III. Adult Orbit
Infections
Inflammation
Orbital Trauma
Tumors
Enucleation

Dacryoadenitis

infection rare, most commonly mumps, gonococcus, HZO, trachoma, mononucleosis and actinomyces

Preseptal Cellulitis

inflam and infxn confined to lids and periorbita anterior to septum

usually 2^o to trauma or skin infxn in kids and adults

EOM’s, pupils, Va, Hertels, all OK

teens and adults can be closely followed as outpt w/ PO abx (Augmentin) but if worsening then CT and admit for IV abx

under 5 yo may have bacteremia, esp from H flu (violaceous erythema w/marked lid swelling are classic) otitis media or pneumonia, more severe dz, need IV 3rd gen cephalosporin abx after blood cx taken

surgical drainage of abscess may be necessary; do not violate septum and cause orb cellulits

Orbital Cellulitis

infxn posterior to orbital septum

90% from extension of acute or chronic bacterial sinusitis, reaminder s/p trauma or surgery or 2^o to extension from other orbital or periorbital infxn, or endogenous w/septic embolization

fever, proptosis, restriction of EOM’s, pain on globe movement

decreased Va, APD, prolonged high IOP can be indications for aggressive management to prevent orbital apex syndrome or cavernous sinus thrombosis

CT of orbit and sinuses to confirm sinus disease, r/o mass, r/o orbital foreign body if h/o trauma (even remote), r/o orbital or subperiosteal abscess which will require surgical drainage

blood culture then broad spectrum IV antibiotics to cover gram + cocci, H. influenzae (although less prevalent in kids 2^o to immunization), anaerobes, typically nafcillin and 3rd generation cephalosporin; ID consult if necessary; kids more often single organism

progression of infxn or no daily improvement on appropriate abx can mean abscess: repeat CT prn and drain w/concomitant sinus drainage prn

cavernous sinus thrombosis: rapid progression of proptosis and neurologic signs of intracranial dysfunction; may lead to meningitis; get neurosurgery consult

Thyroid Eye Disease; Thyroid-Associated Orbitopathy (TAO)

also called Grave’s eye disease, etc.

most common cause of unilateral and bilateral proptosis in adults

typically F (>M by 4X ) between 25 to 50 years old, but more severe in older patients and men

majority have history of thyroid problems with idiopathic hyperthyroidism the most common; up to 15% of women and 30% of men have no thyroid abnormality

acute episodes may occur up to three times during the course of the disease and last from 3 months to one year, with residual cicatricial changes in EOM’s and orb fat leaving resid porptosis and diplopia

Signs

this is a clinical diagnosis

EOMs are targets with IR, MR, LR, and SR in decreasing order being affected

upper and lower lid retraction (increased with fixation) is very specific and common

older age

less proptosis

increased MR size w/horizontal limitations

Exam

hx, sx, thyroid meds and length of time on them

optic nerve function: Va, color vision, pupils for APD, optic nerve appearance, HVF/tangent screen

lid, globe evaluation: ptosis workup, note retraction, measure sclera below, MRD2 (reflex to lower lid margin), tear strips, SLE including K stain, IOP straight, in upgaze

orbit, EOM evaluation: diplopia fields, Hertel

check for goiter

Labs

TSH, T4

consider TRH stimulation test

10% will be hypothyroid

if nl, consider repeat

CT

to confirm dx,examine orbital apex, EOMs; coronals most helpful

muscles tendons are spared (vs pseudotumor)

multiple muscle enlargement w/ IR, MR esp affected virtually diagnostic but rarely pseudotumor or amyloidosis can look similar

TAO associated with myasthenia gravis which can also simulate it, so consider chest CT for thymoma

may need MRI for optimal orbital apex eval

Orbitopathy Classification

Type I

most often in women

minimal orbital and EOM inflammation

symmetric proptosis and lid retraction common

muscles are enlarged due to edema rather than myositis, less diplopia and chemosis

may have considerable keratopathy

Type II

evenly divided among men and women

history of smoking

myositis, restrictive myopathy with diplopia, may be asymmetric, eye usually hypotropic, esotropic

orbital inflammation and compressive neuropathy

chemosis and vasc engorgement over muscles frequent

unilateral or asymmetric

Werner's Classification

NO SPECS, with each class in four grades 0-4, a, b, c: mild to severe

0 No S/S

1 Only signs (lid retraction)

2 Soft tissue involved (chemosis, grit, etc)

3 Proptosis (min <23, mod, marked >28)

4 EOM involved

5 Corneal involvement

6 Sight loss

Treatment

local protective

treat exposure with ointments, drops

consider patching at night

medical/ radiation

to decrease myositis, orbital edema, or optic nerve compression during acute phase, consider 80-100 mg of oral prednisone for 6-8 weeks or IV methyprednisolone

if can’t wean off steroids after 6-8 weeks, consider radiation of 1500-2000 rads (may complicate future surgery); cyclosporin

surgical

wait for stability for at least 6 months before strabismus or lid surgery

STOP smoking which worsens lid retraction

correct strabismus (recession of a restricted rectus muscle, often on adjustable suture preferable to resection of contralat muscle) before lid surgery, but after decompression

IR restriction can cause loss of lower lid retraction on downgaze or increased retraction w/eye fixation

recession of eyelid retractors and lateral permanent tarsorrhaphy (if needed) useful in cosmesis and to lessen exposure; one, two, or three wall orbital decompression for cosmesis or optic nerve compression

Hypothyroidism

myxedema (nonpitting) prominent in eyelids

loss of 1/3 of eyebrows, ?increased in COAG

treatment of thryoid abnl does not appear to affect orbitopathy for better or worse

Pseudotumor; Orbital Inflammatory Syndrome

nonspecific, idiopathic, benign inflammatory process w/pleomorphic inflammatory response, w/varying amounts of fibrosis

peak 40-50 yo, M=F, can affect kids and adults

mimics cellulitis with vision loss (if o.n. or post. sclera involved) so pts present with acute eye pain, restricted movement, and proptosis, often conj injxn and chemosis

Anterior Orbit Form

severe eye pain

may have posterior sceritis with greater ocular inflammation, pain to temples

acute dacryoadenitis

pain superotemporally, swelling, redness, S shaped lid

palpate gland for tenderness

may involve LR

no proptosis or vision loss

myositis

one or two muscle involvement only (thyroid much less likely if + myositis but no IR or MR), with pain on movement, positive forced ductions

proptosis with resistance to ballotment

CT: muscle tendon involved & thickened (vs.TAO)

posterior pattern

with signs/symptoms of orbital apex syndrome

Subacute, Chronic

increased fibrous componant, less inflammation

insidious onset over weeks and months

PPLOV, diplopia, proptosis

chronic sclerosing variant usually with posterior orbital mass and slow onset of symptoms, decompression won’t help b/c mass is non-maleable and won’t fill the increased orbital volume

pain is variable, fibrosis common

Pseudotumor in Children

1/3 bilateral

1/2 have HA, fever, vomiting, abdominal pain, lethargy

can have eosinophilia, high ESR, + ANA

Tolosa Hunt

orbital inflammatory variant restricted to superior orbital fissure and cavernous sinus

Diagnosis

clinical picture as above

may look like cellulitis w/o sinus involvement or h/o trauma

CT:

ring sign of scleritis (tenonitis)

tendons inflamed

diffuse infiltrate with ragged, streaky edges

U/S:

RD, choroidals or thickening of sclera, or edematous Tenon’s

perineural and subTenon’s effusion causes the T sign at optic nerve junction

FANG:

patchy choroidal filling, punctate areas of leakage

CME

Path

pleomorphic cellular infiltrate w/ lymphs, plasma cells, eos w/ variable fibrosis

Treatment

steroids 80mg qd, 5 mg/week taper; PF if w/anterior inflam; acute cases respond rapidly

if no response then IV steroids

if still unresponsive by 2 weeks, orbit bx, XRT 13000 cGys (rads)

cyclophosphomide 200 mg/day

rarely decompression needed if o.n. compression is severe

Vasculitis

Temporal Arteritis

jaw claudication, wt loss, tender temp art, fever, high ESR

CRAO or AION

see neuro-op

Polyarteritis Nodosa

usually doesn’t cause orbital dz, more retinal and choroidal infarction

Wegener's Granulomatosis

small vessel necrotizing granulomatous vaculitis of any organ system including orbit

lesions of upper and lower respiratory tract, necrotizing glomerulonephritis

+ANCA (anti-neutrophil cytoplasmic antibodies)

25% have scleritis

Lethal Midline Granuloma

nebulous dz, relentless destruction of nose, paranasal sinuses, oral pharynx, palate

Blowout (Orbital Floor) Fracture

Exam

CT w/ axial, direct coronal views most helpful to note EOM entrapment, size of fx, other injury i.e. orbital heme (proptosis w/ high IOP), globe injury, CN II injury

plain films OK for dx fx only

most do not require surgery

early surgery for marked muscle restriction confirmed on CT, forced duction

should observe 1-2 weeks, oral steroids (prednisone 1 mg/kg/day w/ taper) to decrease swelling and fibrosis

antibiotics (Keflex) and nasal decongestants (Afrin), tell pt not to blow nose to decrease orbital emphysema

Surgery Indications

residual marked diplopia w/in 30^o of 1^o gaze 2^o to resrtiction (of IR)

large (50%) floor fx esp w/ large med wall fx b/c likely to get enoph

enophthalmos > 2 mm: usually there is initial proptosis 2^o to orb infl/edema which resolves; if + initial enoph or no prop, later surgery for enoph more likely

surgical repair easier within the first 2 weeks

surgical steps: usually inf fornix incision, elevate periorbita from orb floor, free tissues from fx, implant (silastic or miniplate depending on size) over floor defect

Medial Orbital Fracture

if indirect (blowout) extension of floor fx, no sx needed unless MR entrapped

lid/orbit emphysema common

direct naso-orbital fracture more serious, depressed nasal bridge; compl inlc cerebral/ocular damage, ant ethmoid art. damage w/ severe epistaxis, CSF rhinorrhea, traumatic telecanthus, needs miniplate stabilization

Zygomatic Fracture

tripod fx often has 4 zygoma breaks @ lat & inf orb rim, zyg arch, lat wall of max sinus

can involve orb floor

if displaced, can have cosmetic deformity, trismus (2^o to impingement on coronoid process of mandible)

Orbital Apex Fracture

often w/traumatic optic neuropathy (needs spinal cord dose IV steroids, maybe decompression w/in 5 days, see neuro-op), other fractures

look for CSF rhinorrhea, CC fistula

Orbital Roof Fracture

infrequent

may have intracranial lesions, CSF rhinorrhea, pneumocephalus

neurosurgery consult

Orbital Emphysema

if severe can cause CRAO, etc if loculated ball valve type wound

usually smaller medial wall injuries

air usually located in area of wound

if decreased Va, high dose steroids

air decompression:

CT for localization

retrobulbar needle into air pocket

fill syringe w/saline, take out plunger, watch for bubbles to appear

look on CT for incranial air: needs neurosurgery consult

Intraorbital Foreign Bodies

Imaging

plain film or CT if radio-opaque

MRI may be better for some

u/s good option

Removal Indications

esp if vegetable matter, or easy to get to in anterior orbit

may watch if inert, smooth edges, or in posterior orbit

Blunt Forehaed Trauma

can cause visual loss from traumatic optic neuropathy 2^o to direct (stretch,tear, contusion, etc) or indirect (small nutrient vessels to nerve) injury to fixed canalicular or chiasmal portion of o.n.

Treatment: spinal cord injury-dose I.V. steroids (see neuro-op sxn)

Tumors

w/u includes all for thyroid, plus palpation, retropulsion (1-4), facial asymmetry, transillumination, all need CT/MRI

observe most benign tumors (capillary and cavernous hemangioma, dermoid cyst) and tumors not easily resected (lymphangioma, plexiform neurofibroma, optic nerve meningioma, juvenile pilocytic astrocytoma)

benign tumors of orbit usually primary

S-100 staining for any neural crest cell derivative

Primary Orbital Tumors

Dermoid Cysts

if not noted until adulthood often not palpable b/c in post superotemporal orbit

choristoma: has tissue elements foreign to site

may displace globe, adnexa inferonasally

may erode/remodel bone, adhere to dura

keratin leak is inflammatory, can cause scarring

surgery difficult, try to keep wall intact

Vascular Tumors

Cavernous Hemangioma

most common benign solitary lesion in adults

30-60 yo female is typical

CT: smooth discrete lesion, fills with dye after 20 min; coronal cuts important to know tumor position relative to o.n. for sugical plan

MRI: hypointense to fat on T1, hyperintense to fat on T2

U/S: high reflectivity (A-scan high amplitude internal echoes)

well encapsulated and tolerated

path shows large cavernous spaces w/rbcs

surgery for symptoms esp optic nerve compression

usually lateral orbitotomy with complete resection from intraconal position generally feasible

Hemangiopericytoma

uncommon, 30-60 yo

encapsulated, hypervascular and hypercellular

well defined with contrast CT

conjunctival prolapse, hyperemia of cul de sac conjuctiva

may restrict motility

looks blue at surgery

must be completely excized b/c can recur and be malignant

benign-appearing lesions may become cancerous, recur and metastasize, while histologically "malignant" lesions may remain localized

AVMs

developmentally abnl formed arteries and veins w/o intevening capillary bed

corkscrew episcleral vessels

treatment: first arteriography, then occlusion of feeder vessels before surgery; otherwise exanguination possible

AV Fistula

trauma or degeneration causes communication btw previously nl artery and vein

spontaneous/degenerative fistula often w/HTN, atherosclerosis

high flow and severe

dx often apparant

from extradural branch of ICA, ECA or both

low flow w/subtle sx: proptosis, mild pain

chronic red eye (2^o to dilated episcl vessels)

asymmetric IOP (2^o to incr episcl venous P)

CT w/ enlarged sup orb vein, diffuse enlargement all EOMs from venous engorgerment

small fistulas may spontaneously close

increased ocular pulse amplitude (differance of IOP systolic/diastolic BP) when compared to fellow eye

can use pneumotonometer with paper strip for fifteen seconds; a difference of 1.5 mmHg very suggestive of fistula

CT as above/MRI, orbital color doppler, selective arteriography

difficult

embolize vs ligation ICA

control IOP

may need orbital decompression

Orbital Varicies

dilations of previously existing venous channels

proptosis w/dependent head position or post-Valsalva

CT w/contrast before & post-Valsalva shows enlargement of lesion 2^o decreasing venous return

treatment conservative; incomplete excision only when varix causes o.n. or globe damage

Histiocytic Tumors

Litterer Siwe, eosinophilic granuloma, JXG

lytic skull lesions, proptosis

Neural Tumors

Meningioma

benign but invasive, usually arise intracranially (therefore these are secondary) from arachnoid, extend thru bone, sup orb fissure, or optic canal into orbit

often w/ progressive vision loss, mild proptosis, female predominance

can be seen w/ NF but much less often than gliomas

if near the sella

o.n. compression w/ early Va loss

o.n. shunt blood vessels, papilledema, o.n. atrophy

proptosis, temporal fossa mass

lower lid edema, chemosis

primary orbital

typically arise from arachnoid of o.n. sheath

affect vision early, mean age @ presentation is lower, worse prognosis in children

can invade EOMs and globe

CT shows radiolucent center compared to o.n. glioma (denser center)

diagnostic tests

skull film: hyperostosis associated w/Ca⁺² of tumor, lytic lesions can be seen

CT as above, and hyperostosis w/ abnl Ca⁺² of tumor

MRI to outline tumor extent

Treatment

transcranial orbitotomy surgery on o.n. meningioma will cause blindness so only operate if useful vision lost or if intracranial structures threatened

Schwannomas (Neurilemomas)

young to middle aged

slow proptosis over yrs up to 4 mm

occur in 1% of pts with NF1

benign

choroidal striae with hyperopia, usually no diplopia

usually superior orbit

perineural spread and compression can cause pain

at surgery it is well encapsulated, yellow, and can be stripped away from nerves

can recur but low malignant potential

proliferations of Schwann cells encapsulated by perineurium

Antoni A (Verocay bodies, whorls and palisades) and B (myxoid with degeneration) patterns, no axons present

S100 positive

Neurofibromas

most common nerve sheath tumor

hamartoma of endoneural fibroblasts, Schwann cells, axons

localized

present in early to middle age

like schwannomas, 10% have NF1

not encapsulated but cannot tell from CT/MRI

can be multiple

remove by superior orbitotomy, can undergo malignant transformation

plexiform (diffuse)

seen in children (see Peds)

Optic Nerve Glioma

primarily a tumor of childhood (see Peds)

malignant optic glioma (glioblastoma) is very rare; middle-aged males, rapid blindness and death

Lacrimal Gland Tumors

look for fullness of upper lid, asymmetry of superior sulcus, abnormal lid contour

majority lac gl masses are idiopathic inflammatory dacryoadenitis

CT very goood for differentiating inflam from tumor: inflam and lymphoid w/in gland cause diffuse enlargement, elongated shape, contour around globe; neoplasms are isolated, globular, displace & indent globe

especially S-shape, often palpable

check for mobility, smooth, rubbery or nodular

proptosis is evidence of posterior growth, otherwise globe is down and medial

Epithelial Tumors:

50% benign mixed

50% carcinomas (50% of these are adenoid cystic, remainder: malignant mixed, 1^o adeno ca, mucoepidermoid ca, squamous ca)

Benign Mixed Tumor (Pleomorphic Adenoma)

most common epithelial tumor

30-50 yo, M sl>F

palpable, painless, slow (hx often reveals sx > 1 year) growing w/globe displ down, medial, axial proptosis

incites bony cortication, enlargement/expansion lac gl fossa, firm lobular mass

CT

gland is oblong if inflammatory, globular if malignant

pathology

metaplasia of epithelial cells to form stroma, cartilage

benign epithelial cell nests with loose mesenchymal connective tissue

variability of above = mixed tumor

microscopic extension into pseudocapsule causes recurrence if margins not adequate @ excision

treatment

must excise it all w/ lateral orbitotomy with en bloc excision including pseudocapsule;

don’t biopsy b/c of 1/3 chance of recurence, sig risk of malignant degeneration

Malignant Mixed Tumor

often arise from 1^o benign mixed or from recurrent benign mixed if incomplete excision

path similar to benign mixed but w/malignant change

least common epithelial tumor

treatment

frequent exenteration, bone removal necessary

fatality rate of 50%

Adenoid Cystic Carcinoma (Cylindroma)

PAIN from bone destruction, perineural invasion, rapid course diffs from benign mixed

pathology

swiss cheese appearance, stain with mucicarmine, looks benign, infiltration of orbital tissue, incl perineural invasion

basaloid pattern worst prognosis

treatment

death from intracranial extension or systemic mets after multiple recurrences

Nonepithelial Lacrimal Gland Tumors

Inflammatory

1/2 of lacrimal tumors

orbital inflammatory syndrome: see pseudotumor

sarcoidosis: African or Scandinavian descent, systemic dz, non-caseating granuloma w/monos, typical bilateral lac gl involvement, conj bx or lac gl bx or gallium scan may establish dx, ACE, lysozyme, CXR for hilar adenopathy, anergy on skin test

benign lymphoproliferative lesions of lac gl: middle aged F, dry eye, if w/rheumatoid arthritis, then classic Sjogren’s

Fibro-osseous Tumors

uncommon, may involve orbit

Fibrous Dysplasia

benign proliferative disorder

single bone or polyostotic (w/cutaneous pigmentation and precocious puberty in F = Albright syndrome)

Osteomas

benign, involve frontal sinuses

malignant chondro- or osteosarcomas destroy bone

treatment

XRT for most orbital lymphoid lesions that are confined to orbit (50% of lymphomas)

chemorx for systemic, rx can be controversial

Secondary Orbital Tumors

Intraocular Origin

choroidal melanoma, retinoblastoma

Eyelid Origin

Sinus Origin

2^o orb tumors most commonly from nose, paranasal sinuses

Brain Origin

sphenoid wing meningioma, may compress optic n., supraorbital fissure, F>M

Nonepithelal Tumors from Sinuses, Nose, Facial Bones

osteoma, fibrous dysplasia, sarcoma most common

Mucocele/ Mucopyocele

progressive cystic enlargement of sinuses from ostia blockage

usually no clear hx of sinusitis, trauma, infection, or polyps

cystic, lined w/respiratory epithelium, filled w/thick mucoid secretions

invade orbit by expansion, erosion of orb wall bones, usually from frontal or ethmoid

CT diagnostic

Frontal Sinus

most commonly w/inferior globe displacement and diplopia

can present as discharging lesion of upper lid

if infected (pyocele), can have fistula through upper lid

Anterior Ethmoidal Sinus

medial canthal mass with telecanthal appearance

less diplopia

r/o encephalocele

Sphenoid and Posterior Ethmoidal Sinuses

optic nerve compression, VF defects

cranial nerve palsies, pain

Maxillary Sinus

sudden nontraumatic enophthalmos

Treatment

surgical evacuation, reestablish drainage vs sinus obliteration

Squamous Cell Carcinoma

most common epithelial tumor secondarily invading orbit

maxillary sinus, nasopharynx, oropharynx origin

nasal obstruction, epistaxis, epiphora

treatment

sx excision w/XRT, exenteration if periorbital involvement

Fibrous Histiocytoma

40 yo, upper nasal orbit benign tumor

path: histiocytes, fibroblasts, cartwheel (storiform) pattern

Metastatic Tumors

breast mets in F, lung (bronchogenic) mets in M most common

pain, proptosis, inflammation, bone destruxn, early ophthalmoplegia

+ h/o known 1^o tumor in 75%, orb met = presenting sign in 25%

EOMuscles often involved

CEA, FNA vs orbitotomy for bx

breast mets can cause fibrous response w/enophthalmos, motility restriction

fresh tissue for hormone receptor

prostate mets can cause pseudotumor picture

treatment

palliative local radiation

wide excision for some isolated mets

Enucleation

entire globe removal for blind/painful, maligancy, cosmesis, severe trauma w/S.O. risk

Orbital Implants

usually 20-22 mm for adults, <18 mm rarely indicated even for very young

Inert Spheres

silicone, methyl methacrylate; inexpensive; relatively poor motility

Hydoxyapatite

bioreactive: vascularization can take 6-12 months, +/- sew recti to original positions

can drill and place peg after 6-9 months (w/MRI or bone scan confirmation of bioincorporation) for excellent direct prosthesis-shell motility

higher exposure rate

Exposure

can occur w/in first 3-4 months, rarely after one year

10% when wrapped in banked sclera

can be difficult to treat

if < 3mm in diameter, can heal spontaneously

larger defects may need burring down of implant with drill and mild debridement of conjunctiva and Tenon’s

may need a dermis fat graft to cover the central defect

Porous Polyethylene (Medpore)

bioreactive petroleum-based implant

less expensive than hydroxyapatite, probably easier to work with, motility arguably equal

Evisceration

globe contents removal; sclera, o.n. intact

Advantages

less anatomy disruption

+/- good prosthesis motility

some prefer in endophthalmitis

simpler prcedure can even be done @ bedside in unstable pt

Disadvantages

unsuspected intraoc tumor

sympathetic ophthalmia risk (should remove all uvea)

Socket Complications

GPC

esp w/prostheses

may need rx w/ mast cell stabilizers

good prosthesis hygiene is essential

Deep Superior Sulcus

need to increase orbital volume by either larger secondary implant or a second implant subperiosteally under the first

tighten lax lower lid if present +/- dermal fat graft

Fornix Contracture

prevent by preserving conj, wearing conformer post-op

Implant Exposure/Extrusion

fornices can’t hold a prostheic shell

treatment:

incise/excise scarred tissue

full thickness mucous membrane grafts

Misc Anophthalmic Problems

ectropion

2^o to prosthesis weight

Rx w/ tightening lateral (occ. medial) tendon

entropion

2^o to contracture/cicatrix

Rx w/marginal rotation vs. mucous memb graft

ptosis

2^o to implant migration, upper fornix cicatrix, levator damage

Rx w/internal levator advancement (Mullerectomy) b/c predicable & makes higher crease vs ext levator advancement w/intra-op adjustment

Exenteration

removes orbital soft tissues, incl globe

for destructive tumors extending into orbit not responsive to XRT; intraoc malig melanoma or RB w/extension outside globe, w/o mets; malignant epithelial tumors of lac gl; sarcoma and other tumors that don’t respond to non-sx; phycomycosis

subtotal, total, extended