iris trans illumination defects
Post on 10-Mar-2015
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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
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