Orbital dysmorphology and Eyelid
disorders
Dr. Peter Raus
Mostly complex disorders
2
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12-02-14
(early) ossification
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12-02-14
…especially the lesser sphenoid wings
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12-02-14
..or craniosynostosis
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12-02-14
Dolichocephalie (scaphocephalie)
Brachycephalie
Plagiocephalie
Acrocephalie
Oxycephalie
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12-02-14
In Ophthalmology
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Hypotelorism

Hypertelorism

(Exorbitism)
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12-02-14
Ethymology…
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
‘telouros’ = ‘distant’

‘hyper’ = ‘over’

‘hypo’ = ‘below’
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12-02-14
Hypotelorism
trigonocephaly


9
Reduced distance
between medial orbital
walls
(reduced ICD +reduced
OCD)
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12-02-14
Hypotelorism in Trigonocephaly
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Trigonocephalie
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Hypertelorism
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Lateralization of
entire orbit

(increased ICD and
OCD)
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12-02-14
Craniofrontonasal Syndrome
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Apert Syndrome
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12-02-14
Exorbitism
Crouzon
1

Reduced orbital volume
(shallow)

(and so exophthalmus)
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12-02-14
Orbital Dysmorphology : take home
message
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Lot of different syndromes
Not always genetically determined
-drugs (epilepsy, FAS,..),…
Check the whole body for DD
Exophthalmos, squint, optic atrophy, ptosis,
…
Early treatment sometimes
necessary/possible (multiple interventions!)
Dysmorphology or not?
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12-02-14
Hypotelorism?

1
Sarah Jessica Parker
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12-02-14
Hypertelorism?

1
Jacqueline KennedyOnassis
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12-02-14
Truetube: My changing face
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12-02-14
Telecanthus
primary


Increased distance
between medial canthi
Normal interpupillary
distance
secundary



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Increased distance
between medial canthi
Increased interpupillary
distance
(as in hypertelorism)
12-02-14
Dystopia Canthorum
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12-02-14
Dystopia Canthorum
Waardenburg
Syndrome(s)
 Iris, deafness, white hair
patch, D.C,…)
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12-02-14
Cryptophthalmus
(partial) absence of
eyelid(s)


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2
Corneal maldevelopment
Microphthalmia
Lagophthalmus
Eyebrow
e.g.Fraser Syndrome
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12-02-14
Congenital coloboma of eyelid



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Various degrees possible
Correction is surgical for
cosmetic reasons or
exposure keratopathy
+dermoid:
Goldenhar(cause?)
12-02-14
Ankyloblepharon
May be dominantly
inherited.
2
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12-02-14
Congenital ectropion (of eyelids)
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Vertical deficiency of skin
Down syndrome
Ichtyosis
Blepharophimosis
12-02-14
Congenital ectropium



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Difficult surgical
correction
Skin transplantation
Position of punctum
lacrimale!
12-02-14
Euryblepharon



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

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Horizontal widening
palpebral fissure
Lateral portion lower
eyelids
Vertical shortening
Horizontal lengthening
Canthal tendon?
Orbicularis insertion?
12-02-14
Euryblepharon
Victor Sciavelli
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12-02-14
Congenital entropium vs epiblepharon
epiblepharon
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entropion
12-02-14
Epicanthus
Skin fold
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Tarsalis

Inversus

Palpebralis

Supraciliaris
12-02-14
Telecanthus vs epicanthus
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3
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Blepharophimosis
Ptosis
Epicanthus inversus
12-02-14
Jumping man flap
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Trichiasis vs. distichiasis
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12-02-14
Entropium or trichiasis?
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12-02-14
Distichiasis: therapy
-<5 lashes?
(permanent) epilation
-Double row of lashes
surgical correction!
-treatment of associated
problems
(lymphedema,..)
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12-02-14
Permanent Epilation
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Surgical correction
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Congenital ptosis
-Quality of the muscle?
‘sligthly’ overcorrect.
-Amblyopia?
-Jaw winking?
-Levator function very
poor? Frontalis
suspension
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12-02-14
Frontalis Supension
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Surgery or not? Thank you!
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