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Anterior segment disease
Conditions for final exam
124
Pathology
Graduate
04/14/2013

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Term
iridodyalisis
Definition
Disinsertion of the iris root from the ciliary body. Induced by trauma and looks like two pupils.
Term
Sphincter tear
Definition
Radial tears around the pupil causing a traumatic dilation of the pupil.
Term
Iridodenesis
Definition
Jiggling of the iris often in aphakic patients where the motion of the vitreous transfers to the iris. Focal iridodenesis is traumatic and may result from iridodyalisis
Term
Hyphema
Definition
Settled blood in the anterior chamber. Graded by the portion of the anterior chamber that is filled with blood. Grade 4 is known as a blackball or 8-ball hyphema.
Term
Microhyphema
Definition
Red blood cells leaking into the anterior chamber
Term
Corneal blood staining
Definition
A staining of the cornea as a result of long standing hyphema. Initially presents with yellow crystals in the stroma. Can impede vision.
Term
Dellen
Definition
Dehydration of the limbal cornea causing thinning and formation of little divots. May be associated with pterygia, pingueculae, or chemosis
Term
Superficial punctate keratitis
Definition
Epithelial inflammation with intraepithelial infiltrates. May cause FBS and photophobia. Often related to dry eye and allergies.
Term
Superior limbic keratoconjunctivitis
Definition
Inflammation and injection of the superior limbal area.
Term
Fungal keratitis
Definition
Fungal infection of the corneal. Often presents with fuzzy borders and satellite lesions. May also be gelatinous or have immune rings. Very hard to treat. Aspergillus is the most common cause
Term
Interstitial keratitis
Definition
May also be called disciform keratitis. It is stromal inflammation. May present with deep vascularization of the stroma and hemes and the vessels may resolve into ghost vessels which look a lot like prominent corneal nerves. Most common cause is herpes simplex.
Term
Cogan's syndrome
Definition
Defined by interstitial keratitis and hearing loss
Term
Protozoan keratitis
Definition
Protozoan infection of the cornea. Most commonly caused by acanthamoeba. Present with a stromal ring, pain, +PAN,injection, follicles, cells and flare and sometimes follicles, hypopyon and prominent corneal nerves. Treat with brolene, PHMB/chlorhexidine, miconazole or ketoconazole and pentamidine
Term
Bacterial corneal ulcer
Definition
Relatively rare. bacterial infection of the cornea resulting in ulceration. Mostly caused by s. aureus, s. pneumoniae, and pseudomonas. Typically treated with 4th gen fluoroquinolone
Term
marginal keratitis
Definition
antigen-antibody infiltrates in the peripheral cornea. May coalesce into arcs. Often a result of staph hypersentivity.
Term
Infiltrates
Definition
Focal areas of anterior stromal inflammation and accumulation of leukocytes and debris. May be hypersentivity reaction or related to a virus, bacteria, fungus or protozoan. Tend to be multiple, small, mild to no staining, and more peripheral.
Term
neurotrophic keratitis
Definition
large ulcers associated with damage to the trigeminal innervation of the cornea. Typically have gray, rolled borders. May be caused by herpes, diabetes or other neurological conditions or surgery. May also be related to abuse of topical anesthetics or Riley Day syndrome.
Term
exposure keratitis
Definition
cornea becomes dry and inflamed from environmental exposure. Can be related to lagophthalmos, parkinsons and proptosis among other things.
Term
infectious crystalline keratitis
Definition
Complication of long term steroid use. Results in an infiltration of s. viridans which make crystal like lesions in the cornea. These present with intact epithelium and the appearance of star like infiltrates.
Term
thygeson's keratitis
Definition
common bilateral keratitis in females. Has multiple, pinpoint, granular, elevated intra-epithelial lesions. So it looks like large SPK with a crushed chalk like appearance. treat with steroids.
Term
filamentary keratitis
Definition
gelatinous mucin strangs that adhere to the corneal epithelium. Often caused by the lid not being able to rewet the cornea with tears. Will present with FBS and SPK that looks like band keratopathy. You can remove these filaments with forceps or just treat with a bandage CL and steroid
Term
xerophthalmia
Definition
aqueous deficiency dry eye associated with vitamin A deficiency. Complain of night blindness and may have a bitot spot, keratomalacia or loss/change of epithelial cells and goblet cells
Term
bullous keratopathy
Definition
blister like edema on the cornea in the epithelium. Can distort vision and cause pain and erosion. Can happen after phaco, particularly in those with prior guttata. Can also happen in patients with prolonged high IOP due to endothelial damage.
Term
Kaye's dots
Definition
epithelial infiltrates at the edges of sutures. May or may not be a sign of rejection.
Term
Krachmer spot
Definition
stromal infiltrates that look like infiltrates related to adenovirus. a sign of graft rejection
Term
khoudadoust line
Definition
lines of inflammation and precipitates in the endothelium. a sign of graft rejection.
Term
sjogrens syndrome
Definition
chronic autoimmune disease causing drying of mucous membranes and destruction of exocrine glands. Way more common in women. Related to the "sicca complex" or xerostomia (dry mouth), xerophthalmia (dry eyes), and exocrine gland infiltration. May look like jowls of a dog. May also be associated with increase risk of lymphoma. Secondary form often due to rheumatoid arthritis
Term
herpes simplex 1 primary infection
Definition
initial infection has seropurulent vesicles on the lids/adnexa and conjunctival effects. Will also have +PAN and possibly a recent URI fever or FBS. mostly occurs in children
Term
herpes simplex virus epithelial keratitis
Definition
HSV1 recurrence. Usually unilateral. hallmark sign is a dendritic ulcer but may look like linear SPK that won't heal. Can progress to geographic erosions or become stromal. Can cause non-granulomatous uveitis with associated trabeculitis and iritis atrophy
Term
herpes simplex virus stromal keratitis
Definition
herpes simplex 1 recurrence, usually after long standing epithelial involvement. this is immune mediated and inflammatory which causes an immune ring (Wessely). The stromal inflammation is called interstitial keratitis and can lead to thinning and neovascularization which can progress to ghost vessels. Can lead to neurotrophic ulcers, edema, opacification, endothelial folds etc. Put a steroid on it for a LONG time.
Term
herpes zoster
Definition
herpes 3. can reactivate and cause shingles. If shingles affects the tip of the nose (hutchinsons sign) there is a good chance it is affecting the cornea because both are innervated by the nasociliary branch of CN5. Presents with vesicular lesions, bleph, ptosis, keratitis, scleral thinning and inflammation in other tissues like the uveal tract (non-granulomatous) or optic nerve.May also cause CN palsys. Very painful. Keratitis looks like pseudo-dendrites with tapered ends. They are actually elongated infiltrates and not ulcers and are plaque like. Will progress to deep stromal keratitis. May also have nummular keratitis (granular subepithelial infiltrates surrounded by stromal haze).
Term
prominent corneal nerves
Definition
white stripe looking things in the cornea. related to endocrine neoplasias, keratoconus, neurofibromatosis, fuchs', refsum's syndrome, trauma, congenital glaucoma, graft failure, and inflammation
Term
pannus
Definition
fibrovascular intraepithelial growth around the cornea. three types. pannus crassus: thick with lots of vessels and very dense. pannus ciccus: dry glossy surface. pannus tenuis: thin with few vessels and light opacity.
Term
anterior unveitis
Definition
occurs in the iris or pars plicata or both (ie iritis or iridocyclitis). iridocyclitis is the most common type of uveitis in herpes patients
Term
intermediate uveitis
Definition
inflammation of just the pars plana with signs in the vitreous
Term
posterior uveitis
Definition
inflammation of the posterior segment such as chorioretinitis
Term
panuveitis
Definition
inflammation of pretty much the whole globe
Term
UGH syndrome
Definition
Uveitis, glaucoma, and hyphema syndrome. caused by an IOL rotating out of position.
Term
uveitis (general)
Definition
inflammation of the uveal tract. presents with cells and flare, ciliary flush, photophobia, pain, miosis, PAS, decreased VA, keratic precipitates (fine or mutton fat and may coalesce into arlt's triangle), iris nodules, iris atrophy or dilated iris vessels, and IOP changes
Term
koeppe nodules
Definition
nodules at the pupil border of the iris. associated with uveitis
Term
busacca nodules
Definition
nodules on the iris away from the pupil border associated with uveitis
Term
schwartz syndrome
Definition
uveitis caused by low lying retinal detachments
Term
snowballing
Definition
accumulations of vitreous white cells in the inferior vitreous just before the retina. May be referred to as snow banking if it occurs near the pars plana
Term
posner-schlossman syndrome
Definition
Also known as glaucomatocyclitic crisis. trabeculitis caused by cataract surgery. low grade uveitis that is unilateral and more common in men. Also have corneal edema, IOP spikes, and small/fine keratic precipitate.
Term
iris bombe
Definition
complete posterior synechia 360 causing pupillary block (seclusiopupillae) and a buildup of pressure causing a protrusion of the iris.
Term
ankylosing spondylitis
Definition
HLA-B27 positive disorder that occurs in young adult males. Causes sacroiliac fusino (fusion of the vertebral column) which causing an arched spine and lower back pain. can cause bilateral uveitis with tons of flare to the point of plasmoid aqueous
Term
inflammatory bowel disease
Definition
chronic intermittent diarrhea alternating with constipation. causes acute uveitis
Term
reiters syndrome
Definition
can't see (bilateral uveitis and keratitis or mucopurulent conjunctivitis) can't pee (urethritis) can't climb a tree (polyarthritis). Also HLA-B27 positive and in young men.
Term
Psoriatic arthritis
Definition
Uveitis (usually iritis) occuring in people who have both psoriasis and arthritis.
Term
lens induced uveitis
Definition
leaking of lens material causes an immune response. happens in incomplete cataract surgery or hypermature cataracts
Term
juvenile-rheumatoid arthritis
Definition
occurs mostly in young females and causes a bilateral chronic non-granulomatous iritis. The iritis can present before arthritis symptoms. Present as asymptomatic (no ciliary flush!). May also have band keratopathy. Three types (pauciarticular=most common mostly girls ANA +, polyarticular=mostly girls, more joints affected, ANA+, still's disease=rarest, systemic, equal M/F, fever, ANA- and no associated uveitis)
Term
fuch's heterochromiciridocyclitis (fuch's uveitis syndrome)
Definition
chronic unilateral non-granulomatous uveitis (iritis) where stroma of one iris thins and causes transillumination defect (lighter iris). Iris will also have blunt (flat) features. Tend to have fine stellate keratic precipitates that distribute diffusely across the endothelium. may also have glaucoma, cataracts, or vitreal opacities. may also have bleeding in the angle (amsler sign)
Term
syphilis
Definition
sexually transmitted disease. presents in the secondary stage with maculopapular rash on the palms/soles of feet and other symptoms that bring them to us. have iris roseola (vascular papules on the iris that look pink). also has interstitial keratitis and ghost vessels. causes chronic granulomatous uveitis (either panuveitis or iritis both severe). the panuveitis has salt and pepper fundus appearance, flame hemes, vascular sheating, and chorioretinitis. they also can't feel their feet on the group (tabesdorsalis)
Term
tuberculosis
Definition
causes a chronic granulomatous uveitis (usually iritis). may also cause phylectenules, conj. nodules, interstitial k, scleritis. often feel weak, have night sweats and cough up blood :/
Term
lymphoma
Definition
masquerades as a posterior uveitis that doesn't respond to treatment
Term
leukemia
Definition
masquerades as a bilateral uveitis that doesn't respond to treatment. confirm with CBC. May also have heterochromia, hyphema and glaucoma.
Term
sclerouveitis
Definition
uveitis secondary to scleritis
Term
toxoplasmosis
Definition
fungal infection that causes unilateral panuveitis. most common infectious retinitis cause in the immunocompetant!looks like headlights in the fog (dense yellow lesion seen through clouded vitreous). Presents usually around age 25 test with sabin-feldman dye test
Term
toxocariasis
Definition
infection transmitted by dogs that presents between ages 2-9 usually and causes a white pupil. can cause uveitis.
Term
acute retinal necrosis
Definition
unilateral granulomatous uveitis with associated vitritis. (if bilateral called BARN). associated with herpes simplex and zoster
Term
behcet's disease
Definition
triad of symptoms: bilateral iritis (maybe affecting posterior segment), apthous stomatitis (ulcer of the oral mucous), and genital ulcers. Have a +pathergy test
Term
lyme disease
Definition
a treponemal infection transmitted by ticks that can cause acute non-granulomatous uveitis. has a +FTA-ABS test. show a target skin rash and may also have optic neuritis, meningitis, or encephalitis
Term
sarcoidosis
Definition
systemic granulomatous disorder affecting mostly young black men or old while women. bilateral chronic granulomatous uveitis which typically causes dense posterior synechiae. can also form granulomas in the lungs and lymph nodes. often present with mutton fat precipitates and busacca nodules. may also have vascular sheating that look like candle wax dripping, vitreous snowballing, bilateral hilar lymphadenopathy (swollen chest lymph nodes), and lupus pernio (purple "lupus" which is swelling of extremitis)
Term
vogt-koyanagi-harada
Definition
t-cell mediated melanocyte issue. causes severe bilateral granulomatous panuveitis that can become a smoldering low grade uveitis over time. have vitiligo, polisos, deafness, and multiple serous retinal detachments. (if skin signs and uveitis only, just VK. If only neurological stuff just Harada syndrome)
Term
episcleritis
Definition
local inflammation of the episcleral vessels. differentiate from conjunctival injection by using phenyl. may be idiopathic or related to TB, syphilis, RA or other collagen vascular diseases. Can be simple (diffuse or sectoral) or nodular
Term
scleritis
Definition
Inflammation of the deeper scleral vessels. More associated with systemic conditions (like RA) Looks similar to episcleritis but presents with pain, typically which radiates to the jaw. Upon resorption can cause scleral thinning. Can be anterior (diffuse,nodular, or necrotizing(vaso-occlusive, granulomatous,surgically induced)) or posterior
Term
Scleromalacia perforans
Definition
Necrotizing scleritis without inflammation. In elderly female patients with RA. Usually appears superiorly.Painless, rarely perforating, and can cause choroidal protrusion and large sections of scleral thinning
Term
Wegener's granulomatosis
Definition
Idiopathic multisystem granulomatous disorder with small vessel vasculitis. Affects respiratory system/kidneys. Associated with necrotizing progressive granulomatous scleritis. May affect eye without systemic complications.
Term
Relapsing polychondritis
Definition
Idiopathic condition with small vessel vasculitis involving cartilage. Cause of intractable scleritis (necrotizing or non)
Term
polyarteritis nodosa
Definition
Collagen vascular disorder that affects medium sized and small arteries and causes aneurysms. Ocular involvement (scleritis) can precede systemic symptoms.
Term
Scleral hyaline plaque
Definition
Dark gray areas over the muscle insertions. Normal finding in the elderly.
Term
Plateau iris
Definition
Anteriorly rotated ciliary processes which cause a protrusion of the base of the iris root crowding the angle. Appears as a narrow angle on van herick and demonstrates double hump sign on gonio.
Term
Anisocoria
Definition
Uneven pupil sizes. If aniso is bigger in bright light, the large pupil is the problem. If aniso worse in dim light the small pupil is the problem. Can be physiologic (same dim/bright), pharmacologic, mechanical (surgery/trauma and pathology)
Term
Horner's
Definition
Classic triad: meiosis(anisocoria where miotic pupil is the issue so worse in dim light), ptosis (including a reverse ptosis), anydrosis (if 1/2 order). May also have heterochromia. Problem with sympathetic innervation to face and eye. Diagnose with 10% cocaine or apraclonidine. Find lesion location with 1% hydroxyamphetamine. 1st order caused by brainstem issues (trauma, demylination, vascular), neck trauma, and spinal chord tumors. Second order caused by pancoast tumor, aortic dissection, and neck lesions (granular, trauma, post-surgical). Third order caused by internal carotid dissection, fistula, migraine, HZO, or otitis media.
Term
Tadpole pupil
Definition
Distortion of the pupil that is transient lasting less than a few minutes and occurring several times in a day or for weeks at a time. Caused by iris dilator spasm. Related to horner's, adie's and migraine.
Term
corectopia
Definition
Misplaced pupil.
Term
Aide's tonic pupil
Definition
Usually in younger females. Pupil that is dilated (iridoplegia) in bright light and no or slow reaction to light but a slow reaction to accommodation which may be vermiform in nature. Caused by denervation of sympathetic supply to the sphincter muscle and ciliary muscle. Bilateral can occur but very rare (rule our perinauds which has restricted upgaze with nystagmus). Diagnose with 1/8 pilocarpine. Constriction is (+). Ass't with herpes infection, DM, and guillain-barre. Also related to diminished deep knee tendon reflex (Holmes-Aide's syndrome)
Term
Argyl robinson pupil
Definition
Small pupil reacts poorly/not at all to light but normal to convergence. Does not dilate well. Related to tertiary syphilis though DM and long standing aide's can mimic it.
Term
3rd nerve palsy
Definition
Eye is down and out, pupil dilated, severe ptosis. Sudden and painful is usually caused by aneurysm of the posterior communicating artery. Potential of rupture and fatal hemorrhage into subarachnoid space!
Term
Amaurotic pupil
Definition
Absolute afferent pupil defect.Blind eye due to optic nerve lesion, completely unresponsive directly. Pupil dilates when seeing eye occluded, but appears normal with both eyes open due to consensual response.
Term
Relative APD
Definition
AkA marcus gun pupil. Incomplete optic nerve lesion or retinal disease diminishes pupil response to light directly. Mostly caused by different neuropathies and neuritis or a CRAO.
Term
Seclusio pupillae
Definition
Posterior synchiae 360 (iris bombe)
Term
Occlusio pupillae
Definition
A fibrotic membrane that covers the pupil (often due to long standing uveitis and plasmoid aqueous)
Term
Leukocoria
Definition
White pupil. Can be due to cataracts, retinoblastoma, ROP and others.
Term
Rubeosis iridis
Definition
Neovascularization of the iris and angle. Associated with DM CRVO and ocular ischemic syndrome. Can lead to glaucoma and hyphema. If covering entire pupil its called Florid neo.
Term
Pigment dispersion syndrome
Definition
Most common in myopic males. Posterior iris rubs against lens zonules which causes pigment to be removed which accumulates on the posterior cornea as a kruckenberg spindle. May have ITI, posterior bowing of iris (reverse bombe), pigmented AC cells and other pigment accumulations in the angle and on the lens . Can cause glaucoma (50%)
Term
Iris heterochromia
Definition
Can be the full iris (iridum) or just one iris with 2 colors (iridis). Can be congenital (if hypo=horner's, waardenberg,idiopathic. if hyper=nevus of ota, hemartoma) or acquired (hyper=tumor, siderosis. hypo=fuch's heterochromic iridocyclitis or acquired horners)
Term
iris nevus
Definition
Darker pigment spots on iris
Term
Iris melanoma
Definition
May look like an iris nevus but closer to an involving the pupil border. Often will drag the pupil, have intrinsic vascularity and color changes. May be amelanotic.May have multiple lesions (called tapioca appearance) More common in light eyes.
Term
Brushfield's spots
Definition
Circular white/gray nodules usually in an arc formation associated with Down's.
Term
Kunkmann Wolffian bodies
Definition
White/gray nodules in an arc formation that occur idiopathically
Term
Lisch nodules
Definition
Lightly pigmented (brown) gelatinous melanocytic hamartomas on the iris (nodules). In 94% of NF1, typically found in inferior half of iris.
Term
Iris varix
Definition
Dilated vein on the iris can lead to heme
Term
Nuclear sclerosis
Definition
Changes in the lens nucleus causing a yellowing an opacity. Grade 1 defined as the beginning of optical separation with or without yellowing. Grade 4 is brunescent (yellow/brown). Can have white NS which is white in color but very dense, typically are familial and progress more rapidly and are harder to remove.
Term
Cortical cataract
Definition
Cataract changes in the lens cortex. Begin as water clefting and progress usually to a spoke or wedge like pattern. Often ass't with DM
Term
Posterior subcapsular cataract
Definition
Glossy/spotty and highly reflective changes just beneath the posterior capsule. Affect near acuity more than distance. Can occur in DM, trauma or with steroid use.
Term
Anterior subcapsular cataract
Definition
Rare. May present with atopic dermatitis patients. Opacity under the anterior lens capsule
Term
Snowflake cataract
Definition
Typical DM cataract. Caused by acute blood sugar changes. Look like flecks of snow.
Term
Christmas tree cataract
Definition
Multicolored, unilateral, reflective, in the deep cortex or nucleus. Ass't with myotonic dystrophy
Term
Mature cataract
Definition
Any cataract type where the lens is completely opaque
Term
Hypermature cataract
Definition
Cataract that has shrunken and wrinkled the anterior capsule and is losing water
Term
Morgagnian cataract
Definition
Hypermature cataract that has a completely liquified cortex and a sunken nucleus (setting sun). Risk of immune response
Term
Anterior polar/pyramidal cataract
Definition
dense circular and well defined cataract on the anterior pole of lens. May protrude. Has reduplication.
Term
Posterior polar cataract
Definition
Dense circular well defined opacity on posterior pole of lens. Has reduplication and can affect near acuity.
Term
Cerulean cataract
Definition
Bluish spot like cataract on posterior cortex. Ass't with coronary cataracts and other types and can be sectoral.
Term
Coronary cataract
Definition
Multiple finger or bowling pin shaped opacities making a ring in the posterior cortex.
Term
Pulverulent/powder cataract
Definition
Hollow sphere with a central opacity involving fetal nucleus
Term
Y suture cataract
Definition
Opacity of y suture. If congenital it is very well defined. If occurred later it is more stellate
Term
Embryonal cataract
Definition
Luminescent opacities between embryonic nucleus and anterior Y suture. Small dots or propellar shaped. Occur in 25% of people.
Term
zonular/lamellar cataract
Definition
U-shaped "riders" of opacity that surround the edge of the nucleus.
Term
Reduplicating cataract
Definition
Opacity, typically on the anterior capsule, that looks like opacities in the following layers. Usually anterior to nucleus and caused by intrauterine or post-natal injury
Term
chlorpromazine cataract
Definition
antioer subcapular cataract that form a star like pattern. Side effect of long term use of an antipsychotic
Term
Rosette cataract
Definition
Cataract caused by trauma. A feathery rounded opacity with central suture lines. can be early (occuring within weeks to months of the trauma) or later (occuring years later). Early ones tend to be more anterior whereas the late ones are usually direcly on top of the nucleus
Term
Vossius ring
Definition
Traumatic lens defect caused by the pigmented part of the iris pushing against the anterior lens. Leaves a circular pigment ring.
Term
Epicapsular stars
Definition
Small light brown, reflective, star shaped deposites on the anterior capsule. Remnants of the tunica vasculosa lentis
Term
Posterior capsular opacification
Definition
Aka prosterior capsular fibrosis. Opacification of the posterior capsule after cataract surgery.
Term
Elschnig pearls
Definition
Remaining epithelium after cararact surgery that begins to proliferate and form pearl like opacities which can coalesce and impede vision
Term
Endophthalmitis
Definition
Infection inside the eyeball. BAD. Be on the lookout after surgeries for any hypopyon since this is a classic indicator. Refer out immediates for intavitreal antibiotics or vitrectomy
Term
Anterior capsular contraction
Definition
Post cataract surgery when the capsular rhexis (hole in the remaining anterior capsule) shrinks and opacifies
Term
Lens exfoliation
Definition
True versions are rare. Occurs when the lens delaminates as a result of intense infrared radiation. Results in the anterior capsule thickening and splitting into two, appearing to float
Term
Pseudoexfoliation syndrome
Definition
A result of systemic basement membrane disorder where the anterior lens capsule begins to deposit in flaky sheet like formations. Where the iris contacts the lens it will peel and where it doesn't will be clear (target pattern). Has a glaucoma risk. May have transillumination defects
Term
Bells Palsy
Definition
Palsy of CN 7 due to inflammation, possibly viral related. Diagnosis of exlcusion. Differentiate from a space occupying lesion (cerebellopontine tumor). May have dry eye
Term
Myokymia
Definition
Eyelid muscle twitching (mueller's or ciliary portion of orbicularis oculi). If involving the superior oblique (SOM) will have diplopia, vertical nystagmus, oscillopsia. If myokymia accompanies a facial twitch they may have a brainstem disease or aneurysm
Term
Benign essential blepharospasm
Definition
Involuntary tonic spasm of the obicularis oculi. Can render patients functionally blind for a period of time. Varients = meige's (BEB with focal motor prob in the mouth) and breughel (BEB with mandibular and neck muscle involvement)
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