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

5 Cornea 151

A

B

Fig. 5.7 (A) Interstitial keratitis from congenital syphilis with ghost vessels on retroillum ination. (B) Herpes simplex virus interstitial keratitis and corneal scarring.

Management

Treat m en t begin s w ith iden t ificat ion of th e cau se. Serological test ing is ver y im - por tan t in m aking a diagn osis. A screen ing test such as a Rapid Plasm a Reagin (RPR) or a t repon em e-specific test su ch as Flu orescen t Tropon em al An t ibody (FTA-ABS) or m icroh em agglut in at ion assay (MHA-TP) can detect syph ilis. System ic t reat m en t is in dicated depen ding on th e u n derlying cau se (e.g., system ic an t ibiot ics w ith syph ilis, t u bercu losis, Lym e disease). In flam m at ion in in terst it ial kerat it is cases is con t rolled w ith topical cor t icosteroids an d cycloplegic agen t s on ce an in fect ious cau se is elicited . See th e sect ion on HSV an d HZV for t reat m en t .

152 Color Atlas of Ophthalm ology

Fig . 5.8 Punctate keratitis and multiple corneal opacities t ypical of Thygeson superficial punctate keratitis.

Thygeson Superficial Punctate Keratitis

Th is n on specific su perficial pun ct ate kerat it is t ypically occurs in you ng ch ildren to older adu lts. Th e cau se is u n kn ow n , alth ough specu lat ion exists as to w h eth er a virus is th e u n derlying agen t . Mult iple corn eal lesion s develop bu t t ypically respon d rapidly to cor t icosteroids, suggest ing an im m un ological basis for kerat it is.

Presentation

Sym ptom s in clude recu rren t episodes of tearing, ph otoph obia, foreign body sen sa- t ion , an d m ild blurred vision . Conju n ct ival inject ion is usually m in im al or absen t . Corn eal fin dings in clu de m u lt iple raised epith elial lesion s th at are gray to w h ite gran ular opacit ies th at w a x an d w an e in locat ion an d n u m ber an d are u sually bilateral w ith poten t ial asym m et r y (Fig. 5.8).

Differential Diagnosis

Subepith elial in filt rates w ith epidem ic keratoconju n ct ivit is (EKC), EBV, staphylococcal hypersen sit ivit y, con tact len s over w ear kerat it is

Management

Su ppor t ive t reat m en t w ith topical lubrican t tears or gels w ith or w ith out ban dage con tact len s w ear can be u sefu l in m ilder cases. Low -dose topical cort icosteroids can prom ote rapid resolut ion of corn eal lesion s; h ow ever, lesion s t ypically recur w ith discon t in uat ion of drops. Long-term cort icosteroid t reat m en t w ith flu oro- m eth olon e 0.1%or lotepredn ol 0.5%can w ork w ell at var ying dose regim en s w ith slow ly tapering doses from an in it ial dosing of fou r t im es a day. Topical cyclosp o- rin e an d top ical t rifluridin e h ave also been u sed w ith an ecdot al success. Th e lesion s even t u ally resolve, bu t th is m ay take m on th s to years for perm an en t resolu - t ion of recu rren t episodes.

Shield Ulcer

Sh ield ulcer is a n on in fect ious corn eal u lcer in th e set t ing of vern al keratoconju n c- t ivit is th at is often bilateral an d season al (m ore com m on in spring). A h istor y of atopy is com m on . Th e con dit ion m ore often presen ts in m ales.

5 Cornea 153

Fig . 5.9 Peripheral vernal shield ulcers seen in vernal keratoconjunctivitis.

Presentation

Presen tat ion in cludes itch ing, ph otoph obia, foreign body sen sat ion , blu rred vision , redn ess, an d m ucou s disch arge. Sh ield ulcer can be associated w ith gian t papillar y conju n ct ivit is an d lim bal follicles kn ow n as Horn er-Tran t as dots (collect ion s of degen erated eosin op h ils an d epith elial cells). A su perior pu n ctate keratopathy t yp i- cally develops follow ed by a breakdow n of epith elium an d even t ual oval-sh aped ulcers (sh ield ulcers) w ith u n derlying st rom al opacificat ion . Associat ion can occur w ith keratocon u s an d floppy eyelid syn drom e (Fig. 5.9).

Differential Diagnosis

Atopic keratoconju n ct ivit is, in fect ious u lcer, im m u n e-m ediated u lcer

Management

Pat ien t s sh ou ld avoid p oten t ial allergen s an d u se cool com p resses, p reser va- t ive-free lu br ican t s, top ical an d /or system ic an t ih ist am in e or m ast cell st abilizers, an d top ical or system ic cor t icosteroid s. Alter n at ive top ical or system ic im - m u n osu p p ressan t agen t s su ch as cyclosp or in e A m ay p rovid e ben efit w it h close follow -u p .

Exposure Keratopathy

Exposu re keratopathy is also referred to as n europaralyt ic keratopathy. Causes in - clude facial n er ve palsy, severe proptosis, an d scarring of th e lids. Th e con dit ion m ay be associated w ith w eak Bell p h en om en on . It is th e resu lt of im proper w et t ing of th e ocu lar su rface by th e tear film .

154 Color Atlas of Ophthalm ology

Presentation

Sym ptom s range from m ild in ferior pu n ctate epith elial ch anges to severe corn eal m elt ing w ith corn eal perforat ion .

Differential Diagnosis

Neu rot roph ic keratopathy, recu rren t erosion s, in fect iou s keratopathy, dr y eye, au - toim m un e diseases

Management

Frequen t in st illat ion s of lubrican t s an d t aping th e lids form th e m ain stay of th e t reat m en t . Lid surgeries to correct th e lid abn orm alit ies m ay be required .

Filamentary Keratitis

An ocu lar surface in flam m ator y con dit ion ch aracterized by m ucou s plaques an d st ran ds of th e corn eal surface. Filam en ts represen t st ran ds of epith elial cells attach ed to th e corn eal su rface over a core of m u cus w ith firm at t ach m en t to th e adjacen t corn eal n er ves.

Com m on con dit ion s associated w ith th is fin ding in clude eyelid m alposit ion su ch as lagop h th alm ia an d poor eyelid blin k, con tact len s over w ear kerat it is, atop ic con - dit ion s, an d ch ron ic ocu lar surface in flam m ator y con dit ion s such as su perior lim - bic keratoconju n ct ivit is, aqueou s tear deficien cy, an d dr y-eye syn drom e.

Presentation

Pat ien ts experien ce pain , foreign body sen sat ion , redn ess, dr yn ess, an d ph otop h o- bia. Mu cous plaqu es in st ran ds or globules are visu alized en t rapped w ith in th e corn eal epith elium . Th e filam en t s st ain w ith fluorescein dye. Conju n ct ivoch alasis, pun ct ate corn eal st ain ing bet w een filam en ts, an d a low tear m en iscus are com - m on associated fin dings (Fig. 5.10).

Fig. 5.10 Filamentary keratitis.

156 Color Atlas of Ophthalm ology

Differential Diagnosis

Corn eal abrasion , recu rren t erosion , in fect ious u lcer, dr y-eye syn drom e, an d au - toim m un e disease

Management

Identify the underlying cause. Lubricant drops, gels, and ointm ents can protect the corneal surface from further breakdow n. Temporary or perm anent punctal closure can also benefit. A tarsorrhaphy is often needed to decrease the am ount of exposed surface area to the cornea. Therapeutic bandage lens wear should be used cautiously w ith close follow -up. A conjunctival or am niotic m em brane graft can be used in severe situations, w ith lam ellar or penetrating keratoplasty used in cases of severe corneal m elting or perforation. Autologous serum drops can also be a useful adjunct to treatm ent.

Recurrent Erosion

Recurren t corn eal erosion (RCE) syn drom e is ch aracterized by a dist urban ce at th e level of th e corn eal epith elial basem en t m em bran e, resu lt ing in defect ive adh e- sion s an d recu rren t breakdow n s of th e epith elium . Com m on cau ses of recu rren t erosion s in clude epith elial basem en t m em bran e dyst rophy, corn eal inju ries, alkali burn s, foreign bodies, post in fect iou s ulcers from h erp es sim plex, Cockayn e syn - drom e, Reis-Bü cklers dyst rophy, p ost vit rectom y, ph otocoagu lat ion , an d con tact len ses, am ong oth ers.

Presentation

Many pat ien t s (80 to 90%) are asym ptom at ic. Som e can presen t w ith pain , blurred vision , ast igm at ism , epith elial blebs, an d foreign body sen sat ion w ith recu rren t erosion . Pat ien t s can presen t w ith a variet y of sign s such as epith elial loss, epith elial m icrocyst s, bu llae, lack of adh eren ce of sh eet s of epith elium , epith elial filam en t form at ion , corn eal abrasion , brow n ish gran u lar edem a (braw ny edem a), an d areas of h ealed epith eliu m , w h ich m ay even resem ble a den drit ic figu re, a pseu doden drite.

Differential Diagnosis

Corn eal abrasion , corn eal dyst rophy, corn eal foreign bodies, dr y eye, an d in fect ive kerat it is

Management

Man agem en t of RCE syn drom e is u su ally aim ed at regen erat ing or repairing th e epith elial basem en t m em bran e to restore th e adh esion bet w een th e epith elium an d th e an terior st rom a. Recurren t corn eal erosion s respon d to top ical lu bricat ion th erapy, ban dage soft con tact len ses, debridem en t , an t ibiot ic oin t m en t , an d patch - ing. Su rgical opt ion s in clu de an terior st rom al pu n ct ure, excim er laser diam on d burr keratectom y, n eodym iu m :yt t riu m -alu m in u m -garn et (Nd:yAG) laser t reat- m en t , an d su perficial ph ototh erapeut ic keratectom y.

5 Cornea 157

Congenital Anomalies

Microcornea

Th e n orm al corn eal diam eter is n orm ally 9.5–10m m at bir th an d 10–12.5m m at adulth ood .

Presentation

Th e term m icrocorn ea is given to an adu lt corn ea w ith less th an 10m m diam eter. Most cases occur sporadically. Microcorn ea m ay occu r in isolat ion or par t of a gen - erally sm all eye (m icroph th alm os). If it occurs as an isolated en t it y, th e n orm al len s size cau ses a disparit y w ith th e sm all corn ea resu lt ing in angle-closu re glau com a.

Differential Diagnosis

Nan oph th alm os, m icrop h th alm os, sclerocorn ea

Management

Treat m en t is directed at any associated ocular abn orm alit ies

Megalocornea

Presentation

Megalocornea is the term given to the condition in w hich the corneal diam eter exceeds 11m m at birth, or 12m m after 2 years of age. It has been associated w ith ectopia lentis, iris transillum ination, pigm ent dispersion, arcus and m ental retardation .

Differential Diagnosis

Buph th alm os, an terior ch am ber dysgen esis syn drom es. As com pared to buph th al- m os, th e size of th e globe an d th e in t raocular pressu re are n orm al.

Management

Treat m en t is directed at any associated ocular abn orm alit ies

Anterior Embryotoxon

It is a congen ital an om aly of th e corn ea w h ich is n ot visually sign ifican t .

Presentation

An terior em br yotoxon refers to a congen it al broad superior lim bu s w ith an oth er- w ise n orm al an terior ch am ber. It is also u sed to describe a congen it al arcu s, arcu s juven ilis, sim ilar to th e arcus sen ilis th at occu rs in old in dividu als.

Differential Diagnosis

Posterior em br yotoxon , Arcus sen ilis, Axen feld Reiger syn drom e.

Management

Obser vat ion an d follow -u p .

158 Color Atlas of Ophthalm ology

Posterior Embryotoxon

It represents a thickened and anteriorly displaced Schwalbe’s line that is easily visible.

Presentation

Posterior em br yotoxon is th e m ost com m on an om aly of th e corn ea. It m ay be seen as a w h ite ring n ear th e lim bus on slit-lam p exam in at ion an d as a prom in en t , an - teriorly displaced Sch w albe’s lin e on gon ioscopy.

Differential Diagnosis

An terior em br yotoxon , Arcus sen ilis, Axen feld Reiger syn drom e.

Management

Obser vat ion an d follow -u p .

Sclerocornea

It is a congen ital an om aly of th e corn ea resu lt ing in decreased vision .

Presentation

Sclerocorn ea refers to a sclera-like appearan ce of th e corn ea, w h ich m ay be p e- riph eral or involve th e w h ole corn ea. It is associated w ith corn eal flat ten ing an d oth er an om alies of an terior ch am ber developm en t .

Differential Diagnosis

Microph th alm os, bu ph th alm os, an d oth er cau ses of opaque corn ea.

Management

Visual progn osis in severe cases is ver y p oor.

Dystrophies

Anterior Corneal Dystrophies

Epithelial Basement Membrane Dystrophy (EBMD)

Also kn ow n as Cogan m icrocyst ic edem a or m ap -dot-fingerprin t corn eal dyst rophy, th is is perh ap s th e m ost com m on of all corn eal dyst roph ies. Bilateral (bu t m ay be asym m et rical) w ith autosom al dom in an t in h erit an ce, bu t m ost cases are sporadic. Th e con dit ion resu lts from abn orm al epith elial t urn over an d redun dan t basem en t m em bran e.

Presentation

Pat ien ts m ay rem ain asym ptom at ic or m ay develop decreased vision , foreign body sen sat ion , ph otop h obia, tearing, an d poten t ially pain fu l corn eal erosion s. Slit-lam p exam in at ion of EBMD sh ow s gray-w h ite su bepith elial patch es (m aps), gray-w h ite

5 Cornea 159

A

B

Fig . 5.12 (A) Fingerprint pat tern seen with epithelial base - ment m em brane dystrophy (EBMD). (B) Map pat tern seen with EBMD.

parallel lin es (fingerprin ts), or su bepith elial m icrocysts (dots). Th e fin dings are best seen on ret roillu m in at ion (Fig. 5.12A,B).

Differential Diagnosis

Trau m at ic corn eal erosion , Meesm an n dyst rophy, early Reis-Bü cklers dyst rophy, corn eal in t raepith elial n eop lasia (CIN)

Management

No t reat m en t is required for asym ptom at ic cases. For sym ptom at ic cases, sodiu m ch loride drops or oin t m en t can be ben eficial. Pat ien ts w ith con curren t dr y-eye disease sh ould use addit ion al preser vat ive-free ar t ificial tears or gels. Th erapeu - t ic ban dage len ses can provide tem porar y relief. Surgical opt ion s in clu de an terior st rom al pun ct u re, Na:Yag st rom al pu n ct u re, epith elial debridem en t , ph ototh erapeut ic keratectom y, or diam on d bu rr keratectom y.

160 Color Atlas of Ophthalm ology

Fig. 5.13 Meesmann dystrophy.

Meesmann Dystrophy

Meesm an n dyst rophy is a rare bilateral au tosom al dom in an t con dit ion occu rring early in life th at resu lts from a th icken ed basem en t m em bran e w ith a fibrogran u - lar “pecu liar su bstan ce” (possibly hyalin e) w ith in th e ep ith elial cells.

Presentation

Pat ien ts m ay presen t w ith pain , blu rred vision , ph otoph obia, tearing, an d redn ess from recu rren t erosion s. Tiny epith elial vesicles are seen from lim bus to lim bu s, m ost clearly on ret roillu m in at ion (Fig. 5.13).

Differential Diagnosis

Cyst in osis, corn eal gu t tae, epith elial basem en t m em bran e dyst rop hy (EBMD), t raum at ic recu rren t erosion

Management

No t reat m en t is requ ired u n less recu rren t erosion s develop . Sim ilar t reat m en ts m ay be perform ed as w ith EBMD, alth ough a deep an terior lam ellar keratoplast y can also be useful in cases recalcit ran t to recurren t erosion t reat m en t opt ion s.

Reis-Bücklers Dystrophy

Reis-Bü cklers dyst rophy is a progressive bilateral autosom al dom in an t dyst rophy th at develops early in life. Gen et ic lin kage occurs from a m u tat ion of th e BIGH3 gen e on ch rom osom e 5q31.

Presentation

Pat ien ts presen t w ith recurren t erosion sym ptom s of blurred vision , pain , ph otoph obia, tearing, an d redn ess. Exam in at ion reveals a superficial gray-w h ite ret icu - lar h aze m ore con cen t rated in th e cen t ral corn ea. Sign ifican t scarring can lead to Salzm an n n odules an d an irregu lar corn eal su rface (Fig. 5.14).