Iris Disorders Flashcards

(82 cards)

1
Q

Define Anisocoria

A

Unequal pupil size

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2
Q

What can cause Anisocoria?

A

Efferent nervous system defect
Physical lesion
Drugs (Pilocarpine, cocaine, tropicamide, scopolamine)

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3
Q

If the anisocoric pupil is the SMALLER one, how would it behave in dim and bright light; what is the most common cause?

A

Will not dilate in the dim light but can constrict in bright light;
Common with Horner’s Syndrome

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4
Q

If the anisocoric pupil is the LARGER one, how would it behave in dim and bright light; what is the most common cause?

A

Will not constrict in bright light but dilate in the dim light;
Oculomotor nerve palsy

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5
Q

What signs presenting with anisocoria could indicate blood/tumor/pathology in the brain; what is the treatment?

A
Aniscoria with:
Confusion
Decreased mental status
Severe headache
Emergency treatment, possibly neurosurgical
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6
Q

What is a Coloboma?

A

Tissue cleft/defect, can be any structure in the eye (iris/lid are what we’ve seen)
AD; Caused by incomplete fusion of fetal fissure during gestation

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7
Q

How can Coloboma present (in the iris)?

A
Iris is still full thickness
Bilateral and tends to be inferiorly located
Defect lined by pupillary ruff
NO impact on VA/accommodation
May lead to lens subluxation
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8
Q

How do you treat Coloboma?

A

You don’t. Can use CLs for aesthetics

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9
Q

What is a Corectopia?

A

Displacement of the pupil form the central location

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10
Q

What is a pseudopolycoria?

A

More than one pupil

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11
Q

What are the four individual anterior chamber cleavage syndroms that make up Axenfeld-Rieger Syndrome?

A
Axenfeld's Anomaly
Axenfeld's Ayndrome
Rieger's Anomaly
Rieger's Syndrome
(Collapsed all four onto a single spectrum hence the new name)
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12
Q

What is Axenfeld’s Anomaly?

A

Peripheral anterior segment defects (posterior embryotoxon and peripheral anterior synechiae)

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13
Q

What is Rieger’s Anomaly?

A

Axenfeld’s anomaly with iris/pupil abnomalities (decentered pupil or multiple)

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14
Q

What is Rieger’s syndrome?

A

Ocular anomalies and systemic developmental defects (dental, craniofacial and skeletal) as well

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15
Q

Describe the presentation of Axenfeld-Rieger Syndrome

A

Bilateral posterior embryotoxon with iris strands attached
Peripheral anterior synechiae and hypoplasia of anterior iris stroma
Iris atrophy and pupil misshapen (discoria)
Dental/craniofacial/skeletal abnormalities
CHECK FOR GLAUCOMA

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16
Q

What is Aniridia?

A

Absence of the iris
Rare bilateral condition
Mutation on neuroectoderm gene PAX6
See corneal, lenticular and fundus changes

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17
Q

What symptoms can present in Aniridia?

A

PHOTOPHOBIA
Nystagmus
Lowered VA
Strabismus

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18
Q

What are the three classes of aniridia?

A

AN-1 (isolated): AD and 85% of cases
AN-2 (Miller syndrome): 13% of cases and associated with Wilm’s tumor, genitourinary anomalies and MR
AN-3 (Gillespie syndrome): AR and 2% of cases, see mental handicaps and cerebellar ataxia

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19
Q

How can Aniridia affect the anterior segment?

A

K Opacity
Microcornea
Dermoids
Sclerocornea

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20
Q

How can Aniridia affect the lens?

A

Subluxation
Opacity
Absence
Persistent Pupillary Membrane

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21
Q

How can Aniridia affect the fundus?

A

Foveal hypoplasia
Optic nerve hypoplasia
Choroidal coloboma

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22
Q

How else can Aniridia affect ocular structures?

A

Nystagmus
Mental retardation
Secondary glaucoma (75%)

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23
Q

Describe Ectropion Uvea

A

Condition in which posterior iris epithelium curls up past the pupillary margin to move onto anterior iris
Congenital - Rare, nonprogressive, pigmented posterior iris epithelium extending over anterior iris; one or both eyes, follow for glaucoma
Acquired, most common presentation, secondary to retinal ischemia or inflammation

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24
Q

Describe the two kinds of Heterochromia

A

Heterochromium Iridium - Unilateral, one iris has more than one color
Heterochromia irides - bilateral, eahc iris is a different color

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25
Describe Hypo and Hyperchromia
Hypochromia - Congenital, Horner's Syndrome | Hyperchromia - Naevus of Ota, Ocular siderosis, diffuse nevus or melanoma, Sturge-Webeter syndrome, Latanoprost (drug)
26
Describe Heterochromia Irides in more detail
Heterochromia where both eyes are different colors Congenital - Associated with cranio-dystosis (Crouzon's Dx) Acquired - Trauma, surgery, inflammation, foreign body, Fuch's Heterochromic Iridocyclitis, Horner's syndrome
27
Describe Fuch's Heterochromic Iridocyclitis
A chronic nongranulomatous anterior UVEITIS in the lighter colored eye Bilateral and affects 20-60 years old Often misdiagnosed or mistreated
28
What is the history of a patient with Fuch's Heterochromic Iridocyclitis?
Floaters from vitreous inflammation/Lower VA secondary to cataracts Low-grade inflammation usually persists over many years and needs no treatment Occassional flare up of inflammation to a moderate level with short term topical corticosteroid therapy
29
In cases of inflammation flare up in Fuch's Heterochromic Iridocyclitis would long-term/high-dose topical corticosteroids be beneficial?
No
30
What is the heterochromic triad seen with Fuch's heterochromic iridocyclitis?
Iritis Heterochromia Cataract
31
What other signs can be seen with Fuch's Heterochromic Iridocyclitis?
Keratic precipitates: pathognomonic (very characteristic to this disease) Diffuse iris stromal atrophy; loss of crypts Prominent radial blood supply Iris nodules, pupillary bolder (Koeppe nodules) and in the iris stroma (Busacca nodules), rubeosis, mydriasis
32
What happens to the iris color in Fuch's Heterochromic Iridocystitis?
Browns are less brow | Blues are more saturated blues
33
What condition can occur secondary to Fuch's Heterochromic Iridocyclitis?
Secondary glaucoma
34
Describe Keratic precipitates
Inflammatory cells with white blood cells; from the iris and ciliary body These enter the aqueous and adhere to the cornea KP's large clusters: granulomatous "mutton fat" KP's punctate: nongranulomatous, may be inferior or diffuse
35
How does a new keratic precipitate look? How does an old one look?
New - White, smooth rounded | Old - shrunken, pigmented or glassy
36
Describe a Persistent Pupillary Membrane (PPM)
Congenital - the remnants of tunica vasculosa lentis Wispy, silky (spider web) appearance Benign and can fade with age NO VA EFFECT
37
Describe Rubeosis Irides
Neovascularization of the iris due to retinal ischemia (release of chemical mediators) Vessels grow from the peripheral retina and migrate across the iris posteriorly-anteriorly
38
What conditions can cause Rubeosis Irides?
Diabetic retionpathy | Central Retinal Vein Occlusion
39
The scarring and contraction of the abnormal vessels seen in Rubeosis Irides can cause what?
Acquired ectropion uvea
40
Describe (Secondary) Iris Atrophy
Localized or diffuse Mainly due to AGING, trauma, surgery, infections Areas of stromal thinning and See depigmentation Leads to corneal pigment dusting and Secondary Glaucoma
41
What other subcategories comprises Iridocorneal-Endothelial Syndrome? (ICE)
Essential/Progressive Iris Atrophy Chandler Syndrome Iris-Nevus (Cogan-Reese) Syndrome
42
Describe Iridocorneal-Endothelial Syndrome
Rare unilateral with a slow progression; Females affected more than males (2:1) Seen in young to middle aged It is an abnormal corneal endothelial layer with movement of endothelial cells off the cornea onto the iris
43
What is the term describing the movement of corneal endothelial cells to the iris?
Proliferative endo-epithelialopathy
44
How does Iridocorneal-Endothelial Syndrome present?
``` Glaucoma in half of cases (clogged TM) Iris Atrophy Peripheral Anterior Synechiae Iris Nodules Corneal endothelial problems/edema Corectopia (distortion of pupil) Pseudopolycoria Ectropion uvea ```
45
Describe Essential/Progressive Iris Atrophy
Unilateral low-grade inflammatory reaction over 1-3 years Rare and mainly in women in their 30s Severe stromal atrophy - Iris holes (seen with retroillumination) Peripheral anterior synchiea can develop Secondary glaucoma or corneal endothelial problems
46
Describe Chandler Syndrome
A less severe essential iris atrophy and is the most commonly seen subcategory of iris atrophy; PAS with severe corneal changes (silver hammered appearance) Blurred VA and halos secondary to edema 10-25% develop unilateral glaucoma Mild or moderate corectopia Stromal atrophy variable GREATER corneal changes/edema but fewer iris abnormalities
47
Describe Iris Nevus Syndrome (Cogan-Reese)
Very rare Diffuse/multiple pigmented or pedunculated iris nodules/nevi Iris atrophy in half of these cases that can be mild to moderate Appearance is smudged or matted Maybe see some diffuse iris melanoma
48
How do you treat ICE Syndromes?
CASE SPECIFIC and determined by degree of corneal edema and severity of the glaucoma Topical aqueous suppressants to manage the glaucoma (beta blockers?) Despite adequate IOP control, corneal edema may persist due to endotheliopathy and require PKP
49
What are some other notes for ICE syndrome?
The three subcategories are all related Progression is unpredictable but many have good outcomes The iris is dragged in the direction of the PAS
50
Describe Iridoschisis
Rare bi lateral condition that is AGE related (60s) Stromal splitting, splitting of anterior (mesodermal) stroma of iris into two layers with fibrils of anterior layer floating in the aqueous Usually inferior iris affected Associated with angle closure glaucoma (90%) secondary to a high IOP MONITOR
51
Describe Iris Nevi
Common hyperpigmentated areas and usually flat Single or multiple and will vary in size, shape, height or color Typically less than 3mm in diameter Mild to no vascularization or pupil distortion
52
Describe an Iris melanoma
``` More common with light irises (3:1) Tend to be inferior iris and are slow growing with neovascularization and pupil distortion (see ectropion irides) Secondary glaucoma and cataracts Low mortality rate (spindle cells) 8% of uveal melanomas ```
53
Describe the aggression of an Iris Melanoma
Most primary tumors of the iris are benign Much less aggressive than melanoma in the choroid or ciliary body Mortality rate is up to 11% depending on metastses and CB involvement Metastases is 2-10% higher if the CB is involved
54
Describe the incidence and presentation of Iris melanomas
No sexual preference, average age of onset is 40-50 years Pigmented or non-pigmented At least 3 mm in daimeter and 1mm in thickness and can be smooth or irregular
55
What is the history for an Iris Melanoma?
Nevus that's been there since childhood and has suddenly started to grow (patient has cosmetic concerns and maybe pain due to increased IOP)
56
What would the clinical presentation of an Iris Melanoma be?
Circumscribed or diffuse nodular shaped melanomas commonly on the inferior iris Can grow anterior into AC or posterior into posterior chamber (Involving AC can invade CB) Evaluate with gonio and ultrasound biomicroscope
57
What is a very characteristic clinical appearance for an iris melanoma that is growing either anterior or posterior?
"Lion's Paw appearance"
58
Describe a diffuse iris melanoma
Can cause acquired heterochromia Associated with glaucoma that responds poorly to treatment, see severe disc cupping and VA loss Higher risk of a metastasis than circumscribed
59
Discribe the objective tests to evaluate an iris melanoma
``` Transillumination Slit lamp with a measurement of nodule Indirect exam with scleral depression Gonio Photography Ultrasound biomicroscopy ```
60
What is the Dx for an iris melanoma?
``` Iris nevus CIliary body melanoma Metastases from lung or breast Primary iris cyst Adenoma of iris pigment epithelium ```
61
Describe an Iris cyst
Hyperpigmentation and hypertrophy of iris pigmented epithlium on posterior iris Uncommon unilateral lesiosn that can be primary or secondary
62
Describe a primary iris cyst
Arise from iris pigmented epithelium/stroma Mostly stationary and asymptomatic Epithelial cyst - Globular, dark brown and seen with a transillumination, can be on the pupil border, midzone or peripheral Stromal cyst - Very rare and tends to be in children, clear anterior wall and are fluid filled
63
Describe a secondary iris cyst
S/P surgery, ocular trauma or long acting miotics FLuid fild Clear anterior wall Tend to enlarge (uveitis and glaucoma)
64
How to diagnose an iris cyst?
High frequency ultrasonography reveals internal echolucency (fluid filled) Large cysts can be seen by slit-lamp examination (take a photo) Periodic observation necessary due to the rare instances of secondary angle closure glaucoma and synchronous ciliary body neoplasia
65
How to treat an iris cyst?
Typically no treatment If performed is for rare instances of secondary glaucoma or when visual axis is blocked Laser has been used to delfate iris pigment epithelial cysts with minimal side effects Periodic observation warrented
66
Describe Albinism
Defect of melanin production resulting in little or no color in skin, hair and eyes
67
What symptoms are common in albinism?
Absence of color in hair, skin or iris Lighter than normal skin and hair Patchy, missing skin color
68
What ocular symptoms show in albinism?
``` Crossed eye (strabismus) Light sensitivity (photophobia) Rapid eye movement (nystagmus( VA issues, even functional blindness ```
69
How can you treat albinism?
Relieve the symptoms and the treatment depends on severity Protect skin and eyes from the sun (sunscreen, cover up and UV protecting sunglasses to deal with photophobia) Genetic testing and counseling
70
What are the kinds of albinism?
Oculocutaneous (Tyrosinase negative and positive) Ocular albinism Autosomal recessive
71
Describe oculocutaneous albinism, tyrosinase negative
Unable to produce melanin and a complete albino (complete iris transillumination is pink, low VA, photophobia, pendular horizontal nystagmus)
72
Describe oculocutaneous albinism, tyrosinase positive
Some melanin is produced, fair to normal complexion, blue/dark brown iris with variable transillumination May or may not have lower VA or nystagmus
73
What other physical signs can occur with albinism?
Abnormal corssing of temporal fibers in optic chiasm causing lower VA Difficulty with healing and infections Ask if the patient is easily bruised, nose bleeds or has hearing loss
74
Describe Ocular Albinism
X-linked or AR Involvement of just the eye and no hair/skin Peripheral RPE affected causing lower VF Female carries are asymptomatic, normal VA, partial iris transillumination and maybe macular stippling
75
Describe the inflammatory Iris Nodule case: Koeppe Nodules
Features of granulomatous inflammation Koeppe nodules - small in size at the pupil border (inflammatory, cell precipitates and may also be found in non-granulomatous uveitis) Busacca noduels are less common on mid iris surface
76
Should you treat the inflammation in iris nodules what happens to the nodules?
When inflammation is treated nodules resolve
77
Describe the inflammatory Iris Nodule case: Lisch Nodules
Lisch nodules - small bilateral melanocytic hamartomas (benign tumor); neurofibromatosis type I, presnt in greater than 94% of patients over 6 year,s clear, yellow brown oval to round dome-shaped papules that project form the surface of the iris and do not affect VA
78
Describe the inflammatory Iris Nodule case: Iris Mamillations
Rare, tiny regular spaced villiform (tiny nipple like) elevated iris lesions with uniform height Overlie areas of hyperpigmented iris Partially or totalyl cover anterior surface of iris Neurofibromatosis I Usually unilateral and occurs sporadically Asyomptomatic and may cause higher IOP
79
What are Brushfield Spots?
Gray or brown spots on iris associated with mongolism (Down's) also in many normal kids
80
What is an Iridodialysis?
A tear in the iris
81
What is Iridodonesis?
Tremulousness of the iris on movement of the yee occuring in subluxation of the lens (jiggling iris)
82
What is Iris Bombe?
Occurs in posterior annular synechia in which an increase of fluid in posterior chamber causes a forward bulging of peripheral iris