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

3 Orbital Infections, Inam m ation, and Neoplasm s 71

Oral an t ibiot ics are in dicated in cases of m ild in flam m at ion , afebrile pat ien t , age m ore th an 5 years, good pat ien t com p lian ce. Drugs of ch oice in clu de am oxicillin - clavu lan ate, or cefaclor, or cot rim oxazole, or er yth rom ycin , clin dam ycin , am oxicillin -cloxacillin for a durat ion of 10 days. In t raven ou s an t ibiot ics are in dicated in m oderate to severe in flam m at ion s. In cases of pat ien t age less th an 5 years, p oor pat ien t com plian ce, im m un ocom prom ised pat ien ts, n o im provem en t w ith or w orsen ing w ith oral an t ibiot ics, dr ugs of ch oice in clu de ceft riaxon e w ith van com y- cin . Supp or t ive t reat m en t in clu des h ot fom en t at ion s, local an t ibiot ics (p olym yxin w ith bacit racin oin t m en t), an d n on steroidal an t i-in flam m ator y drugs (NSAIDs). Explorat ion of th e w oun d is perform ed if n eeded .

Orbital Inflammation

Orbital Cellulitis/Subperiosteal Abscess/Cavernous Sinus Syndrome

In orbit al cellu lites, in fect ion occu rs posterior to th e orbit al sept um , usually secon d - ar y to th e sp read of in fect ion from th e su rroun ding st ruct ures (paran asal sin u ses, lacrim al sac, upper respirator y t ract in clu ding th e m iddle ear) or lid in fect ion , su ch as severe acu te h ordeolu m , skin lacerat ion , or an in sect bite (Fig. 3.2A,B,C).

Presentation

Orbital cellulit is: Eyelid edem a (usually leading to in abilit y to open th e eye),

periorbit al sw elling, rubor, ten dern ess, proptosis, pain w ith eye m ovem en t s, rest ricted ocular m ot ilit y, decreased vision , ret in al ven ou s congest ion , opt ic disk edem a, pu rulen t disch arge, an d decreased periorbital sen sat ion . Th e follow ing are m ain t ypes:

Sin u s-related is th e m ost com m on an d is secon dar y to eth m oidal sin u sit is; it affect s ch ildren an d young adu lts.

Caused by adjacen t st ru ct u res like dacr yocyst it is, m idfacial in fect ion , or den - tal in fect ion .

Post t raum at ic m ost com m on ly develops w ith in 48 to 72 h ou rs of an inju r y th at pen et rates th e orbit al sept u m .

Subperiosteal abscess: Most frequ en tly located along th e m edial w all of th e orbit . Orbital abscess is relat ively less com m on w ith sin usit is but is m ore com m on

in post t rau m at ic or postoperat ive cases. Usually presen t s w ith m edial m ass, n on axial proptosis, local ten dern ess, in creased in t raocu lar pressure, abscess (in t racon al/ext racon al).

Cavernous sinus throm bosis: Bilateral, decreased visu al acuit y; decreased sen sa- t ion along th e first an d secon d division of th e t rigem in al n er ve; rapidly progressive proptosis; paresis of cran ial n er ves III, IV, an d VI; congest ion of th e conju n ct ival vein s; ch em osed conjun ct iva; dilated an d sluggish pu pil; sign s of toxem ia in clu ding h igh -grade fever, decreased level of con sciou sn ess, n au sea, an d vom it ing.

72 Color Atlas of Ophthalm ology

A

B C

Fig. 3.2 (A) Ten-year-old boy with orbital abscess. (B) Magnetic resonance imaging (MRI) of an orbital abscess. (C) MRI, coronal section.

Differential Diagnosis (Table 3.1)

Carot id cavernous fist ula: Spon tan eous or post t rau m at ic, bru it on au scult at ion

3 Orbital Infections, Inam m ation, and Neoplasm s 73

ESR, erythrocyte sedim entation rate; WBC, white blood cell count

Management

Th e pat ien t m u st be adm it ted to th e h ospit al. Gram st ain ing an d cult u re of any open w ou n d an d disch arge sh ou ld be don e at th e earliest oppor t u n it y along w ith com plete an d differen t ial blood cou n t an d blood cu lt ures. Mu corm ycosis m u st be kept in m in d, especially in diabet ic an d im m u n osu ppressed p at ien ts. Lum bar pun ct u re for suspected m en ingit is is perform ed un der a physician’s su per vision . Neu rologic opin ion sh ould be un der t aken if th e gen eral con dit ion of th e pat ien t dict ates th e sam e. In t raven ous an t ibiot ics su ch as ceft riaxon e plu s van com ycin , or van com ycin plus gen tam icin , or van com ycin plu s clin dam ycin w ith or w ith ou t m et ron idazole are given in it ially follow ed by oral an t ibodies for 7 to 14 days. Hot fom en t at ion is applied fou r to five t im es a day. Local an t ibiot ics in clude polym yxin w ith bacit racin oin t m en t . For corn eal exposu re, NSAIDs h elp com bat pain an d in - flam m at ion .

Mon itor for th e follow ing w arn ing sign s:

Dilated pu pils

Marked oph th alm oplegia

Loss of vision

Relat ive afferen t p upillar y defect

Papilledem a

Perivasculit is

Violaceou s lids

Explorat ion of th e w ou n d is in dicated if th e pat ien t is un respon sive to an t ibiot ics, vision is decreasing, orbital abscess is presen t , an d a diagn ost ic biopsy is n eeded . It is im port an t to drain th e orbit al abscess as w ell as th e in fected sin u ses. Th e follow ing oral an t ibiot ics are given on ly after th e con dit ion im proves sign ifican tly: am oxicillin -clavulan ic acid, or cefaclor, or cot rim oxazole, or er yth rom ycin , clin da- m ycin , am oxicillin -cloxacillin w ith or w ith out m et ron idazole.

74 Color Atlas of Ophthalm ology

Thyroid-Related Ophthalmopathy

Graves’ disease or diffuse toxic goiter, is an au toim m u n e process th at in clu des on e or m ore of th e follow ing: hyper thyroidism , oph th alm opathy, an d in filt rat ive der- m opathy.

Presentation

Graves’ disease u sually occu rs w ith hyper thyroidism , but n orm al thyroid fu n ct ion can also be n ot iced . It is five t im es m ore com m on in fem ales (Fig. 3.3A,B). Th ere are t w o t ypes according to level of severit y:

1. Noninfilt rat ive (m ild): Min im al in flam m ator y react ion leading to m ild sym p - tom s an d sign s.

2. Infilt rat ive (severe): Th is t ype h as a m ore fu lm in an t course w ith in flam m a- t ion , in filt rat ion , an d scarring. Th ese pat ien t s h ave ch em osis, proptosis, corn eal exposu re, m yosit is, an d en largem en t of m uscles. It ult im ately leads to corn eal exposu re, rest ricted m ovem en t s, an d diplopia.

Werner classification reflects the severit y of the ophthalm opathy and is well know n by the acronym of NO SPECS (as described below ). Each grade is further subdivided as 0 to 4 and a to c:

Grade 0: No sign s or sym ptom s

Grade 1: On ly sign s (lid ret ract ion )

Grade 2: Soft t issu e involvem en t (e.g., ch em osis)

Grade 3: Proptosis (m in im um )

Grade 4: Ext raocu lar m u scle involvem en t

Grade 5: Corn eal involvem en t

Grade 6: Sigh t loss

Th ere are variou s sign s in thyroid eye disease, w h ich go by th e discoverers’ n am es (Fig. 3.3C,D,E) (Table 3.2).

A

B

Fig. 3.3 (A) Thirt y-five -year-old woman with dysthyroid orbitopathy with lid retraction. (B) Lid lags behind when patient looks down.

3 Orbital Infections, Inam m ation, and Neoplasm s 75

C

D

E

Fig. 3.3 (Continued) (C) Dysthyroid orbitopathy. (D) Com - puted tomographic (CT) scan, extraocular muscle enlarge - ment sparing muscle tendons. (E) Coronal section CT scan.

76 Color Atlas of Ophthalm ology

Table 3.2 Ophthalmic

Most important signs

Von Graefe: Upper lid lag on downgaze

Dalrymple: Upper eye lid retraction Upper eyelid signs

Von Graefe: Upper lid lag on downgaze

Dalrymple: Upper eyelid retraction

Boston: Uneven jerky movement of upper lid on inferior movement

Jellinek: Abnormal pigmentation of upper lid

Riesman: Bruit over eyelid

Snellen sign: Bruit over the eye Eye movement signs

Ballet: Paralysis of one or more extraocular muscles (EOM)

Möbius: cient convergence

Wilder: Jerking of eyes on movement from abduction to adduction Blinking signs

Pochin: Reduced amplitude of blinking

Stellwag: Incomplete or infrequent blinking

Upper eyelids lag on downgaze th sign: Lower eyelid lag on upgaze

Conjunctival signs

Goldzieher: Deep injection of temporal conjunctival vessels

Differential Diagnosis

Orbital pseudotum or, cavernous sinus throm bosis, orbital cellulitis (see Table 3.1)

Management

Man agem ent opt ion s for thyroid eye disease in clude obser vat ion , con ser vat ive in - ter ven tion s, oral cort icosteroids, injected cor ticosteroids, extern al-beam radioth erapy, an d surger y. Obser vat ion an d con ser vative m easures are appropriate for m ild conjun ctival inject ion an d ch em osis w ith n orm al corn ea and opt ic n er ve fu nction . Advice to th e pat ien t in cludes sleeping w ith th e h ead of the bed elevated an d avoid - ing salt or m on osodium glutam ate to m in im ize fluid reten tion . Sunglasses are recom m en ded to m in im ize ph otoph obia. Nonpreser ved art ificial tears h elp decrease th e ocular irritat ion . Glaucom a m edicat ion s sh ould be used on ly in patien ts w ith ver y h igh in t raocular pressures and a fam ily h istor y of glaucom a. Surgical in terven t ion sh ould be perform ed in a stepped fash ion : decom pression first, strabism us surger y second, an d eyelid surger y last . Pat ien ts should stop sm oking an d avoid secon dh an d sm oke to lim it th e autoim m un e exacerbat ion of thyroid eye disease.

Man agem en t of t hyroid orbitop at hy m u st t ake in to accou n t w h eth er th e disease is act ive or ch ron ic an d t h e d egree to w h ich th e m an ifest at ion s im p act t h e

3 Orbital Infections, Inam m ation, and Neoplasm s 77

p at ien t ’s daily life or t h reaten sigh t . For exam p le, m ild eyelid ret ract ion w ith m in im al or n o d r y eye sym ptom s m igh t be m an aged w it h con ser vat ive lu brica- t ion p r ior to elect ive eyelid su rger y on ce th e p at ien t h as st abilized . At t h e op - p osite en d of t h e sp ect r u m is t h e p at ien t p resen t ing w it h acu te opt ic n eu rop a- thy resu lt in g from ap ical crow d in g th at requ ires u rgen t m ed ical an d /or su rgical m an agem en t .

Pat ien ts sh ould be m an aged in close correspon den ce w ith an en docrin ologist , w h o m ay elect to t reat system ic hyp erthyroidism via ph arm acological supp ression , surgical resect ion , or radioact ive iodin e. Recen t st udies suggest a ben efit to steroid th erapy in th e peri-in ter ven t ion al period in m in im izing th e progression of orbitopathy.

Gen eral t reat m en t opt ion s for th e m an agem en t of sequ elae of thyroid orbitopathy in clude ph arm acological th erapy, radiat ion th erapy, an d surgical in ter ven t ion . Mild cases w ith m in im al ocular irritat ion or sym ptom at ic diplopia m ay in it ially be m an aged con ser vat ively w ith lu bricat ing eyedrops an d oin t m en t , n oct u rn al tap - ing, an d Fresn el prism s.

Radioth erapy, gen erally 20 Gy delivered in 10 fract ion s over 2 w eeks, h as long been used as t reat m en t for th e orbit al m an ifestat ion s of thyroid disease as h as been sh ow n to be of ben efit in im provem en t of m ot ilit y. How ever, a recen t prospect ive, ran dom ized st u dy by Gorm an et al dem on st rated n o ben eficial th erapeu - t ic effect of radioth erapy in m oderate, sym ptom at ic thyroid orbitopathy.

Surgical m an agem en t can be divided in to elect ive an d u rgen t in ter ven t ion s. Elect ive su rger y sh ould proceed in th e order of orbit al decom pression , st rabism us su rger y, an d fin ally lid surger y, becau se each in ter ven t ion as listed can in fluen ce th e ou tcom e of th e su bsequen t in ter ven t ion s. Th ere are a m u lt it u de of ap proach es to orbit al decom pression en com passing on e to all w alls, w ith or w ith ou t en doscopic an d t ran sn asal exposure an d w ith or w ith ou t orbit al fat decom pression . St rabism u s su rger y sh ould be u t ilized to m axim ize th e field of bin ocu lar vision an d gen erally in cludes recession of m uscles on adjust able sut u res. Eyelid procedu res in clu de bleph arop last y w ith or w ith ou t rem oval of orbital fat , release of u p - per lid ret ract ion via levator an d or Mu ller m u scle recession , an d repair of low er lid ret ract ion w ith spacer grafts.

In dicat ion s for u rgen t su rgical in ter ven t ion in clude opt ic n europathy from apical crow ding an d corn eal u lcerat ion secon dar y to exposu re. Th e m an agem en t of th ese con dit ion s can in clude m any of th e procedures outlin ed earlier in addit ion to urgen t m edical m an agem en t w ith pu lse cor t icosteroids, ph arm acoth erapy, an d orbit al irradiat ion .

Cor t icosteroids can provide sh ort-term relief for sym ptom s an d sign s of thyroid eye disease, but hyper thyroid pat ien t s can suffer sign ifican t m ood sw ings. In addi- t ion , tapering th e steroids often result s in rebou n d in flam m at ion at least as severe as th e origin al presen tat ion . Oth er im m u n osu ppressive agen ts h ave been used in th e t reat m en t of thyroid orbitopathy as steroid sparing agen ts, in cluding cyclosporin e, cytoxan , m eth ot rexate, an d azath ioprin e. Som e favor com bin at ion th erapy w ith cyclosporin e an d cor t icosteroids. Oth er t reat m en t m odalit ies in clude som a- tost at in an alogu es (oct reot ide), plasm aph eresis, an d in t raven ou s im m u n oglobulin th erapy.

Orbital Inflammatory Pseudotumor

Orbit al in flam m ator y p seu d ot u m or (OIP) con sist s of a sp ect r u m of n ongran u lo- m atou s in flam m ator y con d it ion s of th e orbit , w ith n o kn ow n et iology or system ic associat ion s, th at p rodu ce p roptosis du e to a n on n eop last ic in flam m ator y m ass in th e orbit . (Note: Som e au th ors classify Tolosa-Hu n t syn drom e as a su bt yp e of OIP.)

78 Color Atlas of Ophthalm ology

Fig. 3.4 Middle -aged woman with left eye pseudotum or.

Presentation

OIP presents w ith abrupt onset of pain, proptosis (unilateral), conjunctival chem osis, epibulbar injection, visual loss, diplopia, and restricted ocular m ovem ents (Fig. 3.4). According to the different tissues involved, it is classified as one of the follow ing:

Myosit is

Dacr yoaden it is

Periopt ic n eurit is

Posterior sclerit is or ten on it is

It s clin ical cou rse is variable, an d it m ay be regressed spon tan eously w ith out any t reat m en t , or it m ay h ave prolonged in flam m at ion or in term it ten t act ivit y. A prolonged course m ay result in a frozen orbit secon dar y to fibrosis. Bilateral involve- m en t is rare.

Differential Diagnosis

Graves oph th alm opathy, orbital cellulit is, leu kem ia, cavern ou s sin us th rom bosis, rh abdom yosarcom a (see Table 3.1)

Management

Com puted tom ograph ic (CT) scan s an d m agn et ic reson an ce im aging (MRI) are essen t ial in th e w orkup of susp ected OIP. In flam m ator y sign s predom in ate an d m ay in clu de involvem en t of th e ext raocu lar m u scles (EOMs), orbit al fat , lacrim al glan d, ch oroid, an d sclera. Orbit al ult rasoun d m ay be of use in dem on st rat ing th icken ing of th e posterior Ten on capsu le an d in dist ingu ish ing th e m yosit is of OIP from EOM involvem en t in thyroid orbitopathy becau se th e m uscu lar ten don s w ill classically be involved in OIP an d spared in thyroid disease.

Th e CT orbit sh ow s ext raocu lar m uscle th icken ing involving ten din ous in ser- t ion . In flam m at ion of th e ret robu lbar fat pad an d con t rast en h an cem en t of th e sclera du e to ten don it is m ay produ ce a T sign or ring sign . Orbit al ult rasou n d sh ow s th icken ing of th e posterior Ten on capsule along w ith m uscle belly th icken - ing (un like thyroid related orbitop athy, w h ich t ypically spares th e ten don s).

System ic steroids (60 to 80 m g/day) are given . Rapid respon se is p ath ogn om on - ic. Taper slow ly over m on th s to avoid recu rren ce. Pu lsed in t raven ou s steroids are given in severe vision -th reaten ing cases. Radioth erapy is recom m en ded in steroid - resist an t cases. Ch em oth erapeut ic agen ts su ch as cycloph osph am ide, m eth ot rexate, an d cyclosporin e are used for cases resist an t to steroids an d radioth erapy an d in pat ien t s in toleran t to steroids.

3 Orbital Infections, Inam m ation, and Neoplasm s 79

Orbital Lymphoma

Orbit al lym ph om a is a low -grade m align an cy ch aracterized by proliferat ion of m on oclon al B cells (n on -Hodgkin disease), w h ich arises in lym ph n odes or in ex- t ran odal sites su ch as th e orbit .

Presentation

Th e disease presen t s bet w een th e ages of 50 an d 80 years w ith involvem en t of any par t of th e orbit . Bilateral involvem en t is rare. It occu rs rarely in ch ildren . Most orbit al lym ph om as are low grade. Malign an t lym ph om as can produ ce a palpable m ass th at m ay be presen t in th e an terior orbit . On e can h ave pain less progressive proptosis, accom pan ied by vision loss, occasion al diplopia, lid edem a, ptosis, an d lacrim al glan d involvem en t . A salm on -colored conju n ct ival t um or is ch aracterist ic (Fig. 3.5A,B,C,D).

A

80 Color Atlas of Ophthalm ology

Differential Diagnosis

Metastasis, react ive lym ph oid hyperplasia, pseudot um or, sarcoidosis

Management

Com pu ted tom ograph ic scan sh ow s a w ell-defin ed m ass, located m ostly in th e an - terior-su perior lateral orbit , w h ich m olds to en com pass adjacen t st ru ct u res. Th e lacrim al glan d is frequen tly involved . Ult rason ography sh ow s variable sh ape an d borders of th e lesion , w h ich h as low to m edium in tern al reflect ivit y.

Radioth erapy (2500 to 3000 cGy) is th e t reat m en t of ch oice for less w elldifferen t iated lesion s. Ch em oth erapy can also be t ried . A w ell-differen t iated lesion w ith ou t system ic involvem en t can be obser ved .Visu al progn osis is excellen t if th e disease is con fin ed to th e orbit .

Other Orbital Neoplasms

Dermoid Cyst

Derm oid cyst is a developm en t al, slow -grow ing ch oristom a (t u m ors w ith h istologically n orm al cells in an abn orm al locat ion ), lin ed w ith st rat ified squam ou s epith eliu m an d filled w ith kerat in ized m aterial an d/or lipid . Most of th ese cyst s are located in th e eyelid an d orbit , represen t ing th e single m ost com m on cause of periorbital n eoplasm in ch ildren . Th ey develop because of sequ est rat ion of th e su rface ectoderm pin ch ed off at th e bon e su t u re lin es or along th e lin es of em br y- on ic closure. Th e cyst s are lin ed w ith epiderm is w ith derm al appen dages su ch as h air follicles an d sebaceous glan ds in th e w all.

Presentation

Derm oids are classified according to th e an atom ical site of presen tat ion :

Superficial derm oids (Fig. 3.6A)

In fron t of th e orbital sept um an d su perotem poral or su peron asal quadran ts

Presen t at ion in in fan cy an d ch ildh ood

Palpable, firm , un ilateral, localized m ass, u sually asym ptom at ic, m ay be m o- bile or fixed to th e un derlying st ruct ures an d free from th e overlying skin

Deep derm oids (Fig. 3.6B)

Posterior to th e orbital sept um , associated w ith bony sut u res in th e orbit but m ay exten d across th e bon es in th e fron t al sin us, tem poral fossa, or cra- n iu m

Presen t in adolescen ce, m ay be seen in ch ildren an d adu lt s

Proptosis, ocu lar displacem en t an d bony defect , m ot ilit y rest rict ion , decreased vision . Spon t an eou s rupt u re produces severe orbit al in flam m at ion .