1 Pediatric Cysts and Nodules

Pediatric Iris Cysts Basic clinical distinction ? ? 2 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts Basic clinical distinction Primary Secondary 3 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Basic anatomic distinction ? ? 4 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Basic anatomic distinction Epithelial Stromal 5 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Which is more common--epithelial, or stromal cysts? Epithelial, by a mile 6 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Which is more common--epithelial, or stromal cysts? Epithelial, by a mile 7 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Advanced anatomic distinction ? ? 8 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Advanced anatomic distinction Pupillary Iris pigment cysts epithelium cysts 9 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Pupillary Iris pigment cysts epithelium cysts

Pupillary cysts --Pigment epi cysts at the…[location] --Presents in…infancy 10 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Pupillary Iris pigment cysts epithelium cysts

Pupillary cysts --Pigment epi cysts at the… border --Presents in…infancy 11 Pediatric Iris Cysts and Nodules

Primary epithelial pupillary cysts 12 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Pupillary Iris pigment cysts epithelium cysts

Pupillary cysts --Pigment epi cysts at the…pupil border --Presents in…[age] 13 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Pupillary Iris pigment cysts epithelium cysts

Pupillary cysts --Pigment epi cysts at the…pupil border --Presents in…infancy 14 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Pupillary Iris pigment cysts epithelium cysts

Iris pigment epithelium Pupillary cysts cysts --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers --Presents in…infancy --Presents in…in teensteens --Clinically significant? No[Y/N] 15 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Pupillary Iris pigment cysts epithelium cysts

Iris pigment epithelium Pupillary cysts cysts --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers --Presents in…infancy --Presents in…in teensteens --Clinically significant? No[Y/N] 16 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Pupillary Iris pigment cysts epithelium cysts

Iris pigment epithelium Pupillary cysts cysts --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers --Presents in…infancy --Presents in…[agein…teens range] --Clinically significant? No[Y/N] 17 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Pupillary Iris pigment cysts epithelium cysts

Iris pigment epithelium Pupillary cysts cysts --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers --Presents in…infancy --Presents in…teens --Clinically significant? No[Y/N] 18 Pediatric Iris Cysts and Nodules

Primary epithelial pupillary cysts in a teen 19 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Pupillary Iris pigment cysts epithelium cysts

Iris pigment epithelium Pupillary cysts cysts --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers --Presents in…infancy --Presents in…teens --Clinically significant? [Y/N] 20 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

PupillaryPupillary IrisIris pigmentpigment cystscysts epitheliumepithelium cystscysts

Iris pigment epithelium Pupillary cysts cysts --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers --Presents in…infancy --Presents in…teens --Clinically significant? [Y/N]No 21 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… -- the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 22 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 23 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…[age] infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 24 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 25 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy epithelial --Cysts contain…gobletcell type cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 26 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 27 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separationWhy does of… the presence --Glaucomaof goblet cells predispose cysts to enlargement? the…pupil border epithelialRecall layers that goblet cells produce--Induce…corneal mucin . The decompensation production (and sequestration) --Presents in…infancy --Presentsof in… mucinteens that is responsible--Cyst for leakagecyst enlargement.  chronic iritis --Clinically significant? No[Y/N] 28 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separationWhy does of… the presence --Glaucomaof goblet cells predispose cysts to enlargement? the…pupil border epithelialRecall layers that goblet cells produce--Induce…corneal mucinone word . The decompensation production (and sequestration) --Presents in…infancy --Presentsof in… mucinsameteens word that is responsible--Cyst for leakage cyst enlargement.  chronic iritis --Clinically significant? No[Y/N] 29 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separationWhy does of… the presence --Glaucomaof goblet cells predispose cysts to enlargement? the…pupil border epithelialRecall layers that goblet cells produce--Induce…corneal mucin . The decompensation production (and sequestration) --Presents in…infancy --Presentsof in… mucinteens that is responsible--Cyst for leakage cyst enlargement.  chronic iritis --Clinically significant? No[Y/N] 30 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…[wo words] --Pigment epi cysts at --2o to separation of… --Secondary… the…pupil border epithelial layers --Induce… --Presents in…infancy --Presents in…teens --Cyst leakage  --Clinically significant? [Y/N]No 31 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 32 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers Are such--Induce…corneal cysts potentially decompensation amblyogenic? --Presents in…infancy --Presents in…teens Indeed--Cyst they leakage are  chronic iritis --Clinically significant? No[Y/N] 33 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers Are such--Induce…corneal cysts potentially decompensation amblyogenic? --Presents in…infancy --Presents in…teens Indeed--Cyst they leakage are  chronic iritis --Clinically significant? No[Y/N] 34 Pediatric Iris Cysts and Nodules

Stromal cyst in an infant, obstructing visual axis 35 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…[bad condition] the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 36 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 37 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…[ issue—two words] --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 38 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 39 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  [two words] --Clinically significant? [Y/N]No 40 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 41 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

Epithelial Stromal

Stromal cysts Because--Due to stromalof these sequestration issues, stromal of…epithelial cysts tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presentsrequire in…infancytreatment in most cases! --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? No[Y/N] 42 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

How are stromal cysts treated? There are multiple approaches; each has its advantages and disadvantages. Photodisruption of the cyst is simple and noninvasive, butSecondary risks inciting iritis and/or glaucoma Primarywhen the pro-inflammatory contents of the cyst are released into the AC. Because of this, the Peds book recommends surgical excision. Other sources recommend the less-invasive aspiration with absoluteEpithelial alcohol injection.Stromal

Stromal cysts Because--Due to stromalof these sequestration issues, stromal of…epithelial cysts tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presentsrequire in…infancytreatment in most cases! --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? No[Y/N] 43 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

How are stromal cysts treated? There are multiple approaches; each has its advantages and disadvantages. Photodisruption of the cyst is simple and noninvasive, butSecondary risks inciting iritis and/or glaucoma Primarywhen the pro-inflammatory contents of the cyst are released into the AC. Because of this, the Peds book recommends surgical excision. Other sources recommend the less-invasive aspiration with absoluteEpithelial alcohol injection.Stromal

Stromal cysts Because--Due to stromalof these sequestration issues, stromal of…epithelial cysts tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presentsrequire in…infancytreatment in most cases! --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? No[Y/N] 44 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

How are stromal cysts treated? There are multiple approaches; each has its advantages and disadvantages. Photodisruption of the cyst is simple and noninvasive, butSecondary risks inciting iritis and/or glaucoma Primarywhen the pro-inflammatory contents of the cyst are released into the AC. Because of this, the Peds book recommends surgical excision. Other sources recommend the less-invasive aspiration with absoluteEpithelial alcohol injection.Stromal

Stromal cysts Because--Due to stromalof these sequestration issues, stromal of…epithelial cysts tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presentsrequire in…infancytreatment in most cases! --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? No[Y/N] 45 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…[drug] Epithelial Stromal --Can prophylax with…phenylephrine

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 46 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 47 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine What is the trade name for echothiophate? Phospholine

PupillaryPupillary IrisIris pigmentpigment What sort of drug is it? cystscysts epitheliumepithelium cystscysts A parasympathomimetic

Iris pigment epitheliumWhat is its mechanism of action? Pupillary cysts cysts It is an acetylcholinesterase inhibitor --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers What ocular condition is echothiophate used to treat? --Presents in…infancy --Presents in…teens Accommodative ET --Clinically significant? [Y/N]No 48 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine What is the trade name for echothiophate? Phospholine

PupillaryPupillary IrisIris pigmentpigment What sort of drug is it? cystscysts epitheliumepithelium cystscysts A parasympathomimetic

Iris pigment epitheliumWhat is its mechanism of action? Pupillary cysts cysts It is an acetylcholinesterase inhibitor --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers What ocular condition is echothiophate used to treat? --Presents in…infancy --Presents in…teens Accommodative ET --Clinically significant? [Y/N]No 49 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine What is the trade name for echothiophate? Phospholine

PupillaryPupillary IrisIris pigmentpigment What sort of drug is it? cystscysts epitheliumepithelium cystscysts A parasympathomimetic

Iris pigment epitheliumWhat is its mechanism of action? Pupillary cysts cysts It is an acetylcholinesterase inhibitor --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers What ocular condition is echothiophate used to treat? --Presents in…infancy --Presents in…teens Accommodative ET --Clinically significant? [Y/N]No 50 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine What is the trade name for echothiophate? Phospholine

PupillaryPupillary IrisIris pigmentpigment What sort of drug is it? cystscysts epitheliumepithelium cystscysts A parasympathomimetic

Iris pigment epitheliumWhat is its mechanism of action? Pupillary cysts cysts It is an acetylcholinesterase inhibitor --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers What ocular condition is echothiophate used to treat? --Presents in…infancy --Presents in…teens Accommodative ET --Clinically significant? [Y/N]No 51 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine What is the trade name for echothiophate? Phospholine

PupillaryPupillary IrisIris pigmentpigment What sort of drug is it? cystscysts epitheliumepithelium cystscysts A parasympathomimetic

Iris pigment epitheliumWhat is its mechanism of action? Pupillary cysts cysts It is an acetylcholinesterase inhibitor --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers What ocular condition is echothiophate used to treat? --Presents in…infancy --Presents in…teens Accommodative ET --Clinically significant? [Y/N]No 52 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine What is the trade name for echothiophate? Phospholine

PupillaryPupillary IrisIris pigmentpigment What sort of drug is it? cystscysts epitheliumepithelium cystscysts A parasympathomimetic

Iris pigment epitheliumWhat is its mechanism of action? Pupillary cysts cysts It is an acetylcholinesterase inhibitor --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers What ocular condition is echothiophate used to treat? --Presents in…infancy --Presents in…teens Accommodative ET --Clinically significant? [Y/N]No 53 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine What is the trade name for echothiophate? Phospholine

PupillaryPupillary IrisIris pigmentpigment What sort of drug is it? cystscysts epitheliumepithelium cystscysts A parasympathomimetic

Iris pigment epitheliumWhat is its mechanism of action? Pupillary cysts cysts It is an acetylcholinesterase inhibitor --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers What ocular condition is echothiophate used to treat? --Presents in…infancy --Presents in…teens Accommodative ET --Clinically significant? [Y/N]No 54 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine What is the trade name for echothiophate? Phospholine

PupillaryPupillary IrisIris pigmentpigment What sort of drug is it? cystscysts epitheliumepithelium cystscysts A parasympathomimetic

Iris pigment epitheliumWhat is its mechanism of action? Pupillary cysts cysts It is an acetylcholinesterase inhibitor --Pigment epi cysts at --2o to separation of… the…pupil border epithelial layers What ocular condition is echothiophate used to treat? --Presents in…infancy --Presents in…teens Accommodative ET --Clinically significant? [Y/N]No 55 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…[drug] lephrine

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 56 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary Secondary cysts --Usually due to…echothiophate Epithelial Stromal --Can prophylax with…phenylephrine

Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? [Y/N]No 57 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

What about secondarySecondary cysts after cysts penetrating trauma? --Usually due to…echothiophate That’s certainly a thing,--Can butprophylax—these with… arephenylephrine more likely to Epithelial Stromalbe epithelial downgrowth cysts resulting from the implantation of surface epithelium on the iris Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? No[Y/N] 58 Pediatric Iris Cysts and Nodules

Pediatric Iris Cysts

Primary Secondary

What about secondarySecondary cysts after cysts penetrating trauma? --Usually due to…echothiophate That’s certainly a thing,--Can butprophylax—these with… arephenylephrine more likely to Epithelial Stromalbe epithelial downgrowth cysts resulting from the implantation of surface epithelium on the iris Stromal cysts --Due to stromal sequestration of…epithelial tissue PupillaryPupillary IrisIris pigmentpigment during embryogenesis cystscysts epitheliumepithelium cystscysts --Presents in…infancy --Cysts contain…goblet cells, may cause enlargement Iris pigment epithelium --Result in a number of problems: Pupillary cysts cysts --Obstruct…visual axis --Pigment epi cysts at --2o to separation of… --Secondary…glaucoma the…pupil border epithelial layers --Induce…corneal decompensation --Presents in…infancy --Presents in…teens --Cyst leakage  chronic iritis --Clinically significant? No[Y/N] 59 Pediatric Iris Cysts and Nodules

Epithelial downgrowth cyst secondary to perforating corneal trauma in a child 60 Q Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the pediatric population  L isch nodules  B rushfield spots  J XG nodules

Mnemonic forthcoming… 61 Q Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the pediatric population  L  B  J

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 62 A Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the pediatric population  Lisch nodules   JXG nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 63 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? NF1  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected

 Lisch nodules What is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 64 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected

 Lisch nodules What is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 65 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 66 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 67 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 68 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 69 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the Briefly, what is a phakomatosis?age of the patient times 10. Thus, 50% of 5 year olds will have  BrushfieldA congenital spots conditionthem, involving 60% of 6hamartomatous year olds, etc. At lesions age 10 of andmultip beyond,le organ systems, usually including theessentially CNS , eyes100% andof NF1 skin patients have Lisch nodules.  JXG nodules By what more-descriptiAre Lischve name nodules does clinically the BCSC significant? Peds book refer to them? As neuro-oculocutaneousNo; their only syndromes significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 70 Q/A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the Briefly, what is a phakomatosis?age of the patient times 10. Thus, 50% of 5 year olds will have  BrushfieldA congenital spots conditionthem, involving 60% of 6hamartomatous year olds, etc. At lesions age 10 of andmultip beyond,le organ systems, usually including theessentially CNSabb. , eyes100% andof NF1 skin patients have Lisch nodules.  JXG nodules By what more-descriptiAre Lischve name nodules does clinically the BCSC significant? Peds book refer to them? As neuro-oculocutaneousNo; their only syndromes significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 71 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the Briefly, what is a phakomatosis?age of the patient times 10. Thus, 50% of 5 year olds will have  BrushfieldA congenital spots conditionthem, involving 60% of 6hamartomatous year olds, etc. At lesions age 10 of andmultip beyond,le organ systems, usually including theessentially CNS , eyes100% andof NF1 skin patients have Lisch nodules.  JXG nodules By what more-descriptiAre Lischve name nodules does clinically the BCSC significant? Peds book refer to them? As neuro-oculocutaneousNo; their only syndromes significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 72 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the Briefly, what is a phakomatosis?age of the patient times 10. Thus, 50% of 5 year olds will have  BrushfieldA congenital spots conditionthem, involving 60% of 6hamartomatous year olds, etc. At lesions age 10 of andmultip beyond,le organ systems, usually including theessentially CNS , eyes100% andof NF1 skin patients have Lisch nodules.  JXG nodules By what more-descriptiAre Lischve name nodules does clinically the BCSC significant? Peds book refer to them? As neuro-oculocutaneousNo; their only syndromes significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 73 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the Briefly, what is a phakomatosis?age of the patient times 10. Thus, 50% of 5 year olds will have  BrushfieldA congenital spots conditionthem, involving 60% of 6hamartomatous year olds, etc. At lesions age 10 of andmultip beyond,le organ systems, usually including theessentially CNS , eyes100% andof NF1 skin patients have Lisch nodules.  JXG nodules By what more-descriptiAre Lischve name nodules does clinically the BCSC significant? Peds book refer to them? As neuro- No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 74 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the Briefly, what is a phakomatosis?age of the patient times 10. Thus, 50% of 5 year olds will have  BrushfieldA congenital spots conditionthem, involving 60% of 6hamartomatous year olds, etc. At lesions age 10 of andmultip beyond,le organ systems, usually including theessentially CNS , eyes100% andof NF1 skin patients have Lisch nodules.  JXG nodules By what more-descriptiAre Lischve name nodules does clinically the BCSC significant? Peds book refer to them? As neuro-oculo No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 75 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the WhatIs it is associated the eponymous with NF2? name for NF1? pediatric populationvonYes, Recklinghausen’s but the relationship disease is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected In a word, what sort of condition is NF1?  Lisch nodules A phakomatosisWhat is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the Briefly, what is a phakomatosis?age of the patient times 10. Thus, 50% of 5 year olds will have  BrushfieldA congenital spots conditionthem, involving 60% of 6hamartomatous year olds, etc. At lesions age 10 of andmultip beyond,le organ systems, usually including theessentially CNS , eyes100% andof NF1 skin patients have Lisch nodules.  JXG nodules By what more-descriptiAre Lischve name nodules does clinically the BCSC significant? Peds book refer to them? As neuro-oculocutaneousNo; their only syndromes significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 76 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected

 Lisch nodules What is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 77 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected

 Lisch nodules What is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 78 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected

 Lisch nodules What is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 79 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected

 Lisch nodules What is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 80 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected

 Lisch nodules What is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 81 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected

 Lisch nodules What is the prevalence of Lisch nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 82 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected Briefly, what do Lisch nodules look like?  Lisch nodules DomeWhat isshaped the prevalence tan(nish) of Lisch masses nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 83 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected Briefly, what do Lisch nodules look like?  Lisch nodules DomeWhat isshaped the prevalence tan(nish) of Lisch masses nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the age of the patient times 10. Thus, 50% of 5 year olds will have  Brushfield spots them, 60% of 6 year olds, etc. At age 10 and beyond, essentially 100% of NF1 patients have Lisch nodules.  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 84 Q Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected Briefly, what do Lisch nodules look like?  Lisch nodules DomeWhat isshaped the prevalence tan(nish) of Lisch masses nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the  Brushfield spotsWhat ruleage of thumb of the adherespatient times regarding 10. Thus, the color 50% of of Lisch 5 year nodules? olds will have Lisch nodulesthem, are 60% lighter of 6 yearthan olds,the rest etc. of At the age iris 10 when and beyond,the iris in question isessentially dark, but 100%darker ofthan NF1 the patients rest when have the Lisch iris nodules.is light  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 85 A Pediatric Iris Cysts and Nodules

Lisch nodules are most strongly associated with what congenital condition? Neurofibromatosis type 1 (NF1)  Name 3 types of iris nodules found in the Is it associated with NF2? pediatric populationYes, but the relationship is far weaker—Lisch nodules occur in NF2, but so sporadically that they are not expected Briefly, what do Lisch nodules look like?  Lisch nodules DomeWhat isshaped the prevalence tan(nish) of Lisch masses nodules in NF1? The rule-of-thumb is that Lisch nodule prevalence equals the  Brushfield spotsWhat ruleage of thumb of the adherespatient times regarding 10. Thus, the color 50% of of Lisch 5 year nodules? olds will have Lisch nodulesthem, are 60% lighter of 6 yearthan olds,the rest etc. of At the age iris 10 when and beyond,the iris in question isessentially dark, but 100%darker ofthan NF1 the patients rest when have the Lisch iris nodules.is light  JXG nodules Are Lisch nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 86 Pediatric Iris Cysts and Nodules

Darker on light iris Lighter on dark iris

Lisch nodules 87 Q Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the pediatric population  Lisch nodules Another sort of iris nodule needs mentioning here, because it is in the  Brushfield spots DDx for Lisch nodules. What is it?  JXG nodules  Iris mammillationsone word

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 88 A Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the pediatric population  Lisch nodules Another sort of iris nodule needs mentioning here, because it is in the  Brushfield spots DDx for Lisch nodules. What is it?  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 89 Q Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules Usually unilateral, but bilaterality occurs frequently enough that it can’t be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 90 Q/A Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillarytwo words bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules Usually unilateral, but bilaterality occurs frequently enough that it can’t be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 91 A Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules Usually unilateral, but bilaterality occurs frequently enough that it can’t be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 92 Q Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules Usually unilateral, but bilaterality occurs frequently enough that it can’t be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 93 A Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules Usually unilateral, but bilaterality occurs frequently enough that it can’t be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 94 Pediatric Iris Cysts and Nodules

Iris mammilations 95 Q Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules Usually unilateral, but bilaterality occurs frequently enough that it can’t be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 96 A Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules Usually unilateral, but bilaterality occurs frequently enough that it can’t be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 97 Q Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules Usually unilateral, but bilaterality occurs frequently enough that it can’t be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 98 A Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules Usually unilateral, but bilaterality occurs frequently enough that it can’t be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 99 Q Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodules pigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the ‘Tinyiris pigmented surface iris nodules associated with NF1’--given this, pediatric populationhow on earth are you supposed to differentiate between the two? By appearance. Iris mammillations are always the same color as  isch nodules the Arerest theyof the unilateral, iris. In contrast, or bilateral? Lisch nodules are lighter than the L restUsually of the iris unilateral, when the butiris bilateralityin question occursis dark, frequentlybut darker than the enoughrest when that the it iris can’t is light. be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 100 A Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodules pigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the ‘Tinyiris pigmented surface iris nodules associated with NF1’--given this, pediatric populationhow on earth are you supposed to differentiate between the two? By appearance. Iris mammillations are always the same color as  isch nodules the Arerest theyof the unilateral, iris. In contrast, or bilateral? Lisch nodules are lighter than the L restUsually of the iris unilateral, when the butiris bilateralityin question occursis dark, frequentlybut darker than the enoughrest when that the it iris can’t is light. be used to rule them out  Brushfield spots Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 101 Q Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules In addition to NF1, iris mammillations have another Usually unilateral,important but association. bilaterality What occurs is it? frequently enough thatOculodermal it can’t be melanocytosis,used to rule them aka out nevus of Ota  Brushfield spots Are they associated with NF1?and…oculodermal Yes (albeit not nearly as strongly as Lischmelanocytosisnodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 102 A Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral?  Lisch nodules In addition to NF1, iris mammillations have another Usually unilateral,important but association. bilaterality What occurs is it? frequently enough thatOculodermal it can’t be melanocytosis,used to rule them aka out nevus of Ota  Brushfield spots Are they associated with NF1?and…oculodermal Yes (albeit not nearly as strongly as Lischmelanocytosis nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 103 Q Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral? In addition to NF1, iris mammillations have another  Lisch nodules Usually unilateral, but bilaterality occurs frequently What is oculodermal melanocytosis/nevus of Ota? important association. What is it? enough that it can’t be used to rule them out Ocular melanocytosis + hyperpigmentation of the Oculodermal melanocytosis, aka nevus of Ota ipsilateral periorbitalBrushfield skin spots Are they associated with NF1?and…oculodermal Yes (albeit not nearly as strongly as Lischmelanocytosis nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 104 A Pediatric Iris Cysts and Nodules Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral? In addition to NF1, iris mammillations have another  Lisch nodules Usually unilateral, but bilaterality occurs frequently What is oculodermal melanocytosis/nevus of Ota? important association. What is it? enough that it can’t be used to rule them out Ocular melanocytosis + hyperpigmentation of the Oculodermal melanocytosis, aka nevus of Ota ipsilateral periorbitalBrushfield skin spots Are they associated with NF1?and…oculodermal Yes (albeit not nearly as strongly as Lischmelanocytosis nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 105 Pediatric Iris Cysts and Nodules

Oculodermal melanocytosis (nevus of Ota) 106 Pediatric Iris Cysts and Nodules

Mammillations? Aren’t those a CNS thingamajig? You’re thinking of the mammillary bodies , paired structures that are part of the limbic system

OK, then what are iris mammillations?  Name 3 types of irisTiny nodulespigmented nodules found which, when in present, the are found in vast numbers diffusely scattered across the pediatric populationiris surface

Are they unilateral, or bilateral? In addition to NF1, iris mammillations have another  Lisch nodules Usually unilateral, but bilaterality occurs frequently What is oculodermal melanocytosis/nevus of Ota? important association. What is it? enough that it can’t be used to rule them out Ocular melanocytosisFor more + hyperpigmentation on Nevus of the of Ota,Oculodermal see melanocytosis, slide-set aka nevus O9 of Ota ipsilateral periorbitalBrushfield skin spots Are they associated with NF1?and…oculodermal Yes (albeit not nearly as strongly as Lischmelanocytosis nodules)  JXG nodules  Iris mammillations

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 107 Q Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% What is the clinical significance of Brushfield spots?  JXG nodules They have none When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 108 A Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% What is the clinical significance of Brushfield spots?  JXG nodules They have none When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 109 Pediatric Iris Cysts and Nodules

Brushfield spots 110 Q Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% What is the clinical significance of Brushfield spots?  JXG nodules They have none When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 111 A Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% What is the clinical significance of Brushfield spots?  JXG nodules They have none When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 112 Q Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% What is the clinical significance of Brushfield spots?  JXG nodules They have none When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 113 A Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% What is the clinical significance of Brushfield spots?  JXG nodules They have none When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 114 Q Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% What is the clinical significance of Brushfield spots?  JXG nodules They have none When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 115 A Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% What is the clinical significance of Brushfield spots?  JXG nodules They have none When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 116 Pediatric Iris Cysts and Nodules

Wolfflin nodules 117 Q Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% Are Wolfflin nodulesWhat is athe common clinical finding? significance of Brushfield spots?  JXG nodules Yes--they’re Theyestimated have to none be present in about 25% of the population When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 118 Q/A Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% Are Wolfflin nodulesWhat is athe common clinical finding? significance of Brushfield spots?  JXG nodules Yes--they’re Theyestimated have to none be present in about 25%% of the population When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 119 A Pediatric Iris Cysts and Nodules

 Name 3 types of iris nodules found in the Brushfield spots are most strongly associated with what pediatric populationcongenital condition? Down syndrome

 Lisch nodules What is the prevalence of Brushfield spots in the Down population?  Brushfield spots At least 90% Are Wolfflin nodulesWhat is athe common clinical finding? significance of Brushfield spots?  JXG nodules Yes--they’re Theyestimated have to none be present in about 25% of the population When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? Wolfflin nodules

Think of president Lyndon Baines Johnson (known colloquially as LBJ) as having iris nodules… 120 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 121 What does JXG stand for? A PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 122 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 123 What does JXG stand for? A PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 124 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…  Name 3 types ofThe iris presence nodules of… found in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 125 What does JXG stand for? A PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 126 Pediatric Iris Cysts and Nodules

Touton giant cells Foamy macrophages

JXG 127 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 128 What does JXG stand for? A PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 129 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 130 What does JXG stand for? A PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 131 Pediatric Iris Cysts and Nodules

JXG: Skin papules. The orangish color is classic 132 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 133 What does JXG stand for? A PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 134 Pediatric Iris Cysts and Nodules

JXG: Iris lesion 135 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 136 What does JXG stand for? A PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 137 Pediatric Iris Cysts and Nodules

JXG: Spontaneous hyphema 138 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 139 What does JXG stand for? A PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 140 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 141 What does JXG stand for? A PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) As skin papules

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 142 What does JXG stand for? Q PediatricJuvenile Iris xanthogranulomaCysts and Nodules In three words, what sort of condition is it? It is a nonneoplastic histiocytic proliferation

What are the two hallmarks of JXG histology? The presence of…Touton giant cells  Name 3 types ofThe iris presence nodules of…’foamy macrophages found ’ in the At what age does JXG present? pediatric populationThe majority before age 1 year, and almost all by age 2  Lisch nodules How does JXG usually present? (Hint: It’s not ophthalmic) For more onAs JXG,skin papules see slide-set P3

 Brushfield spotsWhen iris JXG nodules are present, is it uni-, or bilaterally? Unilaterally

 JXG nodules In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough are present, they will cause heterochromia iridis

Should they be removed surgically? Only if the glaucoma is uncontrollable

Think of president Lyndon Baines JohnsonWhat is the(known natural colloquially history of the as disease? LBJ) as having iris nodules… JXG is self-limited, usually resolving by age 5 years 143 Q Pediatric Iris Cysts and Nodules

bilateral vs  Congenital iris ectropion syndrome consists of unilateralunilateral ectropion uveae plus… 144 A Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus… 145 Q Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless,orextra corrugated? 146 A Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless, or extra corrugated? Glassy and cryptless 147 Pediatric Iris Cysts and Nodules

Congenital ectropion uveae syndrome. A, Wide pupil and striking smoothness of the anterior surface of the iris, absence of the circular contraction furrows, and absence of radial folds are shown. (Arrows indicate the iris ectropion.) B, Higher magnification and illumination of the iris to better differentiate the pupillary margin from the iris ectropion (arrows) 148 Q Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless, or extra corrugated? Glassy and cryptless  …iris insertion abnormality--too high or too low? 149 A Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless, or extra corrugated? Glassy and cryptless  …iris insertion abnormality--too high or too low? Too high 150 Pediatric Iris Cysts and Nodules

Congenital ectropion uveae syndrome: Gonioscopy depicting anterior insertion of iris (black arrow) 151 Q Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless, or extra corrugated? Glassy and cryptless  …iris insertion abnormality--too high or too low? Too high  …dysgenesis of what anterior structure? 152 A Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless, or extra corrugated? Glassy and cryptless  …iris insertion abnormality--too high or too low? Too high  …dysgenesis of what anterior structure? The TM (trabecular meshwork) 153 Q Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless, or extra corrugated? Glassy and cryptless  …iris insertion abnormality--too high or too low? Too high  …dysgenesis of what anterior structure? The TM  …what blinding complication? 154 A Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless, or extra corrugated? Glassy and cryptless  …iris insertion abnormality--too high or too low? Too high  …dysgenesis of what anterior structure? The TM  …what blinding complication? Glaucoma 155 Q Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless,orextra corrugated? Glassy and cryptless  …iris insertion abnormality--too high or too low?Toohigh  …dysgenesis of what anterior structure? The TM  …what blinding complication? Glaucoma  What does ectropion uveae refer to, and why is the term technically a misnomer? Ectropion uveae refers to the presence of posterior pigmented iris epithelium on the anterior surface of the iris. It is a misnomer because the posterior pigmented epithelium derives from neuroectoderm, not uvea. 156 A Pediatric Iris Cysts and Nodules

 Congenital iris ectropion syndrome consists of unilateral ectropion uveae plus…  …iris surface abnormality--is it glassy and cryptless,orextra corrugated? Glassy and cryptless  …iris insertion abnormality--too high or too low? Too high  …dysgenesis of what anterior structure? The TM  …what blinding complication? Glaucoma  What does ectropion uveae refer to, and why is the term technically a misnomer? Ectropion uveae refers to the presence of posterior pigmented iris epithelium on the anterior surface of the iris. It is a misnomer because the posterior pigmented epithelium derives from neuroectoderm, not uvea.

(I guess the term ectropion neuroectodermeae didn’t scan well…)