Orbital Disease Flashcards
Average female PD
53-65
What is it called if PD is greater than average?
hypertelorism
What are Hertel exophthalmometry norms?
12-21 white, around 24 AA; >2mm difference b/w eyes is significant
What does pulsatile proptosis tell us?
vascular
What does resistance to retropulsion tell us?
there is something blocking the orbit
What is MRD 1?
UL to corneal reflex
What is MRD 2?
LL to corneal reflex
What is total palpebral width?
MR1 + MR2
Why might a goldmann VF be preferred?
goes out further which is good for neurological defects
What might be a reason not to run a HVF?
reduced acuity, may not see target
What is the brightest stimulus on Goldmann?
V4e
What is a ceco-central scotoma?
scotoma that involves the blindspot
What indicates true ON defect due to papillomacular bundle involvement?
ceco-central scotoma
What is the pneumonic for optic atrophy differentials?
VIN DITTCH, MD
What are the differentials for optic atrophy?
vascular, infectious/inflammatory, neoplastic, demyelinating, idiopathic, toxic/nutritional, traumatic, congenital, hereditary, metabolic/endocrine, degenerative
What are infectious etiologies of orbital disease?
orbital abscess/cellulitis/mucormycosis, syphilis (leuitic), TB
What are endocrine/metabolic etiologies of orbital disease?
thyroid
What are inflammatory etiologies of orbital disease?
sarcoid, orbital inflammatory pseudotumor, tolosa-hunt, granulomatosis with polyangitis
What are space occupying etiologies of orbital disease?
meningioma, mucocele
What are neoplastic etiologies of orbital disease?
orbital rhabdomyosarcoma, lymphoma, metastatic CA
What are vascular etiologies of orbital disease?
carotid cavernous fistula
What causes a carotid cavernous fistula?
rupture of the wall of the carotid artery, or one of its branches, into the cavernous sinus
What alters the hemodynamic state of the cavernous sinus and its venous exits in a CCF?
exposure to arterial pressure
What is the major orbital communication of the cavernous sinus?
superior ophthalmic vein
If the superior ophthalmic vein expands tremendously, what may occur subsequently?
engorgement of all orbital and conjunctival veins; veins become arterialized producing signs and symptoms of venous congestion
What are s/s of CCF?
pulsating exophthalmos, ocular bruit, diplopia, HA, conj chemosis, increased ICP, dilated conj vessels and visual decrease
Why is there some degree of bilateral orbital involvement of CCF?
normally occurring venous communications between the cavernous sinuses
What is the management of CCF?
typically resolves on it’s own but need to watch them
Orbital cellulitis/abscess can be secondary to infections in what locations?
paranasal air sinuses, ethmoid, puncture wound, bug bite, hordeolum
What is orbital cellulitis caused by?
gram positive staph and strep
What should you expect with orbital cellulitis?
fever
What is mucormycosis?
aggressive opportunistic fungal infection, humans are exposed often with soil and decaying vegetation but infection rarely occurs with intact immune system because macrophages phagocytize the spores
How does mucormycosis get to the orbit?
from the paranasal sinus mucosa
T/F the mortality rate of mucormycosis is low
false, high mortality rate; rapidly progressing infection with late diagnosis due to nonspecific symptoms
What patient populations are at risk for mucormycosis?
diabetes (especially ketoacidosis), people who receive multiple blood transfusions, immunocompromised patients with severe neutropenia, those on chronic steroids
What happens if mucormycosis leads to orbital apex syndrome?
ON involvement and vision loss, involvement of nerves III, IV, VI, V1 and V2
What is the cause of syphilis?
treponema pallidum, spiral shaped gram negative highly mobile bacterium
What are ocular findings of syphilis?
focal gummas (soft, non-cancerous growth) along nerves or orbital fissure syndrome
What is the cause of TB?
mycobacterium tuberculosis, small aerobic, nonmotile bacillus `
What are most common presentations of TB?
proptosis, nontender or mildly painful orbital/lid swelling, sinus formation, involvement of bony orbit and lacrimal gland with soft tissue inflammatory mass/abscess formation
Orbital tuberculosis presents with…
destruction of bone with or without sclerosis, extraconal inflammation/abscess formation, extension into the infratemporal fossa or intracranial extension, lacrimal gland involvement
What is the pathogenesis of TED?
antibodies bind antigenic receptor sites, T lymphocytes migrate to orbital tissues, cytokine cascade, GAG secretion and fibroblast proliferation
What does GAG secretion and fibroblast proliferation in TED result in?
swelling/inflammation/fibrosis; if chronic, fatty infiltration of muscles
Why can you have TED at any thyroid state?
it’s an autoimmune condition, Abs to TSH receptor
What is special about hyaluronic acid?
super hyperosmotic, 1 molecule attracts 4 molecules of water
What 3 main things plump the orbit?
adipogenesis (via increased Leptin), HA synthesis, myofibroblast differentiation and proliferation
How does Tepezza work?
blocks the IGF-1R-TSHR complex to prevent orbital edema and adipogenesis from ever starting; shown to reverse the effects of TED
What stage can you use Tepezza in?
active and chronic phases
What is elevated in hyperthyroid?
T3 and T4
What is elevated in hypothyroid?
TSH
What does euthyroid mean?
normal T3, T4, and TSH
What is Grave’s?
hyperthyroid + orbitopathy
What are categories of thyroid disease?
primary hyper, central hyper, primary hypo, secondary hypo
What is primary hyperthyroidism?
thyrotoxicosis and goiter
What is central hyperthyroidism?
secretory pituitary tumor
What is secondary hypothyroidism?
following radiation or resection
Why may a patient be on thyroxine?
hypothyroid initially due to involutional changes or hypothyroid following correction of hyperthyroidism
What are primary causes of thyroid changes?
autoimmune, nodule/tumor, previous radioactive iodine tx, iodine deficiency, medications, pregnancy
What is the central cause of hyperthyroidism?
pituitary gland tumor
What is the treatment of hyperthyroidism?
surgery, radioactive iodine treatment, anti-thyroid medication
What is more likely to cause TED following the procedure, radioactive iodine or surgical resection
radioactive iodine
What is active TED?
inflammatory phase, 6 months to 5 years, average 2 years
What is inactive TED?
fibrotic phase, becomes permanent damage
What are risk factors of TED?
smoking and radioactive iodine treatment
What is NO SPECS?
no s/s, only signs, soft tissue involvement, proptosis, EOM infiltration and fibrosis, corneal changes, sight loss
What are corneal changes in TED?
exposure keratopathy
What causes vision loss in TED?
optic nerve involvement
What is the coca-cola bottle sign?
appearance of the muscles of the orbit in thyroid eye disease; belly of muscle enlarges with tendinous insertions spared
Which muscles are affected first in TED?
IMSLO
When are tendons involved in disease?
in orbital pseudotumor, not TED
Where is IOP highest in TED?
in upgaze because IR presses on the globe
What are TED treatments?
palliative, rescue and rehabilitation
Which TED treatments are done in active phase?
palliative and rescue therapy
Which TED treatment is done in inactive phase?
rehabilitation
What is palliative/supportive therapy for TED?
ocular lubricants and ointments, eye masks/lid tape
What is rescue therapy for TED?
corticosteroid tx IV and orbital radiation
What is rehabilitation therapy for TED?
orbital decompression, strabismus, eyelid repositioning/blepharoplasty
What comes after orbital decompression surgery?
orbital decompression then strabismus surgery then blepharoplasty
T/F after orbital decompression surgery, EOMs are still swollen
true
What kind of disease is orbital inflammatory pseduotumor?
non-specific orbital inflammatory disease
What are other names for orbital inflammatory pseudotumor?
idiopathic orbital inflammation, orbital inflammatory syndrome
What is the most common cause of painful orbital mass in adults?
orbital inflammatory pseudotumor
When localized, what does orbital inflammatory pseudotumor involve?
EOMs, lacrimal gland, sclera, uvea, superior orbital fissure, cavernous sinus
When diffuse, what does orbital inflammatory pseudotumor involve?
orbital fatty tissues
What are the 3 most common orbital diseases in order?
TED, orbital lymphoma and non-specific orbital inflammatory disease
T/F non-specific orbital inflammatory disease is a diagnosis of exclusion
true
What is Tolosa Hunt?
episodic orbital pain associated with paralysis of one or more of CN 3, 4, or 6 which resolves spontaneously but can relapse and remit
What are diagnostic criteria for Tolosa-Hunt?
one or more episodes of unilateral orbital pain persisting for weeks if untreated, paresis of one ore more CNs and/or demonstration of cavernous sinus granuloma by MRI or biopsy, paresis coincides with the onset of pain or follows it within 2 weeks, pain and paresis resolves within 72 hours when treated adequately with corticosteroids
What did biopsied cases of tolosa hunt show?
the syndrome has been caused by granulomatous material in the cavernous sinus, SOF or orbit
What is lagophthalmos?
incomplete closure of the lids
What does sarcoid show on histology?
non-caseating epithelioid granuloma
How is sarcoid diagnosed?
elevated angiotensin-converting enzyme levels
How often does sarcoidosis involve the eye?
1/2 of the time
What does ocular involvement of sarcoid involve?
uveitis, periphlebitis, multifocal choroditis, papillitis, papilledema, lacrimal gland enlargement and dry eye
What is granulomatous with polyangiitis?
Wegner’s, granulomatous and sometimes necrotizing vasculitis targeting the respiratory tract and kidneys
How often is there orbital involvement in granulomatous?
60% of patients; frequently the first or only clinical presentation
What is treatment of granulomatous with polyangiitis?
corticosteroids
What highly suggests GPA (granulomatous)?
proptosis + respiratory disease
What can aid in diagnosis of GPA?
positive ANCA
What is the diagnostic criteria for GPA?
nasal or oral inflammation, respiratory radiographic abnormalities consistent with respiratory tissue destruction, microhematuria or RBC casts on urinary sediment analysis, granulomatous inflammation on biopsy for pathology
When 2 of 4 diagnostic criteria for GPA are met, sensitivity and specificity of diagnosis are..
88.2 and 92%
T/F positive ANCA is conclusive for GPA
false
What is the common demographic for GPA?
men in 5th decade
What is a meningioma?
benign growth often > 50 year old male
Where are meningiomas often located in the head?
40% skull base and 20% sphenoid wing (greater propensity for greater wing)
What systemic condition can meningioma be associated with?
NF2
What is a mucocele?
mucus filled cyst, slow growing
What does a mucocele cause?
bone erosion
What is the most common primary orbital malignancy in children?
rhabdomyosarcoma?
Where are rhabdomyosarcoma’s typically located?
superior and superior nasal orbit leading to proptosis and inferior/inferior temporal displacement of the globe
What age group does rhabdomyosarcoma affect?
90% occur before the age of 16, mean 5-7 years old
What tissue type is rhabdomyosarcoma composed of?
mixed tissue type originates directly from striated muscle
What are two differentials for rhabdomyosarcoma?
orbital cellulitis and concomitant sinus/URT infection
What are s/s of rhabdomyosarcoma?
pain, visual loss, rapidly progressive course
What produces insidiously progressive exophthalmos?
most orbital neoplasms in adults (exception metastatic tumor)
Who is lymphoma more common in?
female population
What is the most common site of lymphoma malignancy?
orbit 46-74%, conj 20-33%, eyelid 5-20%
What percent of adult malignant orbital tumors does lymphoma make up?
55%
What is the survival rate of lymphoma if isolated and treated promptly?
90-94%
What is treatment of lymphoma?
surgical excision, radiotherapy or external beam irradiation
What is the most common primary OAL?
low-grade malignant extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue
What indicates Hodgkin’s lymphoma?
reed-sternberg cells
What indicates Hodgkin’s lymphoma?
reed-sternberg cells
What is a salmon colored lesion on the conj?
likely conjunctival lymphoma
What are common presentations of primary metastatic cancer of the orbit?
proptosis and visual loss
What are common presentations of orbital metastases?
diplopia and pain
What are the most common tumors to metastasize to the orbit?
breast, lung, prostate
What are some examples of congenital hypertelorism causes?
Crouzon syndrome, Waardenburg syndrome, cri du chat syndrome
What is Crouzon?
autosomal dominant genetic disorder known as a branchial arch syndrome
What branchial arch does Crouzon affect?
first aka pharyngeal, which is a precursor of the maxilla and mandible
What can Crouzon’s cause?
premature fusion of certain skull bones
What are important HPIs for orbital disease?
medical history, family history, onset, symptoms, progression
What is pathognomonic for a fungal tumor on MRI?
lesion gets darker from t1 to t2
What lights up on a T2 MRI?
water
What is an osteotomy?
removal of bone
What are 3 stages of visual field recovery following resection of compressive lesion?
initial fast, delayed slower, late mild
What is the cause of initial fast recovery of visual field?
restoration of signal conduction along retinal ganglion cell axons
What is the cause of delayed slower recovery of the VF?
restoration of axoplasmic transport and remyelination of the decompressed optic nerve
What is aspergillus?
saprophytic fungus found in soil and decaying vegetation
What is the most common organism form to contaminate paranasal sinus and orbit in the US?
aspergillus fumigatus, commonly in immunocompromised host
What kind of environment does aspergillus prefer?
hypoxic (anaerobic sinus)
What is invasive aspergillosis?
damage caused by necrosis and/or infiltration; granulomatous inflammation and necrotic fibrosis or fulminate diffuse vascular invasion, thrombosis and tissue necrosis
What is non-invasive aspergillosis?
damage caused by mechanical pressure, does not invade bloodstream; fungus ball, chronic erosive fungal, allergic fungal
Always get an MRI before prescribing…
steroids for orbital pseudotumor, you can kill your patient
T/F Memphis is #6 on the list for Aspergillosis
true
How is CT useful in fungal infection?
shows calcifications that are common in fungal sinusitis, rare in bacterial sinusitis and tumors; shows bony erosion
How is MRI useful in fungal infection?
more important in the invasive form; characteristic very low signal on the T2 weighted image due to mineralization of the fungal tissue with iron and manganese
What is the only way to definitively diagnose aspergillosis?
histological examination
What is the treatment of aspergillosis?
debridement and debulk of invasive form; amphotericin B 0.5 mg/kg/day (toxic SE) and -azole antifungals
How does Fluconazole work?
binds cytochrome P-450; less side effects than amphotericin B
What is the new standard of care treatment of Aspergillosis?
Voriconazole, 96% bioavailability, transient blurred vision and light sensitivity
Why may many children have facial asymmetry?
They are still growing
