Congenital Eyelid Disorders

1. Congenital Eyelid Disorders Juan G. Santiago, MD Department of Ophthalmology UPR School of Medicine

2. Ablepharon • Absence of lids

3. Ankyloblepharon • Partial or complete fusion of lid margins • Temporal • Bilateral • AD • Associated with craniofacial abnormalities • Secondary • Thermal or chemical burns, inflammation, OCP, SJS

4. Blepharophimosis • Horizontally and vertically shortened palpebral fissures with poor levator function • Absent lid crease • Treatment • Surgical correction

5. Blepharophimosis Syndrome • AD (Chromosome 3q) • Ptosis • Telecanthus • Epicanthus inversus • Lower lid ectropion • Hypoplasia of nasal bridge and sup orbital rim • Anteverted ears • Hypertelorism

6. Coloboma • Embryologic cleft involving lid margin • Upper lid • Medial 1/3 • No systemic abnormalities • Full thickness • Lower lid • Lateral 1/3 • Systemic abnormalities • Treacher Collins • Goldenhar’s syndrome • Partial thickness • May have exposure keratitis

7. Droopy eyelid 75% unilateral Nonhereditary Rarely causes amblyopia Blepharophimosis syndrome Congenital Blepharoptosis

8. Myogenic Most common Dysgenesis of levator muscle Poor levator function, loss of lid crease, eyelid lag, lagophthalmos Aponeurotic Birth trauma High or indistinct lid crease Neurogenic Congenital CN 3 palsy Marcus Gunn jaw winking Jaw movement cause elevation of ptotic lid Congenital Blepharoptosis

9. Congenital Horner’s Syndrome • Ptosis, miosis, anhydrosis • May cause amblyopia • Treatment • Surgical repair (levator resections) • Severe cases  frontalis sling

10. Congenital Ectropion • Eversion of eyelid margin due to vertical shortage of anterior lamella • Etiology • Inclusion conjunctivitis • Anterior lamella inflammation • Down syndrome • Treatment • Usually not required

11. Congenital Entropion • Inversion of eyelid margin • Etiology • Lid retractor dysgenesis • Tarsal plate defects • Shortening of posterior lamella • Treatment • May require surgery

12. Distichiasis • Partial or complete accessory rows of eyelashes from or posterior to meibomian orifices • May develop trichiasis

13. Pretarsal skin and orbicularis override the lid margin Causes horizontal fold of tissue to push cilia vertically No entropion Asians Usually lower lid and resolved spontaneous Epiblepharon

14. Epicanthus • Medial canthal vertical skin folds • Due to immature facial bones or redundant skin • Usually bilateral • Produces pseudoesotropia

15. Tarsalis Fold most prominent in upper eyelid Asian eyelid Inversus Fold most prominent in lower eyelid Blepharophimosis Palpebralis Fold is equally distributed in upper and lower eyelids Supraciliaris Fold arises from eyebrow and extends to lacrimal sac Epicanthus

16. Euryblepharon • Horizontally widening of palpebral fissure due to inferior insertion of lateral canthal tendon • Associated with ectropion of lateral third of lid • Poor lid closure with exposure keratitis

17. Microblepharon • Vertical shortening of lids • May have exposure keratitis

18. Widening intercanthal distance due to long medial canthal tendons Associated with Fetal alcohol Waardenburg’s Blepharophimosis Telecanthus

19. Euryblepharon Ankyloblepharon Epicanthus tarsalis Epicanthus palpebralis Epicanthus inversus Epiblepharon Fold of skin over the medial canthus, broader in the upper lid Pretarsal muscle and skin riding above the lid margin with vertical eyelashes Fold of skin over the medial canthus, broader over the lower lid Quiz

20. Horizontal widening of palpebral fissure associated with antimongoloid slant and loss of contact between the lid and globe Partial or complete fusion of eyelids laterally by webs of skin Fold of skin over the medial canthus that is equally broad in both the upper and lower eyelids Euryblepharon Ankyloblepharon Epicanthus tarsalis Epicanthus palpebralis Epicanthus inversus Epiblepharon Quiz