Ординатура / Офтальмология / Учебные материалы / Color Atlas of Ophthalmology The Quick-Reference Manual for Diagnosis and Treatment
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4 External Diseases 111
Fig. 4.8 Giant papillae. Vernal conjunctivitis.
of con tact len ses—h ard, hydrogel, scleral, prosth et ic, etc.). Th e con dit ion can be m ore aggressive in ch ildren , w ith lid sw elling an d pseudoptosis of th e su perior lid (Fig. 4.8).
Differential Diagnosis
Atopic conju n ct ivit is, vern al conju n ct ivit is
Management
Discon t in ue con t act len s or prosth esis w ear for at least 3 to 4 w eeks. Most pat ien ts do n ot require m ore aggressive t reat m en t . Topical m ast cell–st abilizing solu t ion s, an t ih istam in es, an d top ical steroids (drops an d oin t m en t s) can be used . Excep - t ion ally, gian t papillae m ay requ ire steroid depot inject ion at th e t arsu s. Silver n i- t rate or radiosu rger y su rgical curet tage of large/gian t papillae m ay be useful in som e cases (e.g., corn eal ulcerat ion ). Plasm aph eresis h as been su ccessfully u sed as adjun ct th erapy for pat ien ts w ith h igh im m u n oglobu lin E levels (e.g., vern al conju n ct ivit is). En cou raging th e pat ien t to replace con tact len ses frequen tly, u se preser vat ive-free len s solut ion , an d in crease len s hygien e all are good preven t ive m easu res.
Cicatrizing Disorders
Ocular Cicatricial Pemphigoid
Cicat ricial pem ph igoid is an autoim m u n e disorder of un kn ow n et iology, w h ere circulat ing an t ibodies an d com plem en t bin d to th e basem en t m em bran e of m ucosal t issues. It is a ch ron ic, bilateral, papillar y cicat rizing conjun ct ivit is (Fig. 4.9).
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Fig . 4.9 Benign pemphigoid.
Presentation
Age of presentat ion: Aged adu lts (rarely seen in ch ildren )
Sex: Wom en are affected t w ice as frequ en tly as m en .
Main early feat ure: Ch ron ic bilateral papillar y cicat rizing conju n ct ivit is
Ocular findings:
Conju n ct ival hyp erem ia w ith dr y eye sym ptom s, tearing, itch ing, ph otoph o- bia, foreign body sen sat ion s
Disch arge (i.e., catarrh al, m ucou s, m em bran ou s)
Eyelids m an ifest t rich iasis, dist ich iasis, m eibom ian glan d dysfun ct ion , bleph - arit is
Conju n ct iva m an ifest s pap illae, follicles, kerat in izat ion , su bepith elial fibrosis, conju n ct ival kerat in izat ion w ith foresh orten ing of th e forn ices an d sym - bleph aron an d/or an kylobleph aron (en d stage, im m obilizes th e eye)
Corn ea m an ifests su perficial pu n ctate kerat it is, epith elial defect , u lcerat ion s,
n eovascu larizat ion , kerat in izat ion , st rom al opacit ies, perforat ion (pseu do- t rach om a)
Differential Diagnosis
Chlamydial infections (trachom a, inclusion conjunctivitis), atopic keratoconjunctivitis, adenoviral conjunctivitis, long-term ocular m edication (old antiglaucom a m edications, e.g., epinephrine), chem ical (alkali) or therm al burns, radiation exposure (therapeutic or not), Sjögren syndrom e, sarcoidosis, derm atobullous disorders (toxic epiderm al necrolysis, congenital ichthyosiform erythroderm a, epiderm olysis bullosa), erythem a m ultiform e, Corynebacterium diphtheriae conjunctivitis, epitheliom a
Management
No topical agen t is really effect ive in stop ping act ivit y (dexam eth ason e oin t- m en t m ay h elp). Best con t rolled w ith system ic th erapy.
To slow disease p rogression t r y th e follow ing:
Subconjunctival steroid injections such as triam cinolone (Trigon, Bristol-Myers Squibb, New York) or betam ethasone (Celestone, Schering Corp ., Kenilw orth, NJ). We use prolonged-release betam ethasone, injecting as m uch as possible, subconjunctivally (Celestone-Cronodose, Schering Corp., Kenilw orth, NJ the strongest “depot” steroid).
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Abu n dan t ar t ificial tears an d topical lu brican ts in pat ien t s w ith dr y |
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Steroid oin t m en t s (e.g., dexam eth ason e oin t m en t , t w o to four t im es daily) |
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System ic im m u n osu ppressive th erapy, u su ally con t in ued for 9 to 12 m on th s. Mu st be con t rolled by a specialist in im m u n osup pressive th erapy (e.g., diam in odiph enylsu lfon e or cycloph osph am ide + system ic predn ison e)
System ic cor t icosteroids for severely in flam ed eyes th at do n ot respon d to im m u n osu ppression alon e
Treat com plicat ion s su ch as epilat ion of an aberran t lash (m ech an ical, laser, cr yodest ru ct ion ), pu n ctal occlu sion for dr y eye, or lid su rger y for en t ropion .
Stevens-Johnson Syndrome
Stevens-Johnson syndrom e is an acute hypersensitivity reaction consisting of an inflam - m atory vesiculobullous reaction of the skin and m ucous m em brane. Im m une complex deposition is incited by m edications, including sulfonam ides, anticonvulsants, aspirin, penicillin, isoniazid, diam ox (acetazolam ide), and m any m ore, and som etim es infectious organism s (herpes simplex virus, streptococci, adenovirus, mycoplasm a).
Presentation
Th ere is an acu te on set of fever, rash , red eyes, m alaise, ar th ralgias, an d respirator y t ract sym ptom s. Classic “t arget” skin lesion s (m aculop apu les w ith a red cen ter an d a w h ite surroun d on an er yth em atous base) are con cen t rated on th e h an ds an d feet . Oth er sym ptom s in clu de u lcerat ive stom at it is an d h em orrh agic lip cru st ing. Th e m or talit y rate is 10 to 33%.
Ocu lar sign s in clu de m ucopu ru len t or pseu dom em bran ou s conjun ct ivit is, episclerit is, an d irit is in th e acu te ph ase. Late com plicat ion s in clu de conju n ct ival scarring or sym bleph aron , t rich iasis, eyelid deform it ies, tear deficien cy, corn eal n eovascularizat ion , u lcer, perforat ion , or scarring.
Erythem a m ult iform e m ajor (Stevens-Johnson syndrom e): Im m u n e com plex deposit ion in th e derm is w ith su bepith elial vesiculobu llou s react ion of th e skin an d m u cou s m em bran es
Erythem a m ult iform e m inor: On ly skin involvem en t
Toxic epiderm al necrolysis: Th e m ost severe form , causing exten sive in t raepith e- lial vesicu lobu llou s erupt ion s an d epiderm al n ecrosis; m ore com m on in ch ildren an d im m un osu ppressed pat ien ts
Differential Diagnosis
Ocular cicatricial pem phigoid, chem ical burns, radiation, squam ous cell carcinom a
Management
Taking a h istor y to r ule out a precip itat ing factor is im por tan t . Slit-lam p exam in a- t ion , in clu ding eyelid eversion w ith exam in at ion of th e forn ices, conjun ct ival or corn eal cu lt ures, an d con su ltat ion w ith in tern al m edicin e, is a m u st . Hospit alize th e pat ien t , rem ove th e in it iat ing factor, an d provide sup por t ive care as th e m ain - stays of t reat m en t .
Ocu lar m an agem en t in cludes m an agem en t of associated dr y eye, irit is, topical steroids for ocular surface in flam m at ion , daily pseu dom em bran e peel, an d sym - bleph aron lysis w ith a glass rod or m oisten ed cot ton sw ab, system ic or topical vitam in A, an d in t raven ous im m u n oglobulin . To m an age th e late com plicat ion s pen et rat ing keratop last y w ith stem cell t ran splan t , an d am n iot ic m em bran e t ran s- plan t , or perm an en t keratoprosth esis m ay be requ ired .
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Dry-Eye Syndrome
Most dr y eyes are m u lt ifactorial. Th e eye can be dr y as a result of defect ive produc- t ion of tears (e.g., age-related dr y eye in m en opau sal w om en ) or excessive evapora- t ion (e.g., exoph th alm os).
Presentation
Sym ptom s: Foreign body sen sat ion , sen sat ion of ocu lar dr yn ess an d grit t in ess
(in it ially at th e en d of th e day an d later th rough out th e w h ole day), hyperem ia an d ocu lar irrit at ion (exacerbated by sm oky or dr y environ m en t s, in door h eating system s, prolonged reading, or u se of com puters), m u cous disch arge, excessive tearing (secon dar y to reflex secret ion )
Signs: Red eyes, conju n ct ival hyperem ia, decreased tear m en iscus, in creased debris in th e tear film , su perficial pu n ctate keratopathy (w ith flu orescein , lis-
sam in e green , an d/or rose bengal posit ive st ain ing), m u cous plaques an d disch arge, corn eal filam en t s (severe cases), corn eal epith elial defect s or ulcers (in m ore severe cases) (Fig. 4.10).
Differential Diagnosis
Any conjun ct ivit is (especially toxic form s, w h ich are m ore difficult to differen t i- ate). Careful h istor y an d w orku p sh ou ld h elp to establish th e origin of th e dr y eye (e.g., air con dit ion ing, Sjögren syn drom e)
Management
Tru e dr y eye can n ot be cu red, bu t eye sen sit ivit y can be lessen ed an d m easu res taken so th e eyes rem ain h ealthy by m ean s of art ificial tears or tear su bst it u tes. No con t act len s pat ien t or glau com a t rabeculectom ized pat ien t sh ould be out of ar t ificial tear lu bricat ion or tear subst it utes!
First step: Sup plem en tal lubricat ion (m ild an d m oderate kerat it is sicca)
Art ificial tears: Preferably preser vat ive-free ar t ificial tears (drops 4 to 14 t im es a day, depen ding on th e severit y of th e case). Our preferred ocu lar lu - brican t is sodiu m hyaluron ate (0.18 to 0.4%).
Fig . 4.10 Dry eye, lagophthalm os, corneal and conjunctival rose bengal staining.
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Viscous artificial tear drops or gels: How ever, they tem porarily blur the vision .
Lubricat ing oint m ents: For m ore severe cases (gen erally reser ved to bedt im e,
becau se vision blur lasts m in utes or h ours). Not to be used w ith con t act len ses.
In severe cases: Use a patch w ith lubricat ing oin t m en t at n igh t .
In case of abundant m ucus: Rem ove th e m ucou s an d add 10%N-acet ylcystein e, th ree to fou r t im es daily.
Art ificial tear insert (e.g., Lacrisert, Aton Pharm a, Inc., Law renceville, NJ): Place in to th e in ferior cu l-de-sac ever y m orn ing.
Special goggles, m oist ure cham ber glasses: To redu ce evap orat ion an d ret ain h um idit y aroun d th e globe.
In case of suspected inflam m at ion or in case of unsat isfactory results: Tr y topical steroids (before cyclosporin e).
Treat any associated abnorm alities: (e.g., in blepharitis, suppress inflam m ation w ith topical steroids and local antibiotics, and/or system ic tetracyclines)
Interm ediate step:
Tem porary punctal occlusion: With collagen (dissolvable) or silicon e (perm a- n en t) plugs in case of severe aqueou s tear deficien cy (to preser ve en dogen ou s w ater)
In-office cauterizat ion of the inferior lacrim al punct i: If th e pat ien t is sat isfied w ith tem poral occlusion result s
Minim ize exposure: Consider tarsorrhaphy or botulinum -toxin -induced ptosis.
Last step: Su rgical t reat m en t (on ly for ver y severe cases, w ith u lcerat ion or cor- n eal perforat ion )
Closure of perforat ion or descem etocele: Cyan oacr ylate t issu e adh esive
Corneal or corneoscleral patch: For an im pen ding or fran k perforat ion (am n i- ot ic m em bran e, fascia lat a)
Lateral tem porary tarsorrhaphy: For exam ple, after facial n er ve paralysis, after t rigem in al n er ve lesion s, or for severe exoph th alm os secon dar y to thyroid disease
Conjunct ival flap: Aids in preven t ing corn eal m elt an d perforat ion by covering th e corn ea w ith conjun ct iva
Several variet ies of con tact len ses can aid in th e t reat m en t of dr y eye. Hard con - tacts m ay st im u late reflex tearing an d th u s in crease th e volu m e of tears. Som e h ard scleral con t act len ses m ay be ben eficial by preven t ing evap orat ion from a large por t ion of th e ocular su rface. Th e U.S. Food an d Drug Adm in ist rat ion h as ap - proved on e t ype of con tact len s (Proclear Toric XR len s, CooperVision , Fairpor t , NY) w ith an in dicat ion for dr y eyes. Th is is a soft len s th at h as som e u n iqu e proper t ies. It has a h igh w ater con ten t like oth er soft len ses, bu t it also h as a com pon en t th at retain s w ater bet ter th an m ost oth er soft len ses. Th is reduces th e dehydrat ion th at occu rs w ith m ost soft con tact len ses. Clin ical st u dies h ave dem on st rated im prove- m en t in com fort an d sign s of dr yn ess on th e surface of th e eye w ith th is con t act lens w h en com pared w ith a grou p of oth er len ses w ith w h ich it w as tested . Neverth eless, con tact len ses alon e h ave n o place in th e t reat m en t of dr y eyes; con com i- tant u se of art ificial teardrops (an d periodic ch ecku ps) is essen t ial.
In som e cases, sm all pu n ctal plugs m ay be in ser ted in th e in ferior lacrim al pun ct u m to slow drain age an d loss of tears.
Cyclosporine 0.05%ophthalm ic em ulsion (Restasis, Allergan, Inc., Irvine, CA): Can be u sed to relieve dr y eyes caused by su ppressed tear produ ct ion secon dar y to ocu lar in flam m at ion . Safet y for u se in ch ildren an d du ring p regn an cy h as n ot been establish ed .
Autologous serum : It h as a ben eficial effect on corn eal epith elium (20%au tologou s serum dilu ted in n orm al salin e).
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Tet racyclin es: Tradit ion ally u sed as an t ibiot ics, th ey also h ave im port an t an - t iin flam m ator y proper t ies. Ver y u sefu l (doxycyclin e) in t reat ing acn e rosacea dr y eye. Tet racyclin es are also effect ive again st recu rren t corn eal erosion s an d ph lycten u lar keratoconju n ct ivit is.
Oral pilocarpine (Salagen, Novart is Pharm aceut icals UK Ltd., Surrey, UK): In it ially u sed in xerostom ia (salivar y glan d dysfu n ct ion , com m on after irradiat ion of th e n eck in on cology), it h as been fou n d u sefu l in severe xeroph th alm ia by st im u - lat ing tear secret ion (5 to 10 m g/8 h ). Good result s are seen in Sjögren syn drom e after a few w eeks of t reat m en t .
Salivary gland t ransplantat ion into the inferior tarsal conjunct iva: Has been repor ted to be usefu l in dr y-eye con dit ion s w ith severe perm an en t lacrim al glan d dysfu n ct ion s (n ot u sefu l in Sjögren syn drom e). Tech n ically com p lex.
Symblepharon
Sym bleph aron is an adh esion bet w een th e palpebral conjun ct iva an d th e bulbar conju n ct iva or corn ea. Sym bleph aron is usually th e result of a t raum a (surger y, ch em ical or radiat ion bu rn s) or sup erficial eye in flam m at ion (er yth em a m u lt i- form e, t rach om a, burn s, pem ph igoid, Steven s-Joh n son syn drom e).
Presentation
Sym ptom s in clu de adh esion bet w een th e palpebral conju n ct iva an d th e bulbar conju n ct iva or corn ea, lid deform it ies, fu n ct ion al lacrim al occlu sion an d tearing, en t ropion , an d dist ich iasis (Fig. 4.11).
Differential Diagnosis
Diagnosis is apparently clinical and is seldom confused w ith other disease entities.
Management
Treatm ent is often frustrating, and progressive scarring cannot be com pletely con - trolled or reversed. Grow th prevention includes breaking early adhesions w ith a rectal therm om eter t w ice a day and steroid ointm ents. Conform ers can be tried. Surgical Z-plast y w ith am niotic m em brane or m ucosal graft can be tried. The use of
A B
Fig . 4.11 Symblepharon: (A) bet ween bulbar and palpebral conjunctiva; (B) at the lateral canthal angle.
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am niotic m em brane can be beneficial in that it facilitates epithelialization, m aintains norm al epithelial phenot ype (w ith goblet cells w hen perform ed on the conjunctiva), and effectively helps in reducing inflam m ation, vascularization, and scarring. Im m u - nosuppressant therapies (m ethotrexate, cyclophospham ide, cyclosporine, azathioprine, etc.) can help in the acute episodes of the illness. In late cicatricial stages, treat collateral effects such as dr y eye syndrom e, and punctal occlusion .
Pinguecula, Pterygium, and Lipoid Degeneration
Pinguecula
Pinguecu lae are th e m ost com m on ben ign conjun ct ival t u m ors. Th ey u su ally affect m iddle-aged in dividuals bu t can also be foun d in ch ildren an d young peop le, especially th ose w ith dyslipidem ia. Pingu ecu lae h ave n o sex or racial predilect ion . Th ey seem related to ch ron ic exposure to th e su n .
Presentation
Pingueculae are usually asym ptom at ic, yellow ish /w h it ish , sligh tly raised, in terpalpebral lipid -like dep osits in th e n asal (m ore frequen t) an d tem poral lim bal con - jun ct iva. Un i- or bilateral (usually bilateral an d asym m et rical), th ey m ay becom e vascu larized an d in flam ed (pingu eculit is). In a case of in flam m at ion , pat ien t s experien ce foreign body sen sat ion an d conju n ct ival redn ess arou n d th e pingu ecu la, corn eal p un ct ate epith eliopathy, an d corn eal th in n ing secon dar y to dr yn ess (dellen u lcerat ion ). Th ese pat ien ts m ay com plain of dr y-eye sym ptom s an d foreign body sen sat ion (Fig. 4.12A,B).
Fig . 4.12 (A) Pingueculae. |
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(Continued on page 118) |
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B
Fig . 4.12 (Continued) (B) Pinguecula and melanoma.
Differential Diagnosis
Pter ygiu m , conju n ct ival derm oid, conju n ct ival n eoplasia (a u n ilateral, w h ite, vascularized m ass), p h lycten ule, pan n u s, conju n ct ival reten t ion cyst (a clear, fluid - filled sac), lim bal follicles
Management
No t reat m en t is n ecessar y in cases w ith good lubricat ion resu lts, w h ich h ave a low er risk of pingu eculae form at ion (preser vat ive-free tear subst it u tes are preferable).
Con sider surgical part ial or tot al resect ion u n der local an esth esia in th e follow - in g:
Severe cases (gian t pingueculae) w h ere pter ygiu m is presen t an d in terfering w ith vision
Ch ron ically in flam ed severe cases
Un com for table con t act len s w ear
Bad corn eal lu bricat ion w ith dellen form at ion
Cosm et ic problem s
In th e case of in flam m at ion , u se ocu lar lu bricat ion an d m ild topical steroids (e.g., flu orom eth olon e), w ith decongest an ts (e.g., n aph azolin e).
Preven t ion is possible for peop le w ith occupat ion s or h obbies th at in crease th e risk of pinguecu la (sailing, fish ing, skiing, garden ing, outdoor con st ru ct ion w ork). Sun goggles, u lt raviolet-blocking coat ings, or goggles th at lim it dust exposure are h elpful.
Pterygia
Like pinguecu lae, pter ygia h ave been related to exposu re to u lt raviolet ligh t (both ult raviolet A [UV-A] an d UV-B). Risk factors in clu de living in su bt ropical an d t ropical clim ates, ou tdoor act ivit ies (e.g., golf, sailing, fish ing), dr y w in dy clim ates, dust , an d fu m es. A gen et ic predisposit ion h as been described . Th ere are t w o groups of pter ygium pat ien t s: pter ygia w ith m in im al proliferat ion an d at roph ic appearan ce
4 External Diseases 119
A
B
Fig . 4.13 (A) Pterygium . (B) Pte - rygium postsurgical papilloma. (C) Terrien’s marginal degeneration.
C
(slow -grow ing pter ygia, w ith low in ciden ce of recu rren ce after excision ) an d elevated fibrovascu lar pter ygia (rapid grow th , aggressive clin ical cou rse, an d h igh rate of recu rren ce after excision ) (Fig. 4.13A,B,C)..
Th ere is a predilect ion for m ales, an d occu rren ce is m ore frequ en t in th e n asal conju n ct iva.
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Presentation
A t riangular, fleshy, elevated m ass of bu lbar conjun ct iva grow s over th e corn ea w ith in th e in terpalpebral fissure. Sm all pter ygia are asym ptom at ic. Th ey can becom e in flam ed, w ith redn ess, foreign body sen sat ion , an d ocular irrit at ion (dr y- eye sym ptom s). In advan ced cases, pat ien t s experien ce vision problem s (ast igm a- t ism , progression over th e visu al axis), rest rict ion of ocular m ot ilit y, an d blin dn ess (in u n derdeveloped cou n t ries w h ere surger y is u n available).
Differential Diagnosis
Pinguecu lae, am yloidal degen erat ion of th e conju n ct iva, pseu dopter ygium , n eoplasia (e.g., carcin om a in sit u , squ am ous cell carcin om a)
Management
Good in ten sive lubricat ion is essen t ial.
Topical steroids su ch as predn isolon e acetate (Pred For te, Allergan , In c., Ir vin e,
CA) 1%, th ree to fou r t im es daily an d an t ih ist am in es provide relief of in flam m a- t ion .
In dicat ion s for surger y in clude th e follow ing:
Cosm et ic reason s
Discom for t due to recu rren t in flam m at ion
Before it en croach es on th e pu pillar y area
Mult ip le differen t surgical procedu res w ork in th e first grou p of pter ygiu m pa-
t ien t s (at roph ic, slow grow ing), bu t n on e can be said to w ork sat isfactorily in th e secon d group of pat ien ts (fast grow ing, aggressive clin ical course):
Sim p le excision
Sim p le excision an d repair w ith conju n ct iva autop last y
Sim p le excision an d sliding flaps of conjun ct iva
With an d w ith ou t adjun ct ive extern al β radiat ion th erapy (1000 to 2000 rep s at lim bu s or th iotepa 1:2000 solu t ion ) an d/or in t raoperat ive topical m itom y- cin - C or postoperat ive 5-flu orou racil
Prim ar y excision plus free graft s of conjun ct iva an d lim bal t issu e (lim bal au - tograft) or am n iot ic m em bran e: for aggressive or recu rren t pter ygia
Lipoid/Lipid Degeneration of the Cornea and Conjunctiva
Lipid degen erat ion of th e corn ea an d conju n ct iva m ay occur in prim ar y or secon d - ar y form . Th e prim ar y form is usu ally bilateral an d m ore diffu se an d is cau sed by lipid seru m dyscrasias su ch as Tangier fam ilial h igh -den sit y lipoprotein deficien cy or lecith in ch olesterol acylt ran sferase deficien cy. Th e secon dar y form is by far th e m ost com m on form of th is rare disease, is m ore localized, an d is due to th e presen ce of corn eal blood vessels after ocu lar t rau m a or in terst it ial or h erpet ic kera- t it is.
Presentation
W h ite, yellow, or cream -colored den se opacificat ion of th e corn eal st rom a is seen in a diffu se or localized m an n er. Ch olesterol cr yst als occu r in th e corn eal or con - jun ct ival st rom a su rrou n ding aberran t blood vessels. Th e conju n ct ival feeder vessels are dilated, an d th ere are sym ptom s of dr y eye (Fig. 4.14).
Differential Diagnosis
Aspect at slit lam p is diagn ost ic. Ru le out degen erated epith eliom a.