Iris Disorders Flashcards
Define Anisocoria
Unequal pupil size
What can cause Anisocoria?
Efferent nervous system defect
Physical lesion
Drugs (Pilocarpine, cocaine, tropicamide, scopolamine)
If the anisocoric pupil is the SMALLER one, how would it behave in dim and bright light; what is the most common cause?
Will not dilate in the dim light but can constrict in bright light;
Common with Horner’s Syndrome
If the anisocoric pupil is the LARGER one, how would it behave in dim and bright light; what is the most common cause?
Will not constrict in bright light but dilate in the dim light;
Oculomotor nerve palsy
What signs presenting with anisocoria could indicate blood/tumor/pathology in the brain; what is the treatment?
Aniscoria with: Confusion Decreased mental status Severe headache Emergency treatment, possibly neurosurgical
What is a Coloboma?
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
How can Coloboma present (in the iris)?
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
How do you treat Coloboma?
You don’t. Can use CLs for aesthetics
What is a Corectopia?
Displacement of the pupil form the central location
What is a pseudopolycoria?
More than one pupil
What are the four individual anterior chamber cleavage syndroms that make up Axenfeld-Rieger Syndrome?
Axenfeld's Anomaly Axenfeld's Ayndrome Rieger's Anomaly Rieger's Syndrome (Collapsed all four onto a single spectrum hence the new name)
What is Axenfeld’s Anomaly?
Peripheral anterior segment defects (posterior embryotoxon and peripheral anterior synechiae)
What is Rieger’s Anomaly?
Axenfeld’s anomaly with iris/pupil abnomalities (decentered pupil or multiple)
What is Rieger’s syndrome?
Ocular anomalies and systemic developmental defects (dental, craniofacial and skeletal) as well
Describe the presentation of Axenfeld-Rieger Syndrome
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
What is Aniridia?
Absence of the iris
Rare bilateral condition
Mutation on neuroectoderm gene PAX6
See corneal, lenticular and fundus changes
What symptoms can present in Aniridia?
PHOTOPHOBIA
Nystagmus
Lowered VA
Strabismus
What are the three classes of aniridia?
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
How can Aniridia affect the anterior segment?
K Opacity
Microcornea
Dermoids
Sclerocornea
How can Aniridia affect the lens?
Subluxation
Opacity
Absence
Persistent Pupillary Membrane
How can Aniridia affect the fundus?
Foveal hypoplasia
Optic nerve hypoplasia
Choroidal coloboma
How else can Aniridia affect ocular structures?
Nystagmus
Mental retardation
Secondary glaucoma (75%)
Describe Ectropion Uvea
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
Describe the two kinds of Heterochromia
Heterochromium Iridium - Unilateral, one iris has more than one color
Heterochromia irides - bilateral, eahc iris is a different color
Describe Hypo and Hyperchromia
Hypochromia - Congenital, Horner’s Syndrome
Hyperchromia - Naevus of Ota, Ocular siderosis, diffuse nevus or melanoma, Sturge-Webeter syndrome, Latanoprost (drug)
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
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
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
In cases of inflammation flare up in Fuch’s Heterochromic Iridocyclitis would long-term/high-dose topical corticosteroids be beneficial?
No
What is the heterochromic triad seen with Fuch’s heterochromic iridocyclitis?
Iritis
Heterochromia
Cataract
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
What happens to the iris color in Fuch’s Heterochromic Iridocystitis?
Browns are less brow
Blues are more saturated blues
What condition can occur secondary to Fuch’s Heterochromic Iridocyclitis?
Secondary glaucoma
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
How does a new keratic precipitate look? How does an old one look?
New - White, smooth rounded
Old - shrunken, pigmented or glassy
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
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
What conditions can cause Rubeosis Irides?
Diabetic retionpathy
Central Retinal Vein Occlusion
The scarring and contraction of the abnormal vessels seen in Rubeosis Irides can cause what?
Acquired ectropion uvea
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
What other subcategories comprises Iridocorneal-Endothelial Syndrome? (ICE)
Essential/Progressive Iris Atrophy
Chandler Syndrome
Iris-Nevus (Cogan-Reese) Syndrome
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
What is the term describing the movement of corneal endothelial cells to the iris?
Proliferative endo-epithelialopathy
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
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
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
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
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
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
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
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
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
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
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
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)
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
What is a very characteristic clinical appearance for an iris melanoma that is growing either anterior or posterior?
“Lion’s Paw appearance”
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
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
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
Describe an Iris cyst
Hyperpigmentation and hypertrophy of iris pigmented epithlium on posterior iris
Uncommon unilateral lesiosn that can be primary or secondary
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
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)
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
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
Describe Albinism
Defect of melanin production resulting in little or no color in skin, hair and eyes
What symptoms are common in albinism?
Absence of color in hair, skin or iris
Lighter than normal skin and hair
Patchy, missing skin color
What ocular symptoms show in albinism?
Crossed eye (strabismus) Light sensitivity (photophobia) Rapid eye movement (nystagmus( VA issues, even functional blindness
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
What are the kinds of albinism?
Oculocutaneous (Tyrosinase negative and positive)
Ocular albinism
Autosomal recessive
Describe oculocutaneous albinism, tyrosinase negative
Unable to produce melanin and a complete albino (complete iris transillumination is pink, low VA, photophobia, pendular horizontal nystagmus)
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
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
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
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
Should you treat the inflammation in iris nodules what happens to the nodules?
When inflammation is treated nodules resolve
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
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
What are Brushfield Spots?
Gray or brown spots on iris associated with mongolism (Down’s) also in many normal kids
What is an Iridodialysis?
A tear in the iris
What is Iridodonesis?
Tremulousness of the iris on movement of the yee occuring in subluxation of the lens (jiggling iris)
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
