Friedman Anterior segment Flashcards
Pigment Dispersion Syndrome (PDS) demographic
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%)
Pigment Dispersion Syndrome (PDS) signs and Rx?
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)
Reverse pupillary block
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
Mechanism of Pigment Dispersion Syndrome glaucoma
iris pigment obstructs the TM causing elevated IOP and ON damage
natural course of Pigment Dispersion Syndrome glaucoma
burns itself out once all pigment has been liberated (no more pigment to clog TM and raise IOP)
How to confirm ciliary body tumor (imaging/findings)
Imaging:
Use UBM
Anterior segment OCT
scheimpflug imaging
Findings: lenticular astigmatism and cataract shallow anterior chamber sentinel vessel extrascleral extension
Rx of ciliary body tumor
depending on extent of tumor:
Rx is with surgical excision, chemoRx, XRT or enucleation
DDx for non-granulomatous iritis
idiopathic HLA-B27 Fuchs heterochromic iridocyclitis HSV Posner-Schlossman syndrome Lyme Behcet's disease drugs interstitial nephritis
Work-up for non-granulomatous iritis
HLA-B27 sacroiliac X-ray CBC with diff UA VDRL/RPR and FTA-ABS
Disorders assoc/w/HLA-B27 iritis
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
Whipple’s disease
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.)
Pigmented iris lesion DDx
Nevus Melanocytoma melanoma iris pigment epithelial tumor rarely mtz (usually amelanotic)
Iris pigment epithelial cyst
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.
Melanocytoma
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
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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.
How to differentiate iris nevus vs. melanoma:
size (
Test for iris melanoma
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
different presentations of iris malignant melanoma
Diffuse
Tapioca
Ring-shaped or localized
+/-Feeder vessels, involve angle structures, cause sectoral cataract, hyphema, IOP, or glaucoma
iris malignant melanoma Rx and Px
Rx: chemotherapy, XRT, complete surgical excision, enculeation depending on extent
Patient may need Rx of increased IOP
Px: good with mortality rate of
PSC cataract associations
V I: age, atopic dermatitis, inflammation T A M: RP, DM I: ionizing radiation, steroid use N
Complications of RBB block
central anesthesia
retrobulbar hemorrhage
globe penetration/perforation
strabismus (IR fibrosis or myotoxicity)
Aspiration Flow Rate
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.
What to do to change phaco settings if complications
remove manually or with low-flow phaco settings
>lower the irrigation bottle height
>reduce AFR
>reduce vacuum
DDx of monocular diplopia
uncorrected refractive error cataract corneal pathology (irregular astigmatism 2/2 ABMD, scar, ectasia) iris hole(s) rarely macular pathology
Irido-corneal Endothelial Syndrome
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
Iris nevus (Cogan-Reese)
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
Chandler syndrome
Corneal edema (often w/normal IOP) Corneal findings predominate Most common
mild or no iris changes - minimal correctopia, iris atrophy, PAS
Essential iris atrophy
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)
ICE vs mesodermal dysgenesis
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
mesodermal dysgenesis syndromes
PAX6 Neural Crest Dysgenesis
Axenfeld’s anomaly Alagille syndrome Rieger’s anomaly Rieger’s syndrome Peter's anomaly Aniridia
Axenfeld’s anomaly:
Posterior embryotoxon (anteriorly displaced Schwalbe's line) + iris processes to Schwalbe’s line/scleral spur 50% develop glaucoma
Alagille syndrome:
Axenfeld + pigmentary retinopathy, corectopia, esotropia, ONH drusen,
Systemic abnml:
cholestasis/jaundice (biliary hypoplasia), congenital heart dx, flattened facies, bony abnormalities, absent DTRs
Rieger’s anomaly:
Axenfeld + iris hypoplasia / holes
50% develop glaucoma
Rieger’s syndrome:
Rieger’s anomaly + systemic:
Mental retardation, dental, craniofacial (maxillary hypoplasia), skeletal anomalies, pituitary abnormalities, hypospadias, loss of DTRs
Peter’s Anomaly
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
Aniridia
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
Gillespie syndrome
aniridia, cerebellar ataxia and mental deficiency
TASS
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
Post Cataract Endophthalmitis
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 (-)
Marfan syndrome
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)
Homocystinuria
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
Weil-Marchesani
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
Hyperlysinemia
Lysine a-ketoglutarate reductase deficiency
Mental retardation w/ dislocated lenses
Sulfite Oxidase Deficiency
Hemiplegia, athetosis
Death at age 5 with brain damage
Increased urine levels?
sulfite
Cystinosis
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
Ectopia Lentis et Pupillae
Inheritance: AR
Iris displacement inferotemporally OU
Lens dislocation superonasally OU
Microspherophakia
Miosis; poor dilation w/ mydriatic
Factors that predispose to angel closure
small anterior segment (hyperopia, nanophthalmos, microK, microphthalmos)
anterior iris insertion (race)
Shallow AC (large lens, plateau iris configuration, loose or subluxed lens, psuedoexfoliation)
Distinguishing between appositional and synchiae
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
Provocative tests for angle closure
Prone test
Dark room test
prone-dark room test
pharmacologic pupillary dilation
Tests positive if IOP rises > 8 mm Hg
Plateau iris syndrome
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%)
Angle recession glaucoma
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
NVI DDx
V: ocular ischemia (PDR, CRVO, carotid occlusive) I: chronic inflammation T: chronic RD, anterior semgnet ischemia A M: sickle cell retinopathy I N: tumors
Rx for bullous keratopathy
aphakia vitreocorneal touch iridocorneal touch severe or chronic keratitis breaks in DM (i.e. birth trauma)
DDx for flat bleb
Depends on IOP
low IOP - bleb leak or choroidal detachment
high IOP - suprachoroidal hemorrhage, pupillary block, malignant glaucoma
AC difference between malignant glaucoma vs. angle-closure glaucoma
Angle closure: AC is deeper centrally than peripherally
Malignant glaucoma: entire AC is shallow
Hypochromic iris
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
Hyperchromic iris
Oculodermal melanocytosis Pigmented tumors Siderosis Iris ectropion syndrome Extensive rubeosis
Fuchs heterochromic iridocyclitis
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
