Friedman Anterior segment Flashcards

1
Q

Pigment Dispersion Syndrome (PDS) demographic

A
Young, myopic man blurred in exercise
Chafing from iris pigment epithelium
M>F, young adults, myopic>emmetropic
30-50% develop glaucoma
Myopic: increased risk of retinal breaks, lattice degeneration (20%)
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2
Q

Pigment Dispersion Syndrome (PDS) signs and Rx?

A

Signs?
Krukenberg spindle (cornea)
Scheie stripe (post. capsule)
Zentmayer line (ant hyaloid, zonular fibers)
Concave iris + mid-peripheral slit-like TIDs
Pigment in angle
Wide IOP fluctuations

Treatment?
Miotics (minimize iris-zonule touch)
LPI unproven
ALT good
SLT bad (IOP spike; use lower power)
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3
Q

Reverse pupillary block

A

See in PDS - iris has a concave configuration with contact against the ZONULES.

Iris movement –> pigment liberation from the posterior surface as it rubs against the underlying zonules during normal pupillary movement

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4
Q

Mechanism of Pigment Dispersion Syndrome glaucoma

A

iris pigment obstructs the TM causing elevated IOP and ON damage

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5
Q

natural course of Pigment Dispersion Syndrome glaucoma

A

burns itself out once all pigment has been liberated (no more pigment to clog TM and raise IOP)

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6
Q

How to confirm ciliary body tumor (imaging/findings)

A

Imaging:
Use UBM
Anterior segment OCT
scheimpflug imaging

Findings:
lenticular astigmatism and cataract
shallow anterior chamber
sentinel vessel
extrascleral extension
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7
Q

Rx of ciliary body tumor

A

depending on extent of tumor:

Rx is with surgical excision, chemoRx, XRT or enucleation

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8
Q

DDx for non-granulomatous iritis

A
idiopathic
HLA-B27
Fuchs heterochromic iridocyclitis
HSV
Posner-Schlossman syndrome
Lyme
Behcet's disease
drugs
interstitial nephritis
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9
Q

Work-up for non-granulomatous iritis

A
HLA-B27
sacroiliac X-ray
CBC with diff
UA
VDRL/RPR and FTA-ABS
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10
Q

Disorders assoc/w/HLA-B27 iritis

A
PAIR:
Psoriatic arthritis (needs to be arthritis, skin finding of psoriasis alone is not enough)
Ankylosing spondylitis
IBD (inflammatory bowel dz)
Reiter's

Whipple’s disease

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11
Q

Whipple’s disease

A

caused by Tropheryma whipplei, a gram-positive, non-acid-fast, PAS-positive rod (white european men)

Two findings, at least one of which is present in approximately 20 percent of such patients, are considered pathognomonic for Whipple’s disease: oculomasticatory myorhythmia (continuous rhythmic movements of eye convergence with concurrent contractions of the masticatory muscles) and oculo-facial-skeletal myorhythmia.

These abnormalities are almost always accompanied by supranuclear vertical gaze palsy.

Whipple’s disease should be considered in all patients with the four cardinal manifestations (arthralgias, diarrhea, abdominal pain, and weight loss). Suspicion of the diagnosis is more difficult in those patients who do not develop gastrointestinal symptoms. In particular, it should be a consideration in patients with rheumatoid factor-negative migratory polyarthritis that does not respond to immunosuppressive therapy. (See ‘Clinical suspicion’ above.)

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12
Q

Pigmented iris lesion DDx

A
Nevus
Melanocytoma
melanoma
iris pigment epithelial tumor
rarely mtz (usually amelanotic)
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13
Q

Iris pigment epithelial cyst

A

Iris pigment epithelial cysts are commonly located at the iridociliary junction and are round or oval. When observed by slit-lamp examination, they have a smooth brown surface. Visualization of the tumor can be improved by dilation of the pupil.

Iris pigment epithelial cysts have thin walls and sonolucent contents (as imaged by UBM). It is generally accepted that the high reflectivity of the cyst wall is caused by its epithelial cell lining and that its sonolucent core is consistent with a liquid content.

The iris pigment epithelial cyst seen after dilation of the pupil.

Iridociliary cysts typically displace the iris root anteriorly. This can induce a focal plateau-iris configuration with or without angle-closure. Though single cysts are more common, multiple cysts are found in at least one third of cases. When multiple cysts involve more than 180 degrees of the iris, as it does in 10% of patients, angle-closure glaucoma may develop.

The natural history of iris pigment epithelial cysts is poorly understood. Therefore serial observation is warranted.

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14
Q

Melanocytoma

A

aka magnocellular nevus (benign proliferation of melanocytes)
Retina - Deeply pigmented tumor with feathery borders
No capsule, causing rapid growth, autoinfarction, and release of cells
Rarely malignant
=======================
Iris melanocytoma is a dark brown to black dome-shaped mass with little to no ectropion and often with a granular “mound of black sand” appearance. Occasionally there is minor seeding into the anterior chamber angle or onto the iris stroma.

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15
Q

How to differentiate iris nevus vs. melanoma:

A

size (

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16
Q

Test for iris melanoma

A

Iris FA (nevus has a filigree filling patter that becomes hyerpfluorescent early and leaks late or is angiographically silent vs. malignant melanoma has irregular vessels that fill late)

B-scan or UBM to R/O ciliary body involvement

transillumination

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17
Q

different presentations of iris malignant melanoma

A

Diffuse

Tapioca

Ring-shaped or localized

+/-Feeder vessels, involve angle structures, cause sectoral cataract, hyphema, IOP, or glaucoma

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18
Q

iris malignant melanoma Rx and Px

A

Rx: chemotherapy, XRT, complete surgical excision, enculeation depending on extent

Patient may need Rx of increased IOP

Px: good with mortality rate of

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19
Q

PSC cataract associations

A
V
I: age, atopic dermatitis, inflammation
T
A
M: RP, DM
I: ionizing radiation, steroid use
N
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20
Q

Complications of RBB block

A

central anesthesia
retrobulbar hemorrhage
globe penetration/perforation
strabismus (IR fibrosis or myotoxicity)

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21
Q

Aspiration Flow Rate

A

The amount of fluid flowing through the tubing. This is reported in cubic centimeters per minute (cc/min). With a peristaltic pump, flow is determined by the speed of the pump. As flow increases the current in the anterior chamber increases how well particulate matter is attracted to the phaco tip.

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22
Q

What to do to change phaco settings if complications

A

remove manually or with low-flow phaco settings
>lower the irrigation bottle height
>reduce AFR
>reduce vacuum

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23
Q

DDx of monocular diplopia

A
uncorrected refractive error
cataract
corneal pathology (irregular astigmatism 2/2 ABMD, scar, ectasia)
iris hole(s)
rarely macular pathology
24
Q

Irido-corneal Endothelial Syndrome

A

Abnormal proliferation of corneal endothelium (act like epithelial cells)
Cells cover angle causing glaucoma
May develop high PAS + angle closure
Unilateral, middle-age adults, females, NOT familial
Corneal endothelium has beaten bronze appearance
Spectral microscopy shows fewer cells with dark centers (light in PPMD)
Treatment: PKP treats corneal component, meds or surgery for glaucoma

25
Q

Iris nevus (Cogan-Reese)

A

flattening/effacement of iris stroma and pigmented tan iris nodules which are psuedonevi (2/2 normal iris cells taht are bunched up from the overlying membrane)
Less iris atrophy than latter type
correctopia
ectropion uveae

26
Q

Chandler syndrome

A
Corneal edema (often w/normal IOP)
Corneal findings predominate
Most common

mild or no iris changes - minimal correctopia, iris atrophy, PAS

27
Q

Essential iris atrophy

A

Iris atrophy, corectopia, polycoria, highbroad PAS, heterochromia, ectropion uveae

iris holes - either 2/2 maximal pull of endothelial membrane being stretched so thin that holes develop or melting holes (2/2 iris thinning due to iris ischemia)

28
Q

ICE vs mesodermal dysgenesis

A

ICE: Unilateral, middle-age adults, females, NOT familial (nonhereditary), progressive abnml of K endothelium not assoc/w/ any systemic abnormalities

Mesodermal dysgenesis: bilateral, congenital, hereditary

29
Q

mesodermal dysgenesis syndromes

A

PAX6 Neural Crest Dysgenesis

Axenfeld’s anomaly
Alagille syndrome
Rieger’s anomaly
Rieger’s syndrome
Peter's anomaly
Aniridia
30
Q

Axenfeld’s anomaly:

A
Posterior embryotoxon (anteriorly displaced Schwalbe's line) + iris processes to Schwalbe’s line/scleral spur
50% develop glaucoma
31
Q

Alagille syndrome:

A

Axenfeld + pigmentary retinopathy, corectopia, esotropia, ONH drusen,

Systemic abnml:
cholestasis/jaundice (biliary hypoplasia), congenital heart dx, flattened facies, bony abnormalities, absent DTRs

32
Q

Rieger’s anomaly:

A

Axenfeld + iris hypoplasia / holes

50% develop glaucoma

33
Q

Rieger’s syndrome:

A

Rieger’s anomaly + systemic:
Mental retardation, dental, craniofacial (maxillary hypoplasia), skeletal anomalies, pituitary abnormalities, hypospadias, loss of DTRs

34
Q

Peter’s Anomaly

A

Progressive central leukoma with clear periphery
Absent Descemet’s + endothelium with iris adhesions
Sporadic; 60% bilateral
50% develop glaucoma

Peters’ Plus (60% w/ systemic sx):
cardiac, craniofacial, skeletal anomalies

35
Q

Aniridia

A

85% familial (PAX6; AD)
13% sporadic, associated w/ WAGR (sporadic associated with large chromosomal deletions)
Wilm’s Tumor, Aniridia, Genitourinary, Retardation
2% Gillespie syndrome (AR)
MR + cerebellar ataxia
50-75% develop glaucoma

36
Q

Gillespie syndrome

A

aniridia, cerebellar ataxia and mental deficiency

37
Q

TASS

A

first 24-48 hours
diffuse K edema
fibrin in AC
low IOP (may be high later), and minimal or no vitritis

less redness, pain, and AC rxn than in endophthalmitis

Rx: topical steroids

38
Q

Post Cataract Endophthalmitis

A

94% 2/2 gram + bacteria

Mild
Staph epi (most common)

Severe
Staph aureus
Strep species
G(-) species

Late onset / Chronic
P acnes
white plaque on capsule
Staph epi / coag (-)

39
Q

Marfan syndrome

A

Inheritance / Gene?
AD (fibrillin; Ch 15)

Ocular findings?
Lens dislocation (superotemporal)
Myopia, retinal detachment

Systemic findings?
Tall, long limbs, hyperflexible joints

Life threatening problem?
Dilation of aorta (risk of dissection)

40
Q

Homocystinuria

A

Inheritance / Gene?
AR (Cystathionine β-synthase)

Elevated levels?
Homocystine and methionine

Ocular findings?
Lens dislocation (inferonasal)

Systemic findings?
Tall, osteoporosis, chest deformities
Seizures, mental retardation
Abnormalities develop after birth

Life threatening problem?
Thromboembolism esp. under GA

Treatment?
Low methionine, high cysteine diet

75% mortality by age 30 yo

41
Q

Weil-Marchesani

A

usually lens dislocated down
Small lens diameter (micherospherophakia), large AP diameter
High (lenticular) myopia

Can see microcornea, glaucoma (pupillary block)

Short, stubby fingers
mental retardation

42
Q

Hyperlysinemia

A

Lysine a-ketoglutarate reductase deficiency

Mental retardation w/ dislocated lenses

43
Q

Sulfite Oxidase Deficiency

A

Hemiplegia, athetosis
Death at age 5 with brain damage
Increased urine levels?
sulfite

44
Q

Cystinosis

A

Cystine crystals in anterior K stroma

3 Forms?
Infantile (AR, dwarfism, rickets, renal failure / Fanconi, death before puberty)

Adolescent (AR, similar but less severe),

Adult (?inheritance, asymptomatic)

Retinopathy only in infantile

Treatment?
Oral & topical cysteamine to stop crystals

45
Q

Ectopia Lentis et Pupillae

A

Inheritance: AR
Iris displacement inferotemporally OU
Lens dislocation superonasally OU
Microspherophakia

Miosis; poor dilation w/ mydriatic

46
Q

Factors that predispose to angel closure

A

small anterior segment (hyperopia, nanophthalmos, microK, microphthalmos)

anterior iris insertion (race)

Shallow AC (large lens, plateau iris configuration, loose or subluxed lens, psuedoexfoliation)

47
Q

Distinguishing between appositional and synchiae

A

Indentation gonioscopy with a zeiss-style 4 mirror lens.

Indenting the cornea forces aqueous fluid peripherally towards the angle.

If:
appositional = Angle opens
OR
Synchial = angle remains closed

48
Q

Provocative tests for angle closure

A

Prone test
Dark room test
prone-dark room test
pharmacologic pupillary dilation

Tests positive if IOP rises > 8 mm Hg

49
Q

Plateau iris syndrome

A

Anteriorly rotated ciliary processes, which push the peripheral iris forward - resulting in a deep chamber centrally and flat iris contour with a sharp drop-opp peripherally

Dilation causes peripheral iris to fold into the angle and occlude the TM. No pupillary block

No improvement with LPI
Treatment?
Gonioplasty (laser peripheral iridoplasty)
Long-term miotic (Pilocarpine 1%)

50
Q

Angle recession glaucoma

A

tear in the ciliary body between the longitudinal and circular fibers of the ciliary muscle

Initial Rx: topical meds

poor outcome with laser trabeculoplasty, so if gtts don’t work: trabeculectomy with anti-metabolite or glaucoma drainage implant

51
Q

NVI DDx

A
V: ocular ischemia (PDR, CRVO, carotid occlusive)
I: chronic inflammation
T: chronic RD, anterior semgnet ischemia
A
M: sickle cell retinopathy
I
N: tumors
52
Q

Rx for bullous keratopathy

A
aphakia
vitreocorneal touch
iridocorneal touch
severe or chronic keratitis
breaks in DM (i.e. birth trauma)
53
Q

DDx for flat bleb

A

Depends on IOP

low IOP - bleb leak or choroidal detachment

high IOP - suprachoroidal hemorrhage, pupillary block, malignant glaucoma

54
Q

AC difference between malignant glaucoma vs. angle-closure glaucoma

A

Angle closure: AC is deeper centrally than peripherally

Malignant glaucoma: entire AC is shallow

55
Q

Hypochromic iris

A
Horner syndrome (congenital)
Usually due to birth trauma to brachial plexus
Incontinentia pigmenti
Fuchs heterochromic iridocyclitis
Waardenburg-Klein syndrome
Hypertelorism; wide nose bridge
Cochlear deafness
White forelock; poliosis
Nonpigmented tumors
Hypomelanosis of Ito
JXG
56
Q

Hyperchromic iris

A
Oculodermal melanocytosis
Pigmented tumors
Siderosis
Iris ectropion syndrome
Extensive rubeosis
57
Q

Fuchs heterochromic iridocyclitis

A

Young, unilateral, chronic

Clinical findings?
Iris stromal atrophy (heterochromia)
Minimal cell / flare in AC +/- ant vit
Diffuse stellate KP

Amsler sign?
Heme during paracentesis from fine angle vessels  (not NV)
no PAS
Cataracts (cortical)
Glaucoma

Infectious cause?
Rubella, CMV
Associated with toxoplasmosis

Treatment?
No steroids