Iris Iris TransilluminatioTransilluminatio

n Defectsn Defects

Differential DxDifferential Dx PseudoexfoliationPseudoexfoliation Pigment Dispersion SyndromePigment Dispersion Syndrome Pseudophakic ChafingPseudophakic Chafing ICE (Iridocorneal Endothelial) SyndromeICE (Iridocorneal Endothelial) Syndrome

Iris Nevus Syndrome (Cogan-Reese)Iris Nevus Syndrome (Cogan-Reese) Chandler SyndromeChandler Syndrome *Essential Iris Atrophy*Essential Iris Atrophy

Infectious (HZV, HSV)Infectious (HZV, HSV) AlbinismAlbinism Axenfeld-RiegerAxenfeld-Rieger Normal Variant (lightly pigmented irides)Normal Variant (lightly pigmented irides) Post surgery/trauma/uveitis (patchy)Post surgery/trauma/uveitis (patchy)

PseudoexfoliationPseudoexfoliation

PseudoexfoliationPseudoexfoliation

Peripupillary TIDsPeripupillary TIDs Fibrillar material Fibrillar material

deposition on lens deposition on lens capsule, pupil capsule, pupil margin, CB, iris, margin, CB, iris, subconj, and subconj, and systemicallysystemically

TM pigmentatoin, TM pigmentatoin, Sampaolesi line (ant Sampaolesi line (ant to Schwalbe line)to Schwalbe line)

Phacodonesis – Phacodonesis – weak zonulesweak zonules

Strongly age Strongly age relatedrelated(rare <50, usually (rare <50, usually

>70)>70) +/- glaucoma+/- glaucoma

responds well to responds well to SLT/ALT but wears SLT/ALT but wears offoff

Pigment Dispersion Pigment Dispersion SyndromeSyndrome

Pigment Dispersion Pigment Dispersion SyndromeSyndrome

SignsSigns Mid-peripheral iris Mid-peripheral iris

TIDs, radial slit-like TIDs, radial slit-like patternpattern

K-spindleK-spindle Heavily pigmented Heavily pigmented

TMTM More common in More common in

younger malesyounger males 25-50% progress to 25-50% progress to

glaucomaglaucoma

Treatment Treatment Considerations:Considerations:

Responds well to Responds well to ALT/SLTALT/SLT

LPI controversial LPI controversial (reduce irido-(reduce irido-zonular contact)zonular contact)

ICEICE Iridocorneal Endothelial SyndromeIridocorneal Endothelial Syndrome

IIris Nevus Syndrome (Cogan-Reese)ris Nevus Syndrome (Cogan-Reese) Multiple pigmented iris nodulesMultiple pigmented iris nodules

CChandler Syndromehandler Syndrome Corneal edemaCorneal edema

EEssential Iris Atrophyssential Iris Atrophy Iris atrophy, corectopia, polycoriaIris atrophy, corectopia, polycoria

Variably associated with glaucomaVariably associated with glaucoma Unilateral, middle-aged femalesUnilateral, middle-aged females

ICEICE

HSV keratouveitisHSV keratouveitis Patchy TIDs Patchy TIDs

(vs. sectoral in VZV)(vs. sectoral in VZV) Elevated IOPElevated IOP +/- stromal keratitis+/- stromal keratitis HEDS HEDS trendtrend of benefit of benefit

if given oral acyclovirif given oral acyclovir Not statistically Not statistically

significantsignificant small sample size small sample size

(n=50)(n=50) 400mg 5x/day x 10 wks400mg 5x/day x 10 wks

In patients also In patients also receiving topical receiving topical steroidssteroids

Albinism . Albinism . Oculocutaneous: low Oculocutaneous: low

melanin per melanin per melanosomemelanosome

Ocular: low # Ocular: low # melanosomesmelanosomes

Albinism patternAlbinism pattern Decr Va/+nystagmusDecr Va/+nystagmus Foveal hypoplasiaFoveal hypoplasia

Albinoidism patternAlbinoidism pattern nl Va/no nystagmusnl Va/no nystagmus

SyndromesSyndromes Hermansky-Pudlak: easy Hermansky-Pudlak: easy

bleeding/bruisingbleeding/bruising Chediak-Higashi: PMN Chediak-Higashi: PMN

abnormality, susceptible abnormality, susceptible to infectionsto infections

TIDsTIDs Hypopigmented Hypopigmented

fundusfundus PhotophobiaPhotophobia

AniridiaAniridia

PAX6 gene PAX6 gene mutationmutation Autosomal Autosomal

dominant vs. dominant vs. sporadicsporadic

Wilms tumor or Wilms tumor or WAGRWAGR

Associated with: Associated with: nystagmus, foveal nystagmus, foveal hypoplasia, K hypoplasia, K opacification, opacification, glaucoma, ON glaucoma, ON hypoplasia, cataracthypoplasia, cataract

Axenfeld-RiegerAxenfeld-Rieger

Spectrum of developmental disorders Spectrum of developmental disorders with anteriorly displaced Schwalbe’s linewith anteriorly displaced Schwalbe’s line

Iris strands, variable iris hypoplasia Iris strands, variable iris hypoplasia mild thinning to marked atrophy with holes, mild thinning to marked atrophy with holes,

corectopia, ectropion uveaecorectopia, ectropion uveae May have small or few teeth, May have small or few teeth,

hypospadias, redundant umbilical skin, hypospadias, redundant umbilical skin, micro/macrocorneamicro/macrocornea

50% glaucoma50% glaucoma

Axenfeld-RiegerAxenfeld-Rieger