Congenital Lid Abnormalities Q  Ankyloblepharon

Home , Ankyloblepharon, Blepharophimosis, Coloboma, Cryptophthalmos, Entropion, Trichiasis

1 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Congenital ectropion

 Epiblepharon

Matching!  Dystopia canthorum

 Congenital entropion

 Euryblepharon

 Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 2 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Congenital ectropion

 Epiblepharon

 Dystopia canthorum

 Congenital entropion

 Euryblepharon

 Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 3 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

What is the classic appearance of cryptophthalmos? Congenital ectropion An expanse of uninterrupted skin from the forehead region to the cheek  Epiblepharon Is there an eye under the skin? Of sorts, but it is virtually always profoundly malformed Dystopia canthorum  Congenital entropion Can relatively normal lids be surgically constructed? Not easily. The problem is, not only are the lids undifferentiated, but their  Euryblepharon architectural elements--tarsal plates, orbicularis muscle, meibomian glands, etc--are absent.  Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 4 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 5 Congenital Lid Abnormalities

Cryptophthalmos 6 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 7 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 8 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 9 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 10 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Epiblepharon

 Dystopia canthorum

 Congenital entropion

 Euryblepharon

 Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 11 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Epiblepharon

 Dystopia canthorum

 Congenital entropion

 Euryblepharon

 Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 12 Congenital Lid Abnormalities

Upper-lid coloboma in Goldenhar 13 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Epiblepharon

 Dystopia canthorum Lid colobomas associated with Goldenhar—are they found in the upper, or the lower lid?  Congenital entropion Depends on who you ask. The BCSC Cornea book says the upper, whereas the Plastics book indicates the lower. EyeWiki Euryblepharonsays upper > lower. The Peds book doesn’t address the issue. Caveat emptor.  Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 14 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Epiblepharon

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 15 Congenital Lid Abnormalities Q  What other ocular structures are commonly colobomatous? Ankyloblepharon --The iris (in general—not in Goldenhar) --The choroid  Blepharophimosis --The optic nerve head  Failure of lid differentiation  Cryptophthalmos Are lid colobomas associated with these others?  AssociatedNo with Goldenhar  Congenital ectropion

 Epiblepharon

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 16 Congenital Lid Abnormalities A  What other ocular structures are commonly colobomatous? Ankyloblepharon --The iris --The choroid  Blepharophimosis --The optic nerve head  Failure of lid differentiation  Cryptophthalmos Are lid colobomas associated with these others?  AssociatedNo with Goldenhar  Congenital ectropion

 Epiblepharon

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 17 Congenital Lid Abnormalities Q  What other ocular structures are commonly colobomatous? Ankyloblepharon --The iris --The choroid What is the pathological process that accBlepharophimosisounts for all of these coloboma types? --The optic nerve head Failure of the embryonic fissure of the optic vesicle to close  Failure of lid differentiation  Cryptophthalmos Are lid colobomas associated with these others?  AssociatedNo with Goldenhar  Congenital ectropion

 Epiblepharon

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 18 Congenital Lid Abnormalities A  What other ocular structures are commonly colobomatous? Ankyloblepharon --The iris --The choroid What is the pathological process that accBlepharophimosisounts for all of these coloboma types? --The optic nerve head Failure of the embryonic fissure of the optic vesicle to close  Failure of lid differentiation  Cryptophthalmos Are lid colobomas associated with these others?  AssociatedNo with Goldenhar  Congenital ectropion

 Epiblepharon

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 19 Congenital Lid Abnormalities Q  What other ocular structures are commonly colobomatous? Ankyloblepharon --The iris --The choroid What is the pathological process that accBlepharophimosisounts for all of these coloboma types? --The optic nerve head Failure of the embryonic fissure of the optic vesicle to close  Failure of lid differentiation  Cryptophthalmos Are lid colobomas associated with these others?  AssociatedNo with Goldenhar  Congenital ectropion

 Epiblepharon

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 20 Congenital Lid Abnormalities A  What other ocular structures are commonly colobomatous? Ankyloblepharon --The iris --The choroid What is the pathological process that accBlepharophimosisounts for all of these coloboma types? --The optic nerve head Failure of the embryonic fissure of the optic vesicle to close  Failure of lid differentiation  Cryptophthalmos Are lid colobomas associated with these others?  AssociatedNo with Goldenhar  Congenital ectropion

 Epiblepharon

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 21 Congenital Lid Abnormalities Q  What other ocular structures are commonly colobomatous? Ankyloblepharon --The iris --The choroid What is the pathological process that accBlepharophimosisounts for all of these coloboma types? --The optic nerve head Failure of the embryonic fissure of the optic vesicle to close  Failure of lid differentiation  Cryptophthalmos Are lid colobomas associated with these others? Why are lid colobomas not associated with the other types?  AssociatedNo with Goldenhar  Congenital ectropion Because the eyelids do not derive embryologically Epiblepharon from the optic vesicle

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 22 Congenital Lid Abnormalities A  What other ocular structures are commonly colobomatous? Ankyloblepharon --The iris --The choroid What is the pathological process that accBlepharophimosisounts for all of these coloboma types? --The optic nerve head Failure of the embryonic fissure of the optic vesicle to close  Failure of lid differentiation  Cryptophthalmos Are lid colobomas associated with these others? Why are lid colobomas not associated with the other types?  AssociatedNo with Goldenhar  Congenital ectropion Because the eyelids do not derive embryologically Epiblepharon from the optic vesicle

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 23 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surfaceAnswer finding starts is common? with ‘O’ O Limbal dermoids  CongenitalL entropion What strabismus syndrome is associated with Goldenhar? D  Euryblepharon Duane syndrome E What nonocular findings are usually present?  CongenitalN tarsal kink --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) A Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaR

 Congenital ptosis 24 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surfaceAnswer finding starts is common? with ‘O’ OAV syndrome Limbal dermoids  CongenitalL entropion What strabismus syndrome is associated with Goldenhar? D  Euryblepharon Duane syndrome E What nonocular findings are usually present?  CongenitalN tarsal kink --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) A Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaR

 Congenital ptosis 25 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surface finding is common? OAV syndrome Limbal dermoids Starts withCongenital ‘L’ L entropion What strabismus syndrome is associated with Goldenhar? D  Euryblepharon Duane syndrome E What nonocular findings are usually present?  CongenitalN tarsal kink --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) A Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaR

 Congenital ptosis 26 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surface finding is common? OAV syndrome Limbal dermoids Starts withCongenital ‘L’ Limbal entropion dermoids What strabismus syndrome is associated with Goldenhar? D  Euryblepharon Duane syndrome E What nonocular findings are usually present?  CongenitalN tarsal kink --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) A Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaR

 Congenital ptosis 27 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surface finding is common? OAV syndrome Limbal dermoids  CongenitalLimbal entropion dermoids What strabismus syndrome is associated with Goldenhar? Etc. D  Euryblepharon Duane syndrome E What nonocular findings are usually present?  CongenitalN tarsal kink --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) A Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaR

 Congenital ptosis 28 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surface finding is common? OAV syndrome Limbal dermoids  CongenitalLimbal entropion dermoids What strabismus syndrome is associated with Goldenhar? Duane syndrome  Euryblepharon Duane syndrome E What nonocular findings are usually present?  CongenitalN tarsal kink --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) A Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaR

 Congenital ptosis 29 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surface finding is common? OAV syndrome Limbal dermoids  CongenitalLimbal entropion dermoids What strabismus syndrome is associated with Goldenhar? Duane syndrome  Euryblepharon Duane syndrome E What nonocular findings are usually present?  CongenitalNothing tarsal starts kink w/ ‘N’ --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) A Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaR

 Congenital ptosis 30 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surface finding is common? OAV syndrome Limbal dermoids  CongenitalLimbal entropion dermoids What strabismus syndrome is associated with Goldenhar? Duane syndrome  Euryblepharon Duane syndrome Ear abnormalities What nonocular findings are usually present?  CongenitalNothing tarsal starts kink w/ ‘N’ --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) A Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaR

 Congenital ptosis 31 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surface finding is common? OAV syndrome Limbal dermoids  CongenitalLimbal entropion dermoids What strabismus syndrome is associated with Goldenhar? Duane syndrome  Euryblepharon Duane syndrome Ear abnormalities What nonocular findings are usually present?  CongenitalNothing tarsal starts kink w/ ‘N’ --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) Also, ‘A’ Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaR

 Congenital ptosis 32 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 EpiblepharonMnemonic: Goldenhar What is the noneponymous name of Goldenhar syndrome? Oculo-Auriculo-Vertebral (OAV) syndrome Goldenhar  Dystopia canthorum In addition to lid colobomas, what other adnexal/surface finding is common? OAV syndrome Limbal dermoids  CongenitalLimbal entropion dermoids What strabismus syndrome is associated with Goldenhar? Duane syndrome  Euryblepharon Duane syndrome Ear abnormalities What nonocular findings are usually present?  CongenitalNothing tarsal starts kink w/ ‘N’ --Ear abnormalities (pre-auricular appendages; aural fistulae) Hemifacial microsomia --Hemifacial microsomia (maxillary/mandibular hypoplasia)  Epicanthus (epicanthal folds) Also, ‘A’ Are Goldenhar pts cognitively impaired? Retardation is present in a minority (~10%)  Lid colobomaRetardation in ~10%

 Congenital ptosis 33 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum

 Congenital entropion

 Euryblepharon

 Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 34 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum

 Congenital entropion

 Euryblepharon

 Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 35 Congenital Lid Abnormalities

Ankyloblepharon 36 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum

An infant has several fine strands of tissue connectingCongenital the upper entropion and lower lid margins. Is this ankyloblepharon? Yes, this is a variant called ankyloblepharon filiformEuryblepharon adnatum How is this managed?  Congenital tarsal kink By cutting the strands under local anesthesia in the office  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 37 Congenital Lid Abnormalities Q/A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum

An infant has several fine strands of tissue connectingCongenital the upper entropion and lower lid margins. Is this ankyloblepharon? Yes, this is a variant called ankyloblepharon filiformEuryblepharontwo more adnatum words How is this managed?  Congenital tarsal kink By cutting the strands under local anesthesia in the office  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 38 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum

 Lid coloboma

 Congenital ptosis 39 Congenital Lid Abnormalities

Ankyloblepharon filiform adnatum 40 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum

 Lid coloboma

 Congenital ptosis 41 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum

 Lid coloboma

 Congenital ptosis 42 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion

 Euryblepharon Not this one, duh  Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 43 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion

 Euryblepharon

 Congenital tarsal kink

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 44 Congenital Lid Abnormalities

Congenital tarsal kink. (A) This child presented with a swollen and sore left eye with blepharospasm. (B) On eversion of the lid the horizontal kink in the tarsus can be seen. It runs the whole length of the tarsal plate, which is bent to 90°. 45 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 46 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 47 Congenital Lid Abnormalities

Epiblepharon 48 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink The ‘redundant lid tissue’—of what is it composed? A band of excess pre-tarsal obicularis muscle and overlying Epicanthusskin (epicanthal folds)

 Lid coloboma

 Congenital ptosis 49 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink The ‘redundant lid tissue’—of what is it composed? A band of excess pre-tarsal obicularis muscle and overlying Epicanthusskin (epicanthal folds)

 Lid coloboma

 Congenital ptosis 50 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface Does epiblepharon usually involve the upper lid, or the lower? Congenital tarsal kink The lower (epiblepharon rarely if ever occurs in the upper lid)  Epicanthus (epicanthal folds) Does it typically involve the entirety of the lower lid, or only a portion?  Lid coloboma Typically it involves only the nasal portion  Congenital ptosis 51 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface Does epiblepharon usually involve the upper lid, or the lower? Congenital tarsal kink The lower (epiblepharon rarely if ever occurs in the upper lid)  Epicanthus (epicanthal folds) Does it typically involve the entirety of the lower lid, or only a portion?  Lid coloboma Typically it involves only the nasal portion  Congenital ptosis 52 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface Does epiblepharon usually involve the upper lid, or the lower? Congenital tarsal kink The lower (epiblepharon rarely if ever occurs in the upper lid)  Epicanthus (epicanthal folds) Does it typically involve the entirety of the lower lid, or only a portion?  Lid coloboma Typically it involves only the nasal portion  Congenital ptosis 53 Congenital Lid Abnormalities Q/A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface Does epiblepharon usually involve the upper lid, or the lower? Congenital tarsal kink The lower (epiblepharon rarely if ever occurs in the upper lid)  Epicanthus (epicanthal folds) Does it typically involve the entirety of the lower lid, or only a portion? temporal v  Lid coloboma Typically it involves only the nasalmedial v portion nasal  Congenital ptosis 54 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface Does epiblepharon usually involve the upper lid, or the lower? Congenital tarsal kink The lower (epiblepharon rarely if ever occurs in the upper lid)  Epicanthus (epicanthal folds) Does it typically involve the entirety of the lower lid, or only a portion?  Lid coloboma Typically it involves only the nasal portion  Congenital ptosis 55 Congenital Lid Abnormalities

Epiblepharon. Note primarily nasal involvement 56 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins Children Dystopia of what twocanthorum ethnic heritages are  Type of congenital entropion most likely to present with epiblepharon  (and whichCongenital is #1)? entropion  Redundant lid tissue causes Asian (#1) and Native-American  Euryblepharon lashes to abut ocular surface Does epiblepharon usually involve the upper lid, or the lower? Congenital tarsal kink The lower (epiblepharon rarely if ever occurs in the upper lid)  Epicanthus (epicanthal folds) Does it typically involve the entirety of the lower lid, or only a portion?  Lid coloboma Typically it involves only the nasal portion  Congenital ptosis 57 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

Epiblepharon in pt of Asian heritage 59

Two other common lid disordersCongenital also involves apposiLid Abnormalitiestion of the lashes to the cornea. What are they? Entropion and trichiasis Congenital lid abnormalities: Matching QAIn a few words, define… Entropion: An inward rotation of the eyelid margin Trichiasis: An inward rotation of eyelashes  Ankyloblepharon

So is epiblepharon a form of entropion, or trichiasis?  Blepharophimosis Neither. Note that, by definition, entropion involves an inward turning of the lid margin, and trichiasis  Failurean inward turning of lid of thedifferentiation lashes. But in epiblepharon, both the lid marginCryptophthalmos and the lashes are positioned normally; it’s the excess skin and muscle that pushes the lashes against the cornea.  Associated with Goldenhar  Congenital ectropion

Two other common lid disorders also involves apposition of the lashes to the cornea. What are they? QAEntropion and trichiasis Congenital lid abnormalities: Matching In a few words, define… Entropion: An inward rotation of the eyelid margin  Ankyloblepharon Trichiasis: An inward rotation of eyelashes  Blepharophimosis So is epiblepharon a form of entropion, or trichiasis?  FailureNeither. Note of that, lid by differentiation definition, entropion involves an inward turninCryptophthalmosg of the lid margin, and trichiasis an inward turning of the lashes. But in epiblepharon, both the lid margin and the lashes are  Associatedpositioned normally; with it’s the Goldenhar excess skin and muscle that pushe s theCongenital lashes against ectropion the cornea.

Two other common lid disordersCongenital also involves apposiLid Abnormalitiestion of the lashes to the cornea. What are they? Entropion and trichiasis Congenital lid abnormalities: Matching QATo summarize (this is worth re-reading until you grock it): In a fewIn entropion words, define…, the abnormally-positioned lid margin causes the normally-directed Entropionlashes: An to inward abut therotation ocular of the surface; eyelid margin  Ankyloblepharon Trichiasisin trichasis: An inward, abnormally-directed rotation of eyelashes lashes growing from the normally-positioned lid margin abut the ocular surface; and  Blepharophimosis So isin epiblepharon epiblepharon a form, an of overridingentropion, or band trichiasis? of tissue causes the normally-directed  FailureNeitherlashes. Note of growing that, lid by differentiation fromdefinition, the normallyentropion -positionedinvolves an inward lid margin turninCryptophthalmos gto of abut the lidthe margin, ocular and surface trichiasis an inward turning of the lashes. But in epiblepharon, both the lid margin and the lashes are  Associatedpositioned normally; with it’s the Goldenhar excess skin and muscle that pushe s theCongenital lashes against ectropion the cornea.

Two other common lid disordersCongenital also involves apposiLid Abnormalitiestion of the lashes to the cornea. What are they? QAEntropion and trichiasis Congenital lid abnormalities: Matching In a few words, define… Entropion: An inward rotation of the eyelid margin  Ankyloblepharon Trichiasis: An inward rotation of eyelashes  Blepharophimosis YouSo evaluateis epiblepharon an infant a formwith epiblepharonof entropion, or and trichiasis? extensive  lash-corneaFailureNeither. Note touch.of that, lid Should by differentiation definition, this be managedentropion surgicallyinvolves an inward turninCryptophthalmosg of the lid margin, and trichiasis oran medically, inward turning and why? of the lashes. But in epiblepharon, both the lid margin and the lashes are  Medically.Associatedpositioned Infant’s normally; eyelashes with it’s the Goldenhar areexcess very skin soft, and and muscle that pushe s theCongenital lashes against ectropion the cornea. typically do not cause significant corneal damage. Epiblepharon usually resolves spontaneously as the  Fusion of all or part of  facial planes mature. If it has not resolved by the time Epiblepharon coarse,the upper/lower mature lashes have developed--andmargins the cornea is suffering--surgical intervention should be Children Dystopia of what twocanthorum ethnic heritages are  considered.Type of congenital entropion most likely to present with epiblepharon  (and whichCongenital is #1)? entropion  Redundant lid tissue causes Asian (#1) and Native-American  Euryblepharon lashes to abut ocular surface Does epiblepharon usually involve the upper lid, or the lower? Congenital tarsal kink The lower (epiblepharon rarely if ever occurs in the upper lid)  Epicanthus (epicanthal folds) Does it typically involve the entirety of the lower lid, or only a portion?  Lid coloboma Typically it involves only the nasal portion  Congenital ptosis 68

Epiblepharon. In this child the lower lid lashes have turned in from birth, but the cornea has remained undamaged. 70 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 71 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 72 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 InFailure euryblepharon, of lid which differentiation portion (ie, medial, central, temporal) Cryptophthalmos of the fissure is widened? The temporal  Associated with Goldenhar  Congenital ectropion What is responsible for this widening, ie, is it the upper, or the lower lid that is displaced?  TheFusion temporal of portion all or of partthe lower of lid is displaced inferiorly Epiblepharon the upper/lower margins Does euryblepharon require surgical intervention?  Dystopia canthorum  ItType does if ofit results congenital in exposure entropion keratitis  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 73 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 InFailure euryblepharon, of lid which differentiation portion (ie, medial, central, temporal) Cryptophthalmos of the fissure is widened? The temporal portion  Associated with Goldenhar  Congenital ectropion What is responsible for this widening, ie, is it the upper, or the lower lid that is displaced?  TheFusion temporal of portion all or of partthe lower of lid is displaced inferiorly Epiblepharon the upper/lower margins Does euryblepharon require surgical intervention?  Dystopia canthorum  ItType does if ofit results congenital in exposure entropion keratitis  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 74 Congenital Lid Abnormalities

Euryblepharon 75 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 InFailureeuryblepharon of lid, whichdifferentiation portion (ie, medial, central, temporal) Cryptophthalmos of the fissure is widened? The temporal portion  Associated with Goldenhar  Congenital ectropion What is responsible for this widening, ie,With is regard it the upper,to which or portion the lower of the lidlid thatis involved…Recall is displaced? thisthis question question regarding which portion of the lid is involved  Fusion of all or part of  Epiblepharon The temporal portion of the lower lid isin displaced epiblepharon. inferiorly What was the answer? the upper/lower margins The nasal portion Does euryblepharon require surgical intervention?  Dystopia canthorum  ItType does if ofit results congenital in exposure entropion keratitisSo, take note: Euryblepharon and epiblepharon involve opposite ends of the lid.Congenital I point this out because,entropion if you can  Redundant lid tissue causesremember which portion is involved for either condition, along with the fact that Euryblepharonthey’re the opposite of one another, lashes to abut ocular surfaceyou will know which portion is involved for both conditions.  Congenital tarsal kink DoesAbnormalepiblepharon wideningusually involve theof upper lid, or the lower? The lower (epiblepharon rarely if ever occurs in the upper lid) interpalpebral fissure  Epicanthus (epicanthal folds) Does it typically involve the entirety of the lower lid, or only a portion? temporal v  Lid coloboma Typically it involves only the nasalmedial v portion nasal  Congenital ptosis 76 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 InFailureeuryblepharon of lid, whichdifferentiation portion (ie, medial, central, temporal) Cryptophthalmos of the fissure is widened? The temporal portion  Associated with Goldenhar  Congenital ectropion What is responsible for this widening, ie,With is regard it the upper,to which or portion the lower of the lidlid thatis involved…Recall is displaced? thisthis question question regarding which portion of the lid is involved  Fusion of all or part of  Epiblepharon The temporal portion of the lower lid isin displaced epiblepharon. inferiorly What was the answer? the upper/lower margins The nasal portion Does euryblepharon require surgical intervention?  Dystopia canthorum  ItType does if ofit results congenital in exposure entropion keratitisSo, take note: Euryblepharon and epiblepharon involve opposite ends of the lidCongenital. I point this out because,entropion if you can  Redundant lid tissue causesremember which portion is involved for either condition, along with the fact that Euryblepharonthey’re the opposite of one another, lashes to abut ocular surfaceyou will know which portion is involved for both conditions.  Congenital tarsal kink DoesAbnormalepiblepharon wideningusually involve theof upper lid, or the lower? The lower (epiblepharon rarely if ever occurs in the upper lid) interpalpebral fissure  Epicanthus (epicanthal folds) Does it typically involve the entirety of the lower lid, or only a portion?  Lid coloboma Typically it involves only the nasal portion  Congenital ptosis 77 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Lid coloboma

 Congenital ptosis 78 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Lid coloboma

 Congenital ptosis 79 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Lid coloboma

 Congenital ptosis 80 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Lid coloboma

 Congenital ptosis 81 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Lid coloboma

 Congenital ptosis 82 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Lid coloboma

 Congenital ptosis 83 Congenital Lid Abnormalities

Dystopia canthorum. Note the telecanthus, and laterally displaced lacrimal puncta 84 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Lid coloboma

 Congenital ptosis 85 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Lid coloboma

 Congenital ptosis 86 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion What is dystopia canthorum?  Congenital entropion  Redundant Laterallid tissue displacement causes of the canthi (ie, telecanthus) PLUS laterally displaced lacrimal puncta  Euryblepharon lashes to abutHow onocular earth are yousurface supposed to recognize that the puncta are ‘too lateral’? Draw an imaginary vertical line from the upper to theCongenital lower puncta. tarsalIf this line kink crosses the  Abnormal wideningcornea, the puncta of are displaced. (Next time you examine a pt at the slit-lamp, take note of What is whetherthe difference such a betweenline crosse telecanthuss their corneaand [it hypertelorismwon’t].) ? interpalpebralTelecanthus fissurerefers to an abnormally increased distance Epicanthus between the medial(epicanthal canthi , whereas folds) hypertelorism refers to an abnormally increased distance between the medial orbital walls  Lid coloboma Which manifests as an increased interpupillary distance? Hypertelorism  Congenital ptosis 87 Congenital Lid Abnormalities Q/A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion What is dystopia canthorum?  Congenital entropion  Redundant Laterallid tissue displacement causes of the canthi (ie, telecanthus) PLUS laterally displaced lacrimal puncta  Euryblepharon lashes to abutHow onocular earth are yousurface supposed to recognize that the puncta are ‘too lateral’? Draw an imaginary vertical line from the upper to theCongenital lower puncta. tarsalIf this line kink crosses the  Abnormal wideningcornea, the puncta of are displaced. (Next time you examine a pt at the slit-lamp, take note of What is whetherthe difference such a betweenline crosse telecanthuss their corneaand [it hypertelorismwon’t].) ? interpalpebralTelecanthus fissurerefers to an abnormally increased distance Epicanthus between the medial(epicanthaltwo words canthi , whereas folds) hypertelorism refers to an abnormally increased distance between the medialthree orbital words walls  Lid coloboma Which manifests as an increased interpupillary distance? Hypertelorism  Congenital ptosis 88 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion What is dystopia canthorum?  Congenital entropion  Redundant Laterallid tissue displacement causes of the canthi (ie, telecanthus) PLUS laterally displaced lacrimal puncta  Euryblepharon lashes to abutHow onocular earth are yousurface supposed to recognize that the puncta are ‘too lateral’? Draw an imaginary vertical line from the upper to theCongenital lower puncta. tarsalIf this line kink crosses the  Abnormal wideningcornea, the puncta of are displaced. (Next time you examine a pt at the slit-lamp, take note of What is whetherthe difference such a betweenline crosse telecanthuss their corneaand [it hypertelorismwon’t].) ? interpalpebralTelecanthus fissurerefers to an abnormally increased distance Epicanthus between the medial(epicanthal canthi , whereas folds) hypertelorism refers to an abnormally increased distance between the medial orbital walls  Lid coloboma Which manifests as an increased interpupillary distance? Hypertelorism  Congenital ptosis 89 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion What is dystopia canthorum?  Congenital entropion  Redundant Laterallid tissue displacement causes of the canthi (ie, telecanthus) PLUS laterally displaced lacrimal puncta  Euryblepharon lashes to abutHow onocular earth are yousurface supposed to recognize that the puncta are ‘too lateral’? Draw an imaginary vertical line from the upper to theCongenital lower puncta. tarsalIf this line kink crosses the  Abnormal wideningcornea, the puncta of are displaced. (Next time you examine a pt at the slit-lamp, take note of What is whetherthe difference such a betweenline crosse telecanthuss their corneaand [it hypertelorismwon’t].) ? interpalpebralTelecanthus fissurerefers to an abnormally increased distance Epicanthus between the medial(epicanthal canthi , whereas folds) hypertelorism refers to an abnormally increased distance between the medial orbital walls  Lid coloboma Which manifests as an increased interpupillary distance? Hypertelorism  Congenital ptosis 90 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion What is dystopia canthorum?  Congenital entropion  Redundant Laterallid tissue displacement causes of the canthi (ie, telecanthus) PLUS laterally displaced lacrimal puncta  Euryblepharon lashes to abutHow onocular earth are yousurface supposed to recognize that the puncta are ‘too lateral’? Draw an imaginary vertical line from the upper to theCongenital lower puncta. tarsalIf this line kink crosses the  Abnormal wideningcornea, the puncta of are displaced. (Next time you examine a pt at the slit-lamp, take note of What is whetherthe difference such a betweenline crosse telecanthuss their corneaand [it hypertelorismwon’t].) ? interpalpebralTelecanthus fissurerefers to an abnormally increased distance Epicanthus between the medial(epicanthal canthi , whereas folds) hypertelorism refers to an abnormally increased distance between the medial orbital walls  Lid coloboma Which manifests as an increased interpupillary distance? Hypertelorism  Congenital ptosis 91 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion Say…Why does this person with dystopia canthorum also haveFusion heterochromia of alliridis? or part of  Epiblepharon Becausethe upper/lowerdystopia canthorum andmargins heterochromia iridis, along with synophrys, are the three classic ophthalmic hallmarks of  Dystopia canthorum WaardenbergType of syndrome congenital entropion  Congenital entropion WhatRedundant non-ophthalmic lidfinding tissue is classic causes for Waardenburg syndrome?  Euryblepharon Thelashes presence ofto a whiteabut forelock ocular (ie, an surface isolated streak of white hair in the forehead region)  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 92 Congenital Lid Abnormalities Q/A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion Say…Why does this person with dystopia canthorum also haveFusion heterochromia of alliridis? or part of  Epiblepharon Becausethe upper/lowerdystopia canthorum andmargins heterochromia iridis, along with synophrys, are the three classic ophthalmic hallmarks of  Dystopia canthorum WaardenbergTypeeponym ofsyndrome congenital entropion  Congenital entropion WhatRedundant non-ophthalmic lidfinding tissue is classic causes for Waardenburg syndrome?  Euryblepharon Thelashes presence ofto a whiteabut forelock ocular (ie, an surface isolated streak of white hair in the forehead region)  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 93 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion Say…Why does this person with dystopia canthorum also haveFusion heterochromia of alliridis? or part of  Epiblepharon Becausethe upper/lowerdystopia canthorum andmargins heterochromia iridis, along with synophrys, are the three classic ophthalmic hallmarks of  Dystopia canthorum WaardenbergType ofsyndrome congenital entropion  Congenital entropion WhatRedundant non-ophthalmic lidfinding tissue is classic causes for Waardenburg syndrome?  Euryblepharon Thelashes presence ofto a whiteabut forelock ocular (ie, an surface isolated streak of white hair in the forehead region)  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 94 Congenital Lid Abnormalities

Waardenburg syndrome: Heterochromia iridis, dystopia canthorum, and mild synophrys (What the heck is synophrys?) 95 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion Say…Why does this person with dystopia canthorum also haveFusion heterochromia of alliridis? or part of  Epiblepharon Because dystopia canthorum and heterochromia iridis, along the upper/lowerWhat the heck margins is synophrys? with synophrys, are the three classic ophthalmic hallmarks of  Dystopia canthorum The formal medical term for a unibrow WaardenbergType ofsyndrome congenital entropion  Congenital entropion WhatRedundant non-ophthalmic lidfinding tissue is classic causes for Waardenburg syndrome?  Euryblepharon Thelashes presence ofto a whiteabut forelock ocular (ie, an surface isolated streak of white hair in the forehead region)  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 96 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion Say…Why does this person with dystopia canthorum also haveFusion heterochromia of alliridis? or part of  Epiblepharon Because dystopia canthorum and heterochromia iridis, along the upper/lowerWhat the heck margins is synophrys? with synophrys, are the three classic ophthalmic hallmarks of  Dystopia canthorum The formal medical term for a unibrow WaardenbergType ofsyndrome congenital entropion  Congenital entropion WhatRedundant non-ophthalmic lidfinding tissue is classic causes for Waardenburg syndrome?  Euryblepharon Thelashes presence ofto a whiteabut forelock ocular (ie, an surface isolated streak of white hair in the forehead region)  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 97 Congenital Lid Abnormalities

Waardenburg syndrome: Synophrys 98 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion Say…Why does this person with dystopia canthorum also haveFusion heterochromia of alliridis? or part of  Epiblepharon Becausethe upper/lowerdystopia canthorum andmargins heterochromia iridis, along with synophrys, are the three classic ophthalmic hallmarks of  Dystopia canthorum WaardenbergType ofsyndrome congenital entropion  Congenital entropion WhatRedundant non-ophthalmic lidcosmetic tissue finding causes is classic for Waardenburg syndrome?  Euryblepharon Thelashes presence ofto a whiteabut forelock ocular (ie, an surface isolated streak of white hair in the forehead region)  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 99 Congenital Lid Abnormalities A Congenital lid abnormalities: Matching  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion Say…Why does this person with dystopia canthorum also haveFusion heterochromia of alliridis? or part of  Epiblepharon Becausethe upper/lowerdystopia canthorum andmargins heterochromia iridis, along with synophrys, are the three classic ophthalmic hallmarks of  Dystopia canthorum WaardenbergType ofsyndrome congenital entropion  Congenital entropion WhatRedundant non-ophthalmic lidcosmetic tissue finding causes is classic for Waardenburg syndrome?  Euryblepharon Thelashes presence ofto a whiteabut forelock ocular (ie, an surface isolated streak of white hair in the forehead region)  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 100 Congenital Lid Abnormalities

Waardenburg syndrome: White forelock 101 Congenital Lid Abnormalities

Note that Waardenburg syndrome has forms that do not involve heterochromia 102 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 103 Congenital Lid Abnormalities A  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos

 Associated with Goldenhar  Congenital ectropion

 Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 104 Congenital Lid Abnormalities Q What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiationmnemonic forthcoming… Cryptophthalmos --Epicanthus p: Upper and lower equally  --EpicanthusAssociated withs: From Goldenhar brow to lower lid  Congenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 105 Congenital Lid Abnormalities Q What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus t arsalis(start here): Primarily upper lid Blepharophimosis --Epicanthus i nversus: Primarily lower lid  Failure of lid differentiationmnemonic forthcoming… CryptophthalmosTIPS --Epicanthus p : Upper and lower equally  --EpicanthusAssociated withs : From Goldenhar brow to lower lid  Congenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 106 Congenital Lid Abnormalities Q/A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus i nversus(next) : Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus p : Upper and lower equally  --EpicanthusAssociated withs : From Goldenhar brow to lower lid  Congenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 107 Congenital Lid Abnormalities Q/A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus p (next): Upper and lower equally  --EpicanthusAssociated withs : From Goldenhar brow to lower lid  Congenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 108 Congenital Lid Abnormalities Q/A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis and lower equally  --EpicanthusAssociated withs :(next) From Goldenhar brow to lower lid  Congenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 109 Congenital Lid Abnormalities A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis and lower equally  --EpicanthusAssociated withsupraciliaris Goldenhar: brow to lower lidCongenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 110 Congenital Lid Abnormalities Q What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily(start here) upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis and lower equally  --EpicanthusAssociated withsupraciliaris Goldenhar: brow to lower lidCongenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 111 Congenital Lid Abnormalities Q/A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis and lower equally  --EpicanthusAssociated withsupraciliaris Goldenhar: brow to lower lidCongenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 112 Congenital Lid Abnormalities

Epicanthus tarsalis 113 Congenital Lid Abnormalities Q/A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily(next) lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis and lower equally  --EpicanthusAssociated withsupraciliaris Goldenhar: brow to lower lidCongenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 114 Congenital Lid Abnormalities Q/A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis and lower equally  --EpicanthusAssociated withsupraciliaris Goldenhar: brow to lower lidCongenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 115 Congenital Lid Abnormalities

Epicanthus inversus 116 Congenital Lid Abnormalities Q/A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: and(next) lower equally  --EpicanthusAssociated withsupraciliaris Goldenhar: brow to lower lidCongenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 117 Congenital Lid Abnormalities Q/A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: Upper and lower lids equally  --EpicanthusAssociated withsupraciliaris Goldenhar: brow to lower lidCongenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 118 Congenital Lid Abnormalities

Epicanthus palpebralis 119 Congenital Lid Abnormalities Q/A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: Upper and lower lids equally  --EpicanthusAssociated withsupraciliaris: Goldenhar: brow(next) to lower lidCongenital ectropion  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 120 Congenital Lid Abnormalities A What are the four types of epicanthus? What’sAnkyloblepharon involved for each? --Epicanthus tarsalis: Primarily upper lid Blepharophimosis --Epicanthus inversus: Primarily lower lid  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: Upper and lower lids equally  --EpicanthusAssociated withsupraciliaris: Goldenhar: From the brow Congenitalto the lower ectropion lid  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 121 Congenital Lid Abnormalities

Epicanthus supraciliaris 122 Congenital Lid Abnormalities

Epicanthal folds overview 123 Congenital Lid Abnormalities Q What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris Goldenhar: : From(with the the possible brow exceptionCongenitalto the of lower inversus) ectropion lid  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 124 Congenital Lid Abnormalities A What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris Goldenhar: : From(with the the possible brow exceptionCongenitalto the of lower inversus) ectropion lid  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 125 Congenital Lid Abnormalities Q What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris Goldenhar: : From(with the the possible brow exceptionCongenitalto the of lower inversus) ectropion lid  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 126 Congenital Lid Abnormalities A What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris Goldenhar: : From(with the the possible brow exceptionCongenitalto the of lower inversus) ectropion lid  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 127 Congenital Lid Abnormalities Q What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: Upper--Is associated and lower with lidsptosis? equally All of them What is pseudostrabismus? --Resolves without surgical intervention? All   --EpicanthusAssociatedA false impression withsupraciliaris (on theGoldenhar part of an: examiner): From(with the rethe thepossible presencebrow exceptionCongenital ofto strabismus the of lower inversus) ectropion lid

 FusionAre pts withof allprominent or part epicanthal of folds more likely to be (mis)diagnosed Epiblepharon with esotropia, or exotropia? theEsotropia upper/lower margins  Dystopia canthorum  TypeWhat of is it congenital about the appearance entropion of these pts that might lead one to incorrectly conclude the pt is ET? The epicanthal folds cover some of the normally-visible sclera, Congenital thereby giving theentropion impression the eyes  Redundantare turned in lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 128 Congenital Lid Abnormalities A What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: Upper--Is associated and lower with lidsptosis? equally All of them What is pseudostrabismus? --Resolves without surgical intervention? All   --EpicanthusAssociatedA false impression withsupraciliaris (on theGoldenhar part of an: examiner): From(with the rethe thepossible presencebrow exceptionCongenital ofto strabismus the of lower inversus) ectropion lid

 Congenital ptosis 129 Congenital Lid Abnormalities Q What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: Upper--Is associated and lower with lidsptosis? equally All of them What is pseudostrabismus? --Resolves without surgical intervention? All   --EpicanthusAssociatedA false impression withsupraciliaris (on theGoldenhar part of an: examiner): From(with the rethe thepossible presencebrow exceptionCongenital ofto strabismus the of lower inversus) ectropion lid

 Congenital ptosis 130 Congenital Lid Abnormalities A What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: Upper--Is associated and lower with lidsptosis? equally All of them What is pseudostrabismus? --Resolves without surgical intervention? All   --EpicanthusAssociatedA false impression withsupraciliaris (on theGoldenhar part of an: examiner): From(with the rethe thepossible presencebrow exceptionCongenital ofto strabismus the of lower inversus) ectropion lid

 Congenital ptosis 131 Congenital Lid Abnormalities Q What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: Upper--Is associated and lower with lidsptosis? equally All of them What is pseudostrabismus? --Resolves without surgical intervention? All   --EpicanthusAssociatedA false impression withsupraciliaris (on theGoldenhar part of an: examiner): From(with the rethe thepossible presencebrow exceptionCongenital ofto strabismus the of lower inversus) ectropion lid

 Congenital ptosis 132 Congenital Lid Abnormalities A What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation  Cryptophthalmos --Epicanthus palpebralis: Upper--Is associated and lower with lidsptosis? equally All of them What is pseudostrabismus? --Resolves without surgical intervention? All   --EpicanthusAssociatedA false impression withsupraciliaris (on theGoldenhar part of an: examiner): From(with the rethe thepossible presencebrow exceptionCongenital ofto strabismus the of lower inversus) ectropion lid

 Congenital ptosis 133 Congenital Lid Abnormalities Q What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris Goldenhar: : From(with the the possible brow exceptionCongenitalto the of lower inversus) ectropion lid  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 134 Congenital Lid Abnormalities A What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris Goldenhar: : From(with the the possible brow exceptionCongenitalto the of lower inversus) ectropion lid  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 135 Congenital Lid Abnormalities Q What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris Goldenhar: : From(with the the possible brow exceptionCongenitalto the of lower inversus) ectropion lid  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 136 Congenital Lid Abnormalities A What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can lower produce lidpseudostrabismus? All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris Goldenhar: : From(with the the possible brow exceptionCongenitalto the of lower inversus) ectropion lid  Fusion of all or part of  Epiblepharon the upper/lower margins  Dystopia canthorum  Type of congenital entropion  Congenital entropion  Redundant lid tissue causes  Euryblepharon lashes to abut ocular surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 137 Congenital Lid Abnormalities Q What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can produce lower pseudostrabismus?lid All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris: Goldenhar: From(with the the possible brow Congenital exceptionto the lower of ectropion inversus lid )  Fusion of all or part of  Epiblepharon the upper/lowerWhy the hedging margins about whether epicanthus inversus can resolve without surgery? Because the BCSC books are somewhat in conflict on theDystopia subject (at canthorum least, the most recent editions in my possession are).  Type of--Per congenital the Peds book: “Becauseentropion the epicanthus [inversus…] may improve with age, repair of the  defects is often delayed.” Congenital entropion  Redundant--Per the lidOrbit tissuebook: “Most causes forms of epicanthus resolve with normal growth…Epicanthus inversus, however, rarely respond to facial growth.” Euryblepharon lashesSo to caveat abut emptor, ocular peeps. surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 138 Congenital Lid Abnormalities A What are the four types of epicanthus?Which type(s):  What’sAnkyloblepharon involved for each? --Is considered a normal variant when found in --Epicanthus tarsalis: Primarilya child upper of Eastlid Asian Blepharophimosis descent? Tarsalis --Epicanthus inversus: Primarily--Can produce lower pseudostrabismus?lid All of them  Failure of lid differentiation--Is associated withCryptophthalmos ptosis? All of them --Epicanthus palpebralis: Upper--Resolves and withoutlower surgicallids equally intervention? All  --EpicanthusAssociated withsupraciliaris: Goldenhar: From(with the the possible brow Congenital exceptionto the lower of ectropion inversus lid )  Fusion of all or part of  Epiblepharon the upper/lowerWhy the hedging margins about whether epicanthus inversus can resolve without surgery? Because the BCSC books are somewhat in conflict on theDystopia subject (at canthorum least, the most recent editions in my possession are).  Type of--Per congenital the Peds book: “Becauseentropion the epicanthus [inversus…] may improve with age, repair of the  defects is often delayed.” Congenital entropion  Redundant--Per the lidOrbit tissuebook: “Most causes forms of epicanthus resolve with normal growth…Epicanthus inversus, however, rarely respond to facial growth.” Euryblepharon lashesSo to caveat abut emptor, ocular peeps. surface  Congenital tarsal kink  AbnormalWhat does the term widening epicanthus refer of to in this context? An epicanthus is a fold of skin extending above and interpalpebralbelow the medial canthal fissure region. How far the fold  Epicanthus (epicanthal folds) extends in each direction is a function of the type of epicanthus--more on this shortly.  Lid coloboma

 Congenital ptosis 139 Congenital Lid Abnormalities Q  Ankyloblepharon

 Blepharophimosis

 Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the threefour other other margins defining defining features ophthal micof blepharophimosis features of blepharophimosis syndrome? syndrome? --Epicanthus--Epicanthus inversus  Dystopia canthorum  Type--Telecanthus--Telecanthus of congenital entropion --Ptosis--Ptosis  Congenital entropion  Redundantand, of course, lid tissue causes  Euryblepharon lashes--Blepharophimosis--Blepharophimosis to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 140 Congenital Lid Abnormalities A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the threefour other other margins defining defining ophthal ophthalmicmic features features of of blepharophimosis blepharophimosis syndrome? syndrome? ----EpicanthusEpicanthus inversus  Dystopia canthorum  Type----TelecanthusTelecanthus of congenital entropion ----PtosisPtosis  Congenital entropion  Redundantand, of course, lid tissue causes  Euryblepharon lashes----BlepharophimosisBlepharophimosis to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 141 Congenital Lid Abnormalities Q  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? ----EpicanthusEpicanthus inversus  Dystopia canthorum  Type--Telecanthus?Telecanthus of congenital entropion --Ptosis?Ptosis  Congenital entropion  Redundantand, of course, lid tissue causes  Euryblepharon lashes--Blepharophimosis?Blepharophimosis to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 142 Congenital Lid Abnormalities Q/A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? ----EpicanthusEpicanthus inversus  Dystopia canthorum  Type----TelecanthusTelecanthus of congenital entropion ----PtosisPtosis  Congenital entropion  Redundantand, of course, lid tissue causes  Euryblepharon lashes--Blepharophimosis? to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 143 Congenital Lid Abnormalities A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? ----EpicanthusEpicanthus inversus  Dystopia canthorum  Type----TelecanthusTelecanthus of congenital entropion ----PtosisPtosis  Congenital entropion  Redundantand, of course, lid tissue causes  Euryblepharon lashes----BlepharophimosisBlepharophimosis to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 144 Congenital Lid Abnormalities

Blepharophimosis syndrome 145 Congenital Lid Abnormalities Q  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? ----EpicanthusEpicanthus inversus  Dystopia canthorum  Type----TelecanthusTelecanthus of congenital entropion ----PtosisPtosis  Congenital entropion  Redundantand, of course, lid tissueWhat causes does it mean to say a pt has blepharophimosis? --Blepharophimosis It means her/his palpebral fissures Euryblepharon are abnormally lashes to abut ocularshort surface in both vertical and horizontal extent  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 146 Congenital Lid Abnormalities A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? ----EpicanthusEpicanthus inversus  Dystopia canthorum  Type----TelecanthusTelecanthus of congenital entropion ----PtosisPtosis  Congenital entropion  Redundantand, of course, lid tissueWhat causes does it mean to say a pt has blepharophimosis? --Blepharophimosis It means her/his palpebral fissures Euryblepharon are abnormally lashes to abut ocularshort surface in both vertical and horizontal extent  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 147 Congenital Lid Abnormalities Q  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? ----EpicanthusEpicanthus inversus  Dystopia canthorum ----TelecanthusTelecanthus  Type of congenital entropionBlepharophimosis syndrome has two other names. What are they? ----PtosisPtosis --  Congenital entropion  Redundantand, of course, lid tissue --causesBlepharophimosis-ptosis-epic anthus inversus syndrome (BPES)  Euryblepharon lashes----BlepharophimosisBlepharophimosis to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 148 Congenital Lid Abnormalities Q/A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? ----EpicanthusEpicanthus inversus  Dystopia canthorum ----TelecanthusTelecanthus  Type of congenital entropionBlepharophimosis syndrome has two other names. What are they? ----PtosisPtosis --Congenital eyelid syndrome Congenital entropion  Redundantand, of course, lid tissue --causesBlepharophimosis-ptosis-epic anthus inversus syndrome (BPES)  Euryblepharon lashes----BlepharophimosisBlepharophimosis to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 149 Congenital Lid Abnormalities A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? --Epicanthus inversus inversus  Dystopia canthorum ----TelecanthusTelecanthus  Type of congenital entropionBlepharophimosis syndrome has two other names. What are they? --Ptosis --Congenital eyelid syndrome Congenital entropion  Redundantand, of course, lid tissue --Blepharophimosis-ptosis-epiccauses anthus inversus syndrome (BPES)  Euryblepharon lashes--Blepharophimosis to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 150 Congenital Lid Abnormalities Q  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? --Epicanthus inversus  Dystopia canthorum  Type--Telecanthus of congenitalIs the ptosis purely entropion structural (ie, 2ndry to blepharophimosis), --Ptosis or is there a problem with the levator?  Congenital entropion  Redundantand, of course,In lidfact levatortissue function causes is usually very poor  Euryblepharon lashes--Blepharophimosis to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 151 Congenital Lid Abnormalities A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? ----EpicanthusEpicanthus inversus  Dystopia canthorum  Type----TelecanthusTelecanthus of congenitalIs the ptosis purely entropion structural (ie, 2ndry to blepharophimosis), --Ptosis or is there a problem with the levator?  Congenital entropion  Redundantand, of course,In lidfact levatortissue function causes is usually very poor  Euryblepharon lashes----BlepharophimosisBlepharophimosis to abut ocular surface  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 152 Congenital Lid Abnormalities Q  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? --Epicanthus inversus  Dystopia canthorum  Type--Telecanthus of congenital entropion --Ptosis Are there any ophthalmic manifestationsCongenital beyond entropion these?  Redundantand, of course, lid tissue causesTwo of note: Hypertelorism and ectropion  Euryblepharon lashes--Blepharophimosis to abut ocular surface Poor levator function  Congenital tarsal kink  Abnormal widening of interpalpebral fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 153 Congenital Lid Abnormalities Q/A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? --Epicanthus inversus  Dystopia canthorum  Type--Telecanthus of congenital entropion --Ptosis Are there any ophthalmic manifestationsCongenital beyond entropion these?  Redundantand, of course, lid tissue causesTwo of note: Hypertelorism and ectropion  Euryblepharon lashes--Blepharophimosis to abut ocular surface Poor levator function  Congenital tarsal kink  Abnormal? widening of interpalpebral? fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 154 Congenital Lid Abnormalities A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of all or part of  Epiblepharon theWhat upper/lower are the three other margins defining ophthalmic features of blepharophimosis syndrome? --Epicanthus inversus  Dystopia canthorum  Type--Telecanthus of congenital entropion --Ptosis Are there any ophthalmic manifestationsCongenital beyond entropion these?  Redundantand, of course, lid tissue causesTwo of note: Hypertelorism and ectropion  Euryblepharon lashes--Blepharophimosis to abut ocular surface Poor levator function  Congenital tarsal kink  AbnormalHypertelorism widening of interpalpebralEctropion fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 155 Congenital Lid Abnormalities Q  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of allWhat or part are the of two main non -  Epiblepharon What are the three other defining ophthalmic features of blepharophimosis syndrome? the upper/lower margins^ --Epicanthus inversus --  Dystopia canthorum  Type--Telecanthus of congenital entropion-- --Ptosis  Congenital entropion  Redundantand, of course, lid tissue causes  Euryblepharon lashes--Blepharophimosis to abut ocular surface Poor levator function  Congenital tarsal kink  AbnormalHypertelorism widening of interpalpebralEctropion fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 156 Congenital Lid Abnormalities Q/A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of allWhat or part are the of two main non -  Epiblepharon What are the three other defining ophthalmic features of blepharophimosis syndrome? the upper/lower margins^ --Epicanthus inversus --Hypoplastic nasal twoDystopia wordsbridge canthorum --Telecanthus one  Type of congenital entropion-- ’Lop‘word ears --Ptosis  Congenital entropion  Redundantand, of course, lid tissue causes  Euryblepharon lashes--Blepharophimosis to abut ocular surface Poor levator function  Congenital tarsal kink  AbnormalHypertelorism widening of interpalpebralEctropion fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 157 Congenital Lid Abnormalities A  Ankyloblepharon syndrome  Blepharophimosis ^  Failure of lid differentiation  Cryptophthalmos Speaking of epicanthus inversus…It is exclusively syndromic. With which syndrome is  Associatedit most closely associated?with Goldenhar  Congenital ectropion Blepharophimosis syndrome  Fusion of allWhat or part are the of two main non -  Epiblepharon What are the three other defining ophthalmic features of blepharophimosis syndrome? the upper/lower margins^ --Epicanthus inversus --Hypoplastic nasal Dystopia bridge canthorum  Type--Telecanthus of congenital entropion-- ’Lop‘ ears --Ptosis  Congenital entropion  Redundantand, of course, lid tissue causes  Euryblepharon lashes--Blepharophimosis to abut ocular surface Poor levator function  Congenital tarsal kink  AbnormalHypertelorism widening of interpalpebralEctropion fissure  Epicanthus (epicanthal folds)

 Lid coloboma

 Congenital ptosis 158 Congenital Lid Abnormalities

Hypoplastic nasal bridge Lop ears

Blepharophimosis syndrome