle form at ion . Th e posterior pole or th e periph er y can be affected, an d th e con dit ion is bilateral.
2. Acquired syphilis: Th is h as th ree com pon en t s: irit is, ch orioret in it is, an d n eu - roret in it is. Th e irit is is ch aracterized by th ree form s: irit is papulosa, irit is n o- dosa, an d irit is roseat a. Ch orioret in it is is ch aracterized by vit reou s h aze, fin e pun ct ate gray to yellow exu dat ion areas, pigm en t accum ulat ion along th e opt ic n er ve an d blood vessels, an d flam e-sh aped h em orrh ages w ith ch orioret in al edem a. Neu roret in it is con sists of opt ic n er ve h ead involvem en t w ith vascular involvem en t of th e su rrou n ding ret in al vessels.
Management
Blood tests in th e form of Ven ereal Disease Research Laborator y test (VDRL) an d th e fluorescen t t repon em al an t ibody–absorpt ion test (FTA-ABS) are don e. Uveit is is t reated w ith topical steroids an d cycloplegics. Th e system ic disease sh ou ld be t reated in conju n ct ion w ith an in tern ist .
Sarcoidosis
Sarcoidosis is a ch ron ic gran u lom atous uveit is of u n kn ow n et iology. It m ay also affect th e lu ngs, eyes, an d skin .
Presentation
Ocular: Sym ptom s of uveal involvem en t are variable an d frequ en tly in clude m ild
to m oderate blurring of vision . It m ay involve all st ru ct ures of th e eye. A sizable propor t ion of pat ien t s develop ch ron ic gran ulom atou s iridocyclit is. Typical fin d - ings are m u t ton fat kerat ic precipitates, Koeppe an d Bu sacca iris n odules, an d sn ow balls in th e in fect iou s an terior vit reous. Nu m m u lar corn eal in filt rates, en - doth elial op acificat ion , an d large iris gran ulom as also occu r. Posterior syn ech iae can be exten sive an d m ay lead to iris bom bé an d angle closure glaucom a. Periph eral an terior syn ech ia m ay be exten sive, involving 360 degrees in advan ced cases. Secon dar y glaucom a can be severe.
6 Intraocular In
am m ation 199
Posterior segm ent involvem ent is characterized by nodular granulom as m easuring ¼to 1 disc diam eter that occur in both the retina and the choroid. Irregular nodular granulom as along venules have been term ed candlew ax drippings or taches de bougie. Linear or patchy retinal periphlebitis presents as sheathing. Cystoid m acular edem a is com m on; retinal neovascularization, disk edem a, and optic nerve granulom as also occur. Palpebral and bulbar conjunctival nodules also occur.
Lungs: Hilar aden opathy.
Central nervous system (CNS): Cran ial an d periph eral n europathy, asept ic m en - ingit is.
Cardiovascular system : Cardiac arrhyth m ias, pericardit is, ar th rit is, m yosit is, er yth em a n odosa, ren al involvem en t h epatosplen om egaly an d bon e m arrow in filt rat ion .
Management
Serum angioten sin -conver t ing en zym e an d lysozym e levels are in creased . Ch est x-ray sh ow s h ilar aden opathy.
Uveitis
Topical, periocu lar, an d system ic steroids, topical cycloplegics. Treat in conju n ct ion w ith an in tern ist .
Tuberculosis
Presentation
Tu bercu lou s uveit is can presen t in clu de th e follow ing w ays:
Acute n ongran ulom atous in fect ion —im m u n ological or allergic in n at ure
Nodu lar gran u lom atous in filt rates th at are fulm in at ing an d caseat ing. Tw o t ypes of n odu les are presen t as sh ow n in Table 6.1.
Relapsing an d recurren t irit is
Low -grade ch ron ic in flam m at ion th at presen ts w ith cataract , glaucom a, or pth isis bu lbi
Ch oroidal lesion s appear as raised m ult iple yellow n odules w ith blurred m argin s. Ret in al periph lebit is an d su bret in al vascu larizat ion s are seen .
Oth er ocu lar feat ures of t uberculosis in clu de ph lycten u losis, episclerit is, n odu - lar sclerit is, an d opt ic n eu rit is.
Management
An t it uberculous t reat m en t is started in conjun ct ion w ith an in tern ist . Steroids sh ould be started on ly after star t ing an t it u bercu lou s t reat m en t .
Table 6.1 Tw o Types of Nodular Granulom |
ltrates |
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Solitary or co nglom erate nodule |
Miliary no dules |
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Otherwise healthy immune |
Im munocompromised patients, |
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responsive patients |
severely debilitated patients |
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Single large tum orlike nodule |
Multiple nodules over the iris at the |
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yellow to white |
papillary margin and ciliary body |
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with dissemination of bacilli |
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200 Color Atlas of Ophthalm ology
Intermediate Uveitis/Pars Planitis
Presentation
The disease is t ypically seen in children . It is also know n as chronic cyclitis. Patients have visual sym ptom s like blurred, distorted vision secondar y to m acular edem a. They also com plain of floaters. The active vitreous inflam m ation show s cells that are sm all, round, nonpigm ented, and num erous. The old vitritis has cells that are irregu - lar, pigm ented, and rem ain in the form ed vitreous. At the edges of the ora serrata there is snow banking that is characteristic of deposition of inflam m atory cells at this region . Anterior segm ent inflam m ation signs such as congestion and tenderness are absent . The sym ptom s of pain or photophobia are also absent or m inim al.
Th ere are several key differen t iat ing feat u res:
Periph eral ret in it is
Perivasculit is
Vit rit is
Sn ow ban king at th e in ferior periph eral ret in a
Differential Diagnosis
Mu lt iple sclerosis, sarcoidosis, t u bercu losis, toxocariasis, syph ilis, Lym e disease
Management
Steroids are given via topical, su bten on s, or oral route. System ic im m u n osup pressan t s an d vit rectom y m ay also be n ecessar y in som e cases.
Posterior Uveitis
Toxoplasmosis
Toxoplasm osis is th e m ost com m on cau se of ch orioret in it is, congen it al in origin . W h en th e m oth er is affected in th e first t rim ester, th ere is a 40%risk of t ran sm ission to th e fet u s.
Presentation
Tw o form s of toxoplasm osis exist: congen ital an d adult . Both form s are ch aracterized by acu te focal ch orioret in it is (Fig. 6.1).
Congenital toxoplasm osis: It is bilateral in 85% of ch ildren an d 80% h ave ch o- rioret in it is. W h en CNS sym ptom s su ch as convulsion s, hydrocep h alus, m en tal retardat ion , an d in t racran ial calcificat ion s are absen t , congen ital lesion s are diagn osed w h en th e ch ild p resen t s w ith esot ropia or exot ropia or decreased vision .
Th e clin ical ch aracterist ics of th e congen it al form are as follow s:
Bilateral
Mu lt ip le ch orioret in al lesion s, especially in th e m acular area
Pu n ch ed -ou t lesion du e to fu ll-th ickn ess n ecrosis
Heavily p igm en ted scar, m ist aken to be congen ital colobom as