Ординатура / Офтальмология / Учебные материалы / Color Atlas of Ophthalmology The Quick-Reference Manual for Diagnosis and Treatment
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4 External Diseases 131
Conjunctival Pigmentations1
Melan ocyt ic ben ign conjun ct ival lesion s are ver y com m on , especially in darkerskin n ed in dividu als, in clu ding blacks or African Am erican s, Hispan ics, an d Git a- n os, in th e lim bal area or caru n cle. Presen t at ion greatly differs from on e pat ien t to an oth er. Th e lesion s h ave n o m align an t poten t ial, alth ough n evi ver y rarely give rise to m align an cy (jun ct ion al an d com pou n d n evi). Ch ron ic u se of topical epi- n eph rin e (as a cosm et ic), silver-con tain ing com pou n ds (argyrosis), ret ain ed iron foreign body, atabrin e, tet racyclin es, clofam icin e, im bibed m ascara gran u les, radiat ion , h orm on es (pregn an cy, Addison disease), ch em icals (arsen ic, th orazin e), an d som e ch ron ic disorders (like t rach om a or xeroderm a pigm en tosu m ) are poten t ial cau ses of pigm en t at ion in crease (darken ing of th e lesion s). Never th eless, th e m ost com m on cau se of pigm en t at ion in crease is th e topical use of prostaglan - din an alogues to low er in t raocular pressure. Conjun ct ival pigm en t at ion is n o sign of m align an cy.
Presentation
Conju n ct ival ben ign epith elial m elan osis presen t s as a flat brow n ish pigm en t at ion seen especially in blacks or African Am erican s, in th e lim bal area or caru n cle. Con - jun ct ival p igm en ted lesion s close to th e lacrim al glan d can be ben ign , bu t alw ays con sider m align an cy because m align an cy m ay be m ore exten sive th an is clin i- cally apparen t . Su spect m align an cy if pigm en t at ion in creases in parallel to su rface grow th (Fig. 4.24A–D).
Differential Diagnosis
Conju n ct ival m elan om a
A B
C D
Fig . 4.24 (A–D) Conjunctival pigm entations.
1Nom en clat u re m ay be con fu sin g, an d classificat ion is often con t roversial: w e h ave u sed th e m ost w id ely accepted n am es.
132 Color Atlas of Ophthalm ology
Management
Use close obser vat ion an d follow -u p w ith ph otograph ic docu m en tat ion . Fluorescein angiography m ay h elp to visualize feeder vessels in m elan om as. In case of dou bt , excision al biopsy sh ou ld be p erform ed .
Malignant Melanoma of the Conjunctiva
Malign an t m elan om a of th e conjun ct iva is rare, but it is th e m ost com m on pig- m en ted m align an cy of th e conjun ct iva an d accou n t s for 2%of all ocu lar m align an - cies. It is far less com m on th an in t raocular ch oroidal m elan om a. It can appear in a previou sly h ealthy par t of th e conju n ct iva (de n ovo, from m elan ocyt ic cells of th e basal layer of conju n ct iva, in ~20 to 25%of cases), from a preexist ing conju n ct ival jun ct ion al or com p oun d n evu s (~20%of cases), from a prim ar y acquired conju n ct i- val m elan osis (~60%of cases). Th e in ciden ce of conjun ct ival m elan om a is in creasing am ong w h ite m en in a t ren d sim ilar to th at of skin m elan om a. Conju n ct ival m elan om a is probably related to su n exposu re (Fig. 4.25).
Presentation
Clin ical presen tat ion is variable, w ith a raised, pigm en ted or n onpigm en ted lesion (som e h ave lit tle or n o pigm en t , w h ich is rare) th at grow s an d/or bleeds an d/or fixates to th e un derlying t issu es. Periph eral corn eal in filt rat ion is possible. Som e grow aroun d th e lim bu s. Som et im es th ere is pigm en t at ion of th e lid m argin s or lid skin (rare, poor progn osis). Region al lym ph n odes (parot id, p reau ricu lar, subm an - dibu lar, an d cer vical) m ay be affected . Distan t m et ast ases are possible, w ith spread by th e lym ph at ic vessels an d bloodst ream . Direct exten sion to th e eyeball an d orbit is possible if th ere is late diagn osis.
Susp ect m elan om a if
a preexist ing n evu s grow s or h as in creased vascularit y
a preexist ing conjun ct ival n evus at th e lim bu s h as rap id ver t ical grow th
a p reexist ing n evu s grow s or h as in creasing n odu larit y or ch anges in pigm en t a- t ion or bleeds, or develops in flam m at ion
a previou sly flat area of pigm en tat ion develop s n odu lar th icken ing
th ere is local conjun ct ival in creased vascu larit y (w ith or w ith out a pigm en ted lesion )
Fig. 4.25 Semilunar fold melanoma.
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th e conju n ct iva fixates to th e sclera
you obser ve h em orrh age in a pigm en ted lesion
you obser ve an u lcerat ive area in th e conjun ct iva th at does n ot h eal w ith topical m edicat ion
Differential Diagnosis
Ben ign pigm en ted lesion s [n evu s, m elan osis, foreign body, pigm en ted |
dep osits |
(ch em icals, m ascara gran u les)]. Suspect m align an t m elan om a in th e |
case of a |
grow ing pigm en ted lesion . Not all conju n ct ival m elan om as are pigm en ted; th ey can look like a squam ou s or sebaceous glan d carcin om a, a papillom a, lym ph oid hyperplasia, an d even a pter ygiu m . Metastat ic t u m or to th e conju n ct iva (ext rem ely rare, e.g., secon dar y m elan om a from cut an eou s t um or).
Management
Man agem en t depen ds on path ological staging [varies in differen t cou n t ries: clin i- cal t um or, n ode, m et ast ases (TNM) classificat ion , path ological classificat ion , an d h istopath ological t ype an d grade].
Can be t reated con ser vat ively w ith th e proton accelerator.
Com plete resect ion of t u m or (w ith care to avoid t issue dissem in at ion ). Path o-
logical exam in at ion is requ ired at th e t im e of excision .
Conju n ct ival m elan om as at th e lim bu s: Absolu te alcoh ol epith eliectom y + w ide local resect ion (par t ial lam ellar scleroconju n ct ivoten on ectom y w ith a 2–3 m m clear zon e) + cr yoth erapy of th e bed an d borders of excision
Palpebral conju n ct ival m elan om as (or forn ical): Surgical resect ion w ith absolute alcoh ol t reat m en t to th e scleral base an d cr yoth erapy to th e surrou n d - ing conju n ct iva
Large invasive m elan om as: Orbit al exen terat ion ? (alm ost alw ays m etast ases h ave already occurred at th e t im e of t reat m en t). We prefer th e proton accelerator.
A con ser vat ive approach w ith topical m itom ycin - C is un der research .
Adju n ct ive radioth erapy an d/or ch em oth erapy m ay be n eeded .
Life-long close obser vat ion follow -up w ith palpat ion of lym ph n odes (th ree to fou r t im es yearly)
Blue Sclera and Scleral Staphyloma
Blue Sclera in Osteogenesis Imperfecta
Blu e sclerae in in fan cy an d ch ildh ood reflect eith er an abn orm al th in n ess of th e sclera or an in creased scleral t ran sparen cy. Th e u n derlying uveal pigm en t produ ces th e blu e-gray appearan ce. Sligh t blue sclerae are com m on in n eon ates, par- t icu larly if th ey are prem at u re. It can be con sidered a n orm al varian t in th e first several m on th s of life. In case of persist ing pron oun ced blu en ess, op h th alm ological evaluat ion is n eeded to ru le ou t th e presen ce of elevated in t raocular pressu re (IOP). Pediat ric an d or th opedic evalu at ion is n eeded to rule ou t in h erited diseases resp on sible for th e blue-t in ted sclerae, such as osteogen esis im perfect a.
Osteogen esis im p erfect a is a rare in h erited congen it al disorder w ith ext rem e bon e fragilit y, caused by m ut at ion s in th e gen es th at codify for t yp e I procollagen (COL1A1 an d COL1A2). At least four t ypes of osteogen esis im perfect a h ave been
134 Color Atlas of Ophthalm ology
Fig . 4.26 Osteogenesis imperfecta. Blue sclera.
described . Th e age w h en fract ures begin varies w idely. Pat ien t s w ith m ild form s m ay presen t w ith fract ures in in fan cy or m ay n ot h ave fract ures u n t il adu lth ood . Th e m ore severe cases m ay h ave fract u res in u tero.
Presentation
Presen t at ion is asym ptom at ic; th ere are n o ocu lar sym ptom s. In pat ien ts w ith osteogen esis im perfect a, th e sclera can be blue or w h ite (Fig. 4.26).
Differential Diagnosis
Blue sclera also m ay occu r in oth er disorders, su ch asalkapton uria, progeria, cleidocran ial dysplasia, cu t is laxa (pseu doxan th om a elast icum ), Eh lers-Dan los syn - drom e, Marfan syn drom e, Ch en ey syn drom e, Men kes syn drom e, an d pykn odysostosis. Som e h igh or ext rem e m yopes m ay presen t w ith “blu e-sclera” due to scleral th in n ing.
Management
No ocu lar t reat m en t is n eeded . Oth er system ic invest igat ion s an d t reat m en t are to be don e. Gen et ic coun seling is h elpfu l.
Blue Sclera and Scleral Staphylomas
Congen it al glaucom a in an in fan t or a you ng ch ild is ch aracterized by th ree m ain sym ptom s: excessive tearing (epiph ora), sen sit ivit y to ligh t (ph otoph obia), an d spasm s or squ eezing of th e eyelids (bleph arospasm ). Th ere are both prim ar y (m aldevelopm en t of an terior ch am ber angle) an d secon dar y form s w ith diverse cau ses. Th e con dit ion is m ore frequen tly bilateral. Its severit y is variable (Fig. 4.27).
W h eth er en largem en t of th e eye occurs or n ot depen ds on th e age of on set of th e glau com a. Th e eye being disten sible in in fan cy, elevated in t raocu lar pressure m ay result in eye en largem en t (buph th alm os). Corn eal en largem en t can also occur in th e early first years, an d th e sclera can expan d during th e first decade. Scleral th in n ing (ect asia) an d bu lging blu ish areas of th in n ed sclera lin ed by uveal t ract (staphylom a) can also be seen in advan ced cases. Th e corn eal h orizon tal diam eter also in creases. Corn eal en largem en t resu lts in ru pt u res in th e Descem et m em - bran e (Haab st riae).
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Fig . 4.27 Staphylomas in congenital glaucoma buphthalmos.
Presentation
Epiph ora, p h otoph obia, an d blep h arospasm are seen in a n ew born or you ng in - fan t . Bu ph th alm os, ectasia, st aphylom as, an d in creased corn eal diam eter m ay also be presen t . Corn eal edem a or Haab st riae m ay be seen at slit-lam p exam in at ion , (w h ich m ay n ot alw ays easy to perform in babies).
Differential Diagnosis
With causes of blu e sclera (e.g., osteogen esis im perfecta), n eoplasias, an d causes of congen ital glaucom a:
Dysgen esis of th e iris, angle, an d periph eral corn ea w ith or w ith out system ic abn orm alit ies (e.g., Rieger an om aly syn drom e, Axen feld an om aly syn drom e, Peter an om aly, an iridia, Marfan syn drom e, Weill-March esan i syn drom e)
Ph akom atoses (e.g., n eu rofibrom atosis, St urge-Weber syn drom e)
Metabolic disease (e.g., ocu locerebroren al syn drom e or Low e disease, h om ocys- t in u ria)
In flam m ator y (e.g., r ubella, juven ile xan th ogran ulom a)
Ch rom osom al delet ion /du plicat ion (e.g., Tu rn er syn drom e, t risom y 13–15)
Management
Topical m edical t reat m en t is used to redu ce IOP un t il surger y can be safely p erform ed . Beta-blockers an d m iot ics rarely w ork (th ough th ey m ay h elp). Carbon ic an hydrase in h ibitors can be u sed to h elp redu ce corn eal edem a an d allow a bet ter in spect ion of an terior ch am ber st ru ct u res. Modern drugs (e.g., prostaglan din an a- logues, brim on idin e tar t rate) can be t ried, alth ough th eir u se in in fan t ile glaucom a is n ot curren tly accepted .
Surger y aim s at redu ct ion of com p licat ion s of IOP in crease (e.g., am blyopia, staphylom as, opt ic n er ve at rophy). We use ou r ow n su rgical tech n ique, called Si- m on’s pect in otom y (surgical ablat ion of t rabecu lodysgen et ic t issue–iridopect in eal t issue by m ean s of an in st ru m en t of our ow n design ), com bin ed, or n ot , w ith a gon iotom y, t rabecu lotom y, or t rabeculectom y. Glau com atous cu pping in in fan ts h as proven to be reversible w h en su ccessful su rger y is perform ed early.
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Conjunctivalization of the Cornea
Den se leu kom as, severe scarring, an d conjun ct ivalizat ion of th e corn ea can be fou n d after bon e m arrow t ran splan t at ion , severe corn eal or in t raocu lar in fect ion s, or ocular radiat ion th erapy. A sim ilar con dit ion can be th e result of severe ch em i- cal burn s or au toim m un e disorders.
Presentation
Severe scarring, leukom a, an d conjun ct ivalizat ion of th e globe are seen (Fig. 4.28).
Differential Diagnosis
Ph th isis bu lbi, at rofia bu lbi, en doph th alm it is sequ elae, Steven s-Joh n son syn drom e, er yth em a m ult iform e, ch em ical bu rn s
Management
Palliat ive t reat m en t to alleviate discom for t con sists of surface lubricat ion w ith n onp reser vat ive art ificial tears or oin t m en t s, pun ct al occlu sion , an d h u m idifiers or m oist u re ch am bers to decrease tear film evaporat ion . Surgical tarsorrh ap hy is used for in t ractable dr y eye. Mydriat ics an d covering th e globe w ith am n iot ic m em bran e can provide relief. Cosm et ic prosth esis after en ucleat ion is p erform ed if n eeded .
Fig . 4.28 Massive corneal neovascularization and opacification following radiotherapy.
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Conjunctival Metaplasia in Ectropion
Metaplasia of th e conju n ct iva is an un com m on disease w h ere th e conju n ct ival m u - cosa ch anges in to a skin like surface due to ch ron ic exposu re of th e conju n ct iva to air an d ligh t . We also describe kerat in izat ion of th e conju n ct iva.
Presentation
Conjun ct ival hyp erem ia an d lacrim at ion are seen in ten se ch ron ic lid eversion (ect rop ion ). Th ere is also
4.29).
a pat ien t su ffering from in - foreign body sen sat ion (Fig.
Differential Diagnosis
Squ am ous cell carcin om a of th e conju n ct iva
Management
In ten se lu bricat ion w ith eye drops, gels, an d oin t m en ts u n t il su rger y of ect ropion is perform ed .
Fig . 4.29 Conjunctival m etaplasia in ectropion.
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Chemosis
Ch em osis con sists of conjun ct ival an d subconju n ct ival edem a. In gen eral, ch em o- sis is a n on specific sign of eye irrit at ion .
Presentation
Liquid collect ion is seen u n der th e conjun ct iva. Som et im es th e conjun ct iva becom es so sw ollen th at th e eyes can n ot close properly. Oth er sign s an d sym ptom s depen d on th e cause of ch em osis (Table 4.2) (Fig. 4.30A–C).
Differential Diagnosis
Any cau se of ch em osis (Table 4.2)
Table 4.2 Com m on Causes of Chem osis
Severe am mation:
Gonococcus, viral or chlamydial conjunctivitis Allergic or hypersensitivit y reaction Endophthalm itis
Panophthalmia Local irritation
Trauma: Ruptured globe
Post trabeculectomy
Postsurgical after scleral or vitreoretinal surgery Generalized edema:
Nephropathy (of any origin) Heart disease (congestive) Quincke edema
Urticaria Myxedema
Venous congestion Cavernous sinus Orbital
Orbital
am mation: Orbital cellulitis
Endocrine thyroid disease (thyrotoxicosis) Drugs:
Atropine Epinephrine
Glaucoma medications uridine
Parasites:
Trichinella spiralis dissemination
4 External Diseases 139
A
B
C
Fig. 4.30 (A) Mild chem osis. (B) Massive chem osis. (C) Massive chem osis.
140 Color Atlas of Ophthalm ology
Management
Treat th e un derlying con dit ion (e.g., t reat allergic conju n ct ivit is if ch em osis is due to an allergic react ion ). Ch em osis after t rabecu lectom y is h igh ly desirable an d does n ot n eed to be t reated: th e n ovice m ay be tem pted to perform early surgical revision of th e filtering bleb, w h ich is on ly n ecessar y if atalam ia w ith corn eal edem a is presen t .
Hyaline Pillat Scleral Plaque
Pillat scleral plaqu e is an involut ive th in n ing of th e sclera, w ith hyalin e degen era- t ion , resu lt ing from t ract ion forces of ext raocu lar m uscles, especially th e lateral rect us.
Presentation
A grayish pun ch ed -ou t area is seen an terior to th e in ser t ion of th e lateral rect us m u scles. Slit-lam p exam in at ion is diagn ost ic. Th ere is n o clin ical m an ifestat ion , but th e p laqu es are visible in older pat ien ts (Fig. 4.31).
Differential Diagnosis
Sclerom alacia, m elan osis bulbi, scleral ect asia, staphylom as
Management
No t reat m en t is n eeded .
Fig . 4.31 Hyaline scleral Pillat’s plaque.