Congenital Anomolies - Lid Disorders

• Cryptophthalmos
  
  • A rare failure of lid differentiation
  • Skin over eye (no lids or palpebral fissure) that may be blends in with the cornea which is usually malformed.
• Congenital Coloboma
  • Often well tolerated Involves primarily the upper lid
  • no keratopathy

Ankyloblepharon

• Fusion of part or all lid margin: variant: Ankyloblepharon filiforme adnatum in which the lid margins are connected by fine strands.

Congenital Entropion

• Usually involves the lower lid
• Distal part of tarsus rotated inward
• Lashes abrade the cornea causing keratopathy
• Permanent corneal damage is uncommon

Epiblepharon

• A horizontal fold of skin adjacent to either the upper or lower lid Tolerated well by the cornea
  • More commonly the lower lid
• Often spontaneously resolves in the initially years of life
• Surgery only indicated for severe cases

Congenital Tarsal Kink

• Child is born with the upper lid bent backwards often with a 180 degree fold in the upper tarsal plate
• Corneal exposure and rubbing by the bent edge can result in ulceration

Distichiasis

• An accessory row of lashes growing from the meibomian orifices or posteriorly
• The lashes are thinner, shorter, less pigmented and commonly well tolerated

Euryblepharon

• Enlargement of the lateral part of the palpebral aperture with downward displacement of the temporal 1/2 of the lower lid.

Epicanthus

• Crescentic fold of skin running vertically between the lids and overlying the inner canthus. There are three types:
  1. Inversus: If the fold is most prominent in the lower eyelid
  2. Tarsalis: (Most people) The fold is most prominent in the upper eyelid
  3. Palpebralis: If the fold is equally distributed in the upper and lower eyelids.

• Telecanthus
  • Normal interpupillary distance but wide intercanthal distance
    i.e. Waardenburg's syndrome
  • not: Hypertelorism, which indicates increased distance between the
    bony orbits.

Blepharophimosis

• Vertically and horizontally shortened Syndrome palpebral fissures
• Epicanthus inversus
• Telecanthus
• Ptosis: with poor levator function and no lid fold
• When should the Ptosis be repaired?
  • Frontalis slings are usually done early in life
  • Repairing telecanthus and epicanthus which might improve with age is delayed

Lipodermoid

• Lipodermoids are epibulbar, developmental growths of normal adipose in an abnormal site, ie. near the lacrimal gland and extending between the superior rectus and lateral rectus muscles posteriorly.
• Differentiation of benign lipodermoids from non-benign lesions is essential.
• When other ocular anomalies or systemic conditions are found in conjunction with lipodermoids then Goldenhar-Gorlin syndrome must be considered.