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 81

Differential Diagnosis

Cavern ou s h em angiom a, m u cocele, opt ic n er ve gliom a, m en ingiom a, n eurilem - m om a

Management

Superficial derm oids

CT scan of the orbit: Rou n d, w ell-defin ed lesion s w ith en h an cing rim an d a lucen t a cen ter, w h ich m ay con t ain calcium . Th ere m ay be a w ell-cort icated bon e defect .

Echography: A w ell-defin ed lesion w ith m edium to h igh in tern al reflect ivit y, w ith an irregular acoust ic st r uct ure; usu ally sh ow s som e com pressibilit y

Com plete surgical excision: In on e piece

Incom plete excision or capsular rupt ure: May lead to recurren ce w ith in filt ra-

t ion

Deep derm oids

CT scan of the orbit: Well-defin ed lesion s w ith an en h an cing rim . Th ey m ay con tain areas of calcificat ion . Th e cen t ral lu m en is n on en h an cing, of variable

Capillary Hemangioma

Capillar y h em angiom a is a p rim ar y, u n ilateral, ben ign h am ar tom a of t igh tly packed cap illaries, ap paren t at birth or w ith in th e first 8 w eeks of life, st raw berr y red to pu rp le. Most regress com pletely w ith in 7 years of age. Th ey are visible on th e surface bu t m ay lie deep in th e orbit . It is m ore com m on ly seen in th e supero- n asal qu adran t of th e u pper eyelid .

Presentation

More com m on in girls. Involvem en t of sup erficial st ru ct u res (derm is) results in a st raw berr y m ark (st raw berr y n evus), single or m u lt iple, u sually elevated . Su ch pat ien t s m ay presen t w ith ptosis, som et im es associated w ith ast igm at ism an d am blyopia. Involvem en t of th e deep par ts (an d an terior orbit) appears as a blu ish m ass w ith a spongy text u re. W h en th e ch ild cries or st rain s, th e m ass becom es m ore p rom in en t an d deep en s in color. On exam in at ion , it is a circu m scribed, soft red m ass w ith a m u lt in odular surface. Large feeding vessels are seen an d can be th e source of bleeding (Fig. 3.7).

82 Color Atlas of Ophthalm ology

A

B

C

Fig. 3.6 (A) Young girl with a superficial derm oid. (B) A 4-year-old child with a dermoid involving the lateral canthal area. (C) A 9-year-old girl with a deep dermoid in the lateral orbit pushing the left eyeball medially.

3 Orbital Infections, Inam m ation, and Neoplasm s 83

D

E

84 Color Atlas of Ophthalm ology

Fig. 3.7 Eight-month-old boy with a capillary hemangioma.

Differential Diagnosis

Nevus flam m eus (darker, does n ot blan ch w ith pressu re), derm oid cyst , en cep h a- locele, lym ph angiom a, in fect ion , n euroblastom a (Table 3.3)

Management

Pat ien t m u st be referred to a pediat rician for w orkup of system ic associat ion (s) like h igh -out put cardiac failu re, Kasabach -Merrit t syn drom e (an em ia, th rom bocytopen ia, low levels of coagulat ing factors due to th eir sequ est rat ion in th e lesion ), or Maffucci syn drom e (en doch ordom atas an d skin h em angiom as). Com - plete oph th alm ologic exam in at ion m u st be don e to rule ou t poten t ial secon dar y even t s, n am ely, am blyopia, com pressive opt ic n eu ropathy, an d corn eal exposu re. Orbit al ult rasoun d suggest s a poorly ou tlin ed lesion , irregu lar in sh ape, h igh in - tern al reflect ivit y, an d an irregu lar acoust ic st ruct ure w ith variable sou n d at ten u - at ion . CT scan sh ow s con t rast en h an cem en t an d defin es th e exten t of th e lesion . Deeper lesion s are w ell defin ed w ith m oderate to in ten se en h an cem en t . On MRI, th e lesion sh ow s h om ogen eous an d h eterogen eou s sign als, being hypoin ten se on T1 an d hyperin ten se on T2 w edging. Flow voids appear as hyperin ten se region s. Gadolin iu m en h an ces th e lesion m oderately.

Obser vat ion is pract iced in m ost of th e cases becau se involu t ion usu ally occu rs in th e follow ing con dit ion s:

Superficial condit ions: Severe cosm et ic deform it y an d deprivat ion am blyopia are th e m ajor in dicat ion s of in ter ven t ion . Th e t reat m en t opt ion s in clu de in t ralesion al (40 m g/m L t riam cin olon e plu s 6 m g/m L betam eth ason e) or system ic (predn isolon e 1 to 2 m g/kg/day) steroid . Oth er opt ion s in clude radioth erapy, yellow -dye laser, an d topical cort icosteroids. Su rger y sh ou ld be reser ved for sm all, circum scribed lesion s.

Deep condit ions: Large lesion s or am blyopia u sually w arran t t reat m en t w ith lo-

cal radioth erapy (500 cGy) or system ic or local cor t icosteroids. Surger y m ay be at tem pted in sm all, circu m scribed lesion s. Progn osis is good for vision an d for life.

3 Orbital Infections, Inam m ation, and Neoplasm s 85

Table 3.3 Vascular Lesions

Lymphangioma

Lym ph angiom a is a rare vascu lar h am ar tom a of lym ph at ic ch an n els th at is h e- m odyn am ically isolated from th e vascular system . Occu rring predom in an tly in ch ildren an d teen agers (m ost frequ en tly in th e first decade of life), th e size of th e lesion fluct uates w ith post ure an d Valsalva m an euver an d w ith u pper respirator y t ract in fect ion s.

Presentation

Su perficial lesion s occu r in th e conjun ct iva or lid an d are visible as cyst ic spaces w ith clear flu id par t ially filled w ith blood . Deep lym ph angiom atous lesion s classically presen t w ith acute ou t set of pain ful proptosis result ing from spon t an eou s h em orrh age w ith in th e orbit . Su ch lesion s are called ch ocolate cysts. Th e t u m or m ass m ay com press th e globe or opt ic n er ve, causing visu al loss, refract ive errors, secon dar y glau com a, congest ion of th e opt ic n er ve, an d visu al field defects (Fig. 3.8A,B).

86 Color Atlas of Ophthalm ology

A

B

Fig. 3.8 (A) Ten-year-old boy with lymphangioma involving the lower nasal orbit. (B) Coronal section, computed tom ographic scan.

Differential Diagnosis

Opt ic n er ve gliom a, plexiform n eurofibrom a, capillar y h em angiom a, pseudot u - m or

Management

Th e orbital lesion is seen as low -den sit y cyst ic, in t racon al an d ext racon al m asses, w ith variable en h an cem en t on m agn et ic reson an ce im aging. With T1 w edging

3 Orbital Infections, Inam m ation, and Neoplasm s 87

th ey are hypoin ten se, w h ereas T2 w edging respon se is variable, dep en ding on th e state of h em oglobin degen erat ion . Angiography sh ow s n o vascular com pon en t .

Man agem en t of lym ph angiom as is ch allenging. Radiat ion an d system ic steroids sh ow lim ited sen sit ivit y. Com p lete su rgical excision is ver y difficu lt because of th e in filt rat ive n at u re of th e t u m or. If acu te h em orrh age cau ses sym ptom s, CO2 laser or con tact n eodym ium :yt t rium -alu m in um -garn et can be t ried for h om eostasis an d obliterat ion of t um or as an altern at ive to evacu at ion , p ar t ial resect ion , or ligat ion . Am blyopia is com m on an d is m ostly from recu rren t h em orrh age or globe com - pression .

Rhabdomyosarcoma

Rh abdom yosarcom a is th e m ost com m on prim ar y m align an t orbit al t um or in ch ildren (70%arise w ith in th e first decade of life). Th is soft t issue m esen chym al t um or accou n ts for u p to 4% of all ch ildh ood m align an cies. It arises from pleuripoten t m esen chym al precu rsors th at n orm ally differen t iate in to st riated m uscle cells.

Presentation

Presen tat ion is u sually in th e first decade of life w ith a rapidly progressive proptosis, m ore com m on ly in boys. It frequ en tly sh ow s a m ass in th e u pper par t of th e orbit , ptosis, an d eyelid edem a. Th e diagn osis is con firm ed by biopsy. It can be grouped in to four categories: em br yon al, alveolar, pleom orph ic, an d bot yroid in th e orbit . Th e m ost com m on h istological varian t is em br yon al follow ed by th e alveolar t ype (Fig. 3.9A,B,C,D).

Differential Diagnosis

Orbit al cellu lit is, pseu dot u m or, lym ph angiom a, m etastat ic n eu roblastom a, ru p - t u red derm oid cyst

Management

In th e past , p at ien ts w ith orbit al rh abdom yosarcom as u n der w en t orbital exen - terat ion . Because of th e m align an t n at ure of th e t um or, it w as th ough t th at radical resect ion p rovided th e best ch an ce for su r vival. Despite th ese m easures, m ort alit y rem ain ed as h igh as 70%. Over th e past 30 years, w ith a com bin at ion of surger y, radiat ion , an d ch em oth erapy, sur vival h as approach ed 90%.

A staging classificat ion w as proposed by th e In tergroup Rh abdom yosarcom a St u dy grou p in 1972. Com plete resect ion of localized disease is categorized as group I. In gen eral, m icroscopic residual disease or lym ph n ode involvem en t is categorized as grou p II. Grou p III in clu des gross residu al disease or in com plete resec- t ion . Group IV disease in clu des cases w ith m et astasis at presen t at ion . Th e st age of disease is depen den t n ot on ly on th e exten t of th e t u m or but largely on th e exten t of resect ion . Th e sam e t u m or can be a group I or II versu s a grou p III depen ding on w h eth er th e su rgeon perform ed an excision or in cision al biopsy, respect ively. Th e recom m en ded regim en of radiat ion an d ch em oth erapy is based on th e stage of disease an d is sum m arized follow ing h ere.

Becau se of rh abdom yosarcom as’ sen sit ivit y to ch em oth erapy an d radiat ion , an in cision al biopsy follow ed by eith er ch em oth erapy, radiat ion , or both is p referred by m ost oph th alm ologist s. Th is approach is especially pruden t w ith large t um ors or t um ors in w h ich an excision al biopsy w ould likely h arm th e opt ic n er ve, ext raocu lar m uscles, or oth er im port an t orbital st ruct ures. Th e decision is m ore difficu lt for th ose sm aller, m ore an terior t um ors w h ere com plete excision w ith ou t en dangering oth er vit al orbit al st ru ct u res is feasible. Most orbit al rh abdom yosar-

88 Color Atlas of Ophthalm ology

Fig. 3.9 (A) Three -year-old boy with rhabdomyosarcoma.

(B) Computed tomographic (CT) scan, sagit tal section.

com as are located su peron asally an d in th e ext racon al space w h ere excision w ould n ot violate th e opt ic n er ve or ext raocular m u scles. Su ch an app roach m ay perm it low er doses of radiat ion .

Judiciou s review of th e CT an d MRI scan s is crit ical for surgical plan n ing. In cision s directly overlying th e t um or are th e preferred approach to biopsy. For exam - ple, m ore p osterior t um ors are best ap proach ed via a cu tan eou s in cision th rough th e lid, w h ereas m ore an terior t u m ors th at are visible in th e conju n ct ival forn ices m ay be ap proach ed via a t ran sforn iceal approach . For excision al biopsies, care sh ould be taken to con t ain th e t u m or in it s pseudocapsu le an d n ot to violate th e periosteu m to preser ve th e n at u ral barrier to spread ou tside th e orbit .

Irradiat ion for orbit al rh abdom yosarcom as plays a secon dar y role to ch em oth erapy in m an agem en t . Conven t ion al fract ion ated doses totaling 4000 to 5000 cGy are u su ally su fficien t to con t rol t um or recurren ce. How ever, at th ese doses, ocular com plicat ion s of orbit al irradiat ion , in clu ding radiat ion ret in op athy, cat aract , dr y eyes, an d radiat ion -associated keratopathy, are relat ively com m on .

3 Orbital Infections, Inam m ation, and Neoplasm s 89

Fig. 3.9 (Continued) (C) CT scan, coronal section. (D) CT scan showing involvement of the eyeball.

With a com bin at ion of su rger y, ch em oth erapy, an d radiat ion , it is p ossible to con t rol th e t u m or an d salvage th e eye in ~90% of cases of orbit al rh abdom yosarcom a.

Supplem en t al ch em oth erapy h as su bstan t ially im proved su r vival rates of pa- t ien t s w ith orbit al rh abdom yosarcom as. Pat ien ts w h o u n dergo surger y, radiat ion , an d ch em oth erapy for rh abdom yosarcom a in cluding but n ot lim ited to th e orbit h ave been sh ow n to h ave a 2-year disease-free su r vival rate of 82%, com pared w ith 53%in th ose w h o un dergo su rger y an d radiat ion alon e.

Vin crist in e an d act in om ycin D h ave been th e m ain stays of ch em oth erap eut ic agen ts em ployed in cases of orbit al rh abdom yosarcom a. New er agen ts such as ifosfam ide an d etoposide h ave been sh ow n to produce a favorable respon se.

90 Color Atlas of Ophthalm ology

Cavernous Hemangioma

Cavern ou s h em angiom a is usually seen in m iddle-aged w om en an d is th e m ost com m on ben ign in t raorbit al t u m or in adult s.

Presentation

Th e t u m or is u n ilateral, solit ar y, an d t ypically located in th e in t racon al area. Proptosis is of th e axial t ype. Th ere is a predilect ion for m iddle-aged w om en , an d th e t u m or m ay grow faster during pregn an cy. Th e t u m or m ay be associated w ith opt ic disk edem a an d ret in al folds (st riae). Th e vision m ay decrease by on e or t w o lin es. Th ere m ay be rest ricted m ovem en t s in ext rem e fields of gaze (Fig. 3.10A–C).

Differential Diagnosis

Oth er in t raorbit al m ass lesion s su ch as derm oid cyst , lym ph om a, sch w an n om a

Management

Com puted tom ograph ic scan con firm s th e diagn osis. Most cavern ou s h em angio- m as can be obser ved . If su rger y is don e, th e t um or is seen to be a w ell-circum - scribed, pu rp le, en capsu lated lesion w ith dist in ct vessels on it s surface.

Carotid Cavernous Fistula

Orbit al vascular abn orm alit ies are a grou p of orbit al disorders, congen it al or acqu ired, arising from a variet y of un derlying con dit ion s. Arterioven ous m alform a- t ion s, h aving feeder vessels from both in tern al an d extern al carot id circulat ion s, m ostly occur after t rau m a (m ales, 15to 30-year age range) bu t m ay also arise spon tan eously (fem ales, 30to 60-year age range). Carot id cavern ou s fist u las (CCFs) occur m ostly after basal skull fract ures or pen et rat ing orbit al t rau m a an d can occu r spon tan eously in person s w ith system ic hyperten sion . Th e h igh -flow CCFs arise w h en th e in tern al carot id ar ter y develops a defect w ith in th e cavern - ous sin us, w h ereas low -flow CCFs develop from th e com m u n icat ion bet w een th e m en ingeal bran ch es of th e in tern al carot id ar ter y an d th e cavern ou s sin u s.

Presentation

Th e pat ien t n ot ices a sw ish ing n oise in th e h ead th at is syn ch ron ou s w ith th e pulse. Becau se of th e proxim it y of th e ocu lar m otor n er ves to th e cavern ous sin u s, im paired ocular m ot ilit y an d diplopia are early fin dings. Proptosis, lid an d orbital edem a, an d dilated an d tor t u ou s conjun ct ival an d episcleral vein s develop later. Elevated episcleral ven ou s pressu re leads to ocular hyper ten sion .

High-flow CCF: Ch em osis, corkscrew dilatat ion of th e epibulbar vessels, orbital edem a, proptosis, pu lsat ile exoph th alm os, audible bru it , secon dar y glau com a, ret in al vascu lar dilatat ion , papilledem a, rapid afferen t papillar y defect , decreased vision , occasion al n er ve palsies (III–VI are m ost com m on )

Low -flow CCF: Ch em osis, in creased episcleral ven ous pressure, ven ous dilata- t ion (Fig. 3.11A,B,C).