Abnormalities of the Human Pupil: Shape, Location, Number, and Size, Exercises of Anatomy

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Abnormalities of Pupil Structure Abnormal Pupil… Size --Congenital miosis (microcorea) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities --Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil… Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil… Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Pupil structure abnormality Abnormal Pupil… Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases (Other than aniridia) 1 Abnormalities of Pupil Structure Abnormal Pupil…Size --Congenital miosis (microcorea) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities --Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 2 Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Pupil structure abnormality 5 Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Pupil structure abnormality 6 Abnormalities of Pupil Structure 7 Congenital microcoria Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Pupil structure abnormality 10 Abnormalities of Pupil Structure 11 Congenital microcoria The patient was a 73-year-old white female with bilateral congenital mydriasis and no other abnormalities. The pupils reacted almost imperceptibly to light. Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality 12 Abnormalities of Pupil Structure 15 Dyscoria Slit-shaped dyscoric pupil OS (note also the ipsilateral microcornea) Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Location Etiology Associated abnormalities Workup Typical iris coloboma Inferonasal Incomplete closure of embryonic fissure Colobo a of CB, choroid, optic nerve, retina; nystagmus If 1+ other organ system abnormal: Do karyotype analysis with extended banding Atypical iris coloboma Anywhere but inferonasal Probably due to hyaloid remnants None None 16 ? ? Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Location Etiology Associated abnormalities Workup Typical iris coloboma Inferonasal Incomplete closure of embryonic fissure Colobo a of CB, choroid, optic nerve, retina; nystagmus If 1+ other organ system abnormal: Do karyotype analysis with extended banding Atypical iris coloboma Anywhere but inferonasal Probably due to hyaloid remnants None None 17 Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Location Etiology Associated abnormalities Workup Typical iris coloboma Inferonasal Incomplete closure of embryonic fissure Colobo a of CB, choroid, optic nerve, retina; nystagmus If 1+ other organ system abnormal: Do karyotype analysis with extended banding Atypical iris coloboma Anywhere but inferonasal Multiple etiologies implicated None None 20 Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Location Etiology Associated abnormalities Workup Typical iris coloboma Inferonasal Incomplete closure of embryonic fissure Colobo a of CB, choroid, optic nerve, retina; nystagmus If 1+ other organ system abnormal: Do karyotype analysis with extended banding Atypical iris coloboma Anywhere but inferonasal Multiple etiologies implicated None None 21 ? ? Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Location Etiology Associated abnormalities Workup Typical iris coloboma Inferonasal Incomplete closure of embryonic fissure Colobo a of CB, choroid, optic nerve, retina; nystagmus If 1+ other organ system abnormal: Do karyotype analysis with extended banding Atypical iris coloboma Anywhere but inferonasal Multiple etiologies implicated None None 22 Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Location Etiology Associated abnormalities Workup Typical iris coloboma Inferonasal Incomplete closure of embryonic fissure Colobo a of CB, choroid, optic nerve, retina; nystagmus If 1+ other organ system abnormal: Do karyotype analysis with extended banding Atypical iris coloboma Anywhere but inferonasal Multiple etiologies implicated None None 25 Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 26 Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 27 Abnormalities of Pupil Structure 30 True polycoria Eye in bright light (A) and dim light (B). Following dilatation with tropicamide and phenylephrine (C,D) Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 31 Abnormal Pupil…Location --Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK --Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 32 Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 35 Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 36 Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae --Bilateral disease --Pupil and lens displaced in opposite directions --pupils often dyscoric Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 37 Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 40 3 high-yield facts about ectopia lentis et pupillae Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases 41 Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal What abnormality of lens shape is present? Microspherophakia How is the pupil dyscoric, ie, what is its shape? Slit-like 42 Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal How is the pupil dyscoric, ie, what is its shape? Slit-like What abnormality of lens shape is present? Microspherophakia 45 Abnormalities of Pupil Structure 46 Ectopia lentis et pupillae: Bilateral inferonasal displacement of dyscoric, slit-like(ish) pupils Abnormalities of Pupil Structure 47 Ectopia lentis et pupillae: Pupil and lens displaced in opposite directions Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal How is the pupil dyscoric, ie, what is its shape? Slit-like What abnormality of lens shape is present? Microspherophakia 50 In a few words, how would you describe the shape of a microspherophakic lens? The name says it all: the lens is small (‘micro’) and round (‘sphero’) What common slit-lamp observation owes to the lens’ small size? Typically, the entirety of the lens equator can be seen in the pupillary aperture when the pt is widely dilated How does refractive status manifest the lens’ spherical shape? Pts are usually highly myopic When you hear microspherophakia, what condition should come immediately to mind? Weill-Marchesani syndrome Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal How is the pupil dyscoric, ie, what is its shape? Slit-like What abnormality of lens shape is present? Microspherophakia 51 In a few words, how would you describe the shape of a microspherophakic lens? The name says it all: the lens is small (‘micro’) and round (‘sphero’) What common slit-lamp observation owes to the lens’ small size? Typically, the entirety of the lens equator can be seen in the pupillary aperture when the pt is widely dilated How does refractive status manifest the lens’ spherical shape? Pts are usually highly myopic When you hear microspherophakia, what condition should come immediately to mind? Weill-Marchesani syndrome Abnormalities of Pupil Structure 52 Microspherophakia. Note the small size, round shape of the lens Abnormalities of Pupil Structure 55 Microspherophakia. Lens is able to fit through the pupillary aperture with mydriasis Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal How is the pupil dyscoric, ie, what is its shape? Slit-like What abnormality of lens shape is present? Microspherophakia 56 In a few words, how would you describe the shape of a microspherophakic lens? The name says it all: the lens is small (‘micro’) and round (‘sphero’) What common slit-lamp observation owes to the lens’ small size? Typically, the entirety of the lens equator can be seen in the pupillary aperture when the pt is widely dilated How does refractive status manifest the lens’ spherical shape? Pts are usually highly myopic When you hear microspherophakia, what condition should come immediately to mind? Weill-Marchesani syndrome Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal How is the pupil dyscoric, ie, what is its shape? Slit-like What abnormality of lens shape is present? Microspherophakia 57 In a few words, how would you describe the shape of a microspherophakic lens? The name says it all: the lens is small (‘micro’) and round (‘sphero’) What common slit-lamp observation owes to the lens’ small size? Typically, the entirety of the lens equator can be seen in the pupillary aperture when the pt is widely dilated How does refractive status manifest the lens’ spherical shape? Pts are usually highly myopic When you hear microspherophakia, what condition should come immediately to mind? Weill-Marchesani syndrome Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal How is the pupil dyscoric, ie, what is its shape? Slit-like What abnormality of lens shape is present? Microspherophakia 60 In a few words, how would you describe the shape of a microspherophakic lens? The name says it all: the lens is small (‘micro’) and round (‘sphero’) What common slit-lamp observation owes to the lens’ small size? Typically, the entirety of the lens equator can be seen in the pupillary aperture when the pt is widely dilated How does refractive status manifest the lens’ spherical shape? Pts are usually highly myopic When you hear microspherophakia, what condition should come immediately to mind? Weill-Marchesani syndrome What is the classic stature of a W-M pt? Quite short What is notable about their digits? They are short as well What is notable about their joints? They are stiff Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal How is the pupil dyscoric, ie, what is its shape? Slit-like What abnormality of lens shape is present? Microspherophakia 61 In a few words, how would you describe the shape of a microspherophakic lens? The name says it all: the lens is small (‘micro’) and round (‘sphero’) What common slit-lamp observation owes to the lens’ small size? Typically, the entirety of the lens equator can be seen in the pupillary aperture when the pt is widely dilated How does refractive status manifest the lens’ spherical shape? Pts are usually highly myopic When you hear microspherophakia, what condition should come immediately to mind? Weill-Marchesani syndrome What is the classic stature of a W-M pt? Quite short What is notable about their digits? They are short as well What is notable about their joints? They are stiff Abnormalities of Pupil Structure Weill-Marchesani syndrome: Short stature Q Nn Abnormalities of Pupil Structure D a Weill-Marchesani syndrome: Short digits Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal How is the pupil dyscoric, ie, what is its shape? Slit-like What abnormality of lens shape is present? Microspherophakia 66 In a few words, how would you describe the shape of a microspherophakic lens? The name says it all: the lens is small (‘micro’) and round (‘sphero’) What common slit-lamp observation owes to the lens’ small size? Typically, the entirety of the lens equator can be seen in the pupillary aperture when the pt is widely dilated How does refractive status manifest the lens’ spherical shape? Pts are usually highly myopic When you hear microspherophakia, what condition should come immediately to mind? Weill-Marchesani syndrome What is the classic stature of a W-M pt? Quite short What is notable about their digits? They are short as well What is notable about their joints? They are stiff Abnormal Pupil…Location Corectopia = displacement of pupil --Slight (.5mm) inferonasal deviation is normal --Deviation up to 1mm considered OK Corectopia associated with lens subluxation = ectopia lentis et pupillae a) Bilateral disease b) Pupil and lens displaced in opposite directions c) Pupils often dyscoric Abnormalities of Pupil Structure Abnormal Pupil…Size 1) Congenital miosis (microcoria) --Due to dilator absence/malformation, or fibrous contraction --Associated with multiple congenital ocular abnormalities 2) Congenital mydriasis --Probably part of the aniridia spectrum Abnormal Pupil…Shape --Dyscoria = congenital misshapen pupil --e.g., coloboma Pupil structure abnormality Abnormal Pupil…Number --Polycoria = >1 pupil --True polycoria requires that each pupil has a…sphincter muscle (is exceedingly rare) --Pseudopolycoria accounts for the vast majority of cases What directions are the pupils and lenses displaced? Pupils inferotemporal, lenses superonasal How is the pupil dyscoric, ie, what is its shape? Slit-like What abnormality of lens shape is present? Microspherophakia 67 In a few words, how would you describe the shape of a microspherophakic lens? The name says it all: the lens is small (‘micro’) and round (‘sphero’) What common slit-lamp observation owes to the lens’ small size? Typically, the entirety of the lens equator can be seen in the pupillary aperture when the pt is widely dilated How does refractive status manifest the lens’ spherical shape? Pts are usually highly myopic When you hear microspherophakia, what condition should come immediately to mind? Weill-Marchesani syndrome What is the classic stature of a W-M pt? Quite short What is notable about their digits? They are short as well What is notable about their joints? They are stiff