Figure 17-4 Congenital eyelid coloboma (cleft), right eye. The eyelid is fused to the globe.

Ankyloblepharon

Fusion of part or all of the eyelid margins is known as ankyloblepharon. This condition may be dominantly inherited. Treatment is surgical. A variant is ankyloblepharon filiforme adnatum, in which the eyelid margins are connected by fine strands (Fig 17-5). This variant is seen in Hay-Wells syndrome (ankyloblepharon–ectodermal dysplasia–clefting syndrome), a form of ectodermal dysplasia that includes cleft lip or palate.

Figure 17-5 Ankyloblepharon filiforme adnatum. The eyelid margins are fused by a fine strand. The eyelids were easily separated with blunt scissors in the office, without anesthesia. (Courtesy of Amy Hutchinson, MD.)

Congenital Ectropion

Congenital ectropion is a disorder characterized by eversion of the eyelid margin; it usually involves the lower eyelid secondary to a vertical deficiency of the skin. Lateral tarsorrhaphy may be necessary for mild cases. More severe cases may require a skin flap or graft.

Congenital Entropion

Congenital entropion is a rare abnormality in which eyelid inversion is present at birth. It does not resolve spontaneously. Surgery should be performed when corneal integrity is threatened.

Epiblepharon

Epiblepharon is a congenital anomaly characterized by a horizontal fold of skin adjacent to either the upper or lower eyelid—most commonly, the lower eyelid—that may turn the lashes inward, against the cornea. The child’s cornea often tolerates this condition surprisingly well. Unlike congenital entropion, epiblepharon often resolves spontaneously. Ocular lubricants may be beneficial. Cases that do not resolve or that cause chronic corneal irritation require surgical repair.

Congenital Tarsal Kink

In this condition, the tarsal plate of the upper eyelid is folded at birth, resulting in entropion. The

cornea may be exposed and traumatized, leading to ulceration. The clinician can manage minor defects by manually unfolding the tarsus and taping the eyelid shut with a pressure dressing for 1–2 days. More severe cases require surgical incision of the tarsal plate or excision of a V-shaped wedge from the inner surface to permit unfolding.

Distichiasis

The presence of a partial or complete accessory row of eyelashes growing out of or slightly posterior to the meibomian gland orifices is known as distichiasis (Fig 17-6). The abnormal lashes tend to be thinner, shorter, softer, and less pigmented than normal cilia and are therefore often well tolerated. Treatment is indicated if corneal irritation is present.

Figure 17-6 Distichiasis. An accessory row of eyelashes exits from the meibomian gland orifices. (Reproduced with permission

from Patil BB, Bell R, Brice G, Jeffery S, Desai SP. Distichiasis without lymphoedema? Eye (Lond). 2004;18(12):1270–1272.)

Euryblepharon

In euryblepharon, the lateral aspect of the palpebral aperture is enlarged, with downward displacement of the temporal half of the lower eyelid. This condition gives the appearance of a very wide palpebral fissure or a droopy lower eyelid. Euryblepharon may be associated with Kabuki syndrome.

Epicanthus

Epicanthus, a crescent-shaped fold of skin running vertically between the eyelids and overlying the inner canthus, is shown in Figure 17-7. There are 4 types:

1.epicanthus tarsalis: fold is most prominent in the upper eyelid

2.epicanthus inversus: fold is most prominent in the lower eyelid

3.epicanthus palpebralis: fold is equally distributed between the upper and lower eyelids

4.epicanthus supraciliaris: fold arises from the eyebrow and terminates over the lacrimal sac

Epicanthus may be associated with blepharophimosis or ptosis, or it may be an isolated finding. Surgical correction is only occasionally required.

Figure 17-7 Epicanthus, bilateral. Top, Epicanthus tarsalis. Bottom, Epicanthus palpebralis. (Top image reproduced with permission

from Crouch E. The Child’s Eye: Strabismus and Amblyopia. Slide script. San Francisco: American Academy of Ophthalmology; 1982. Bottom image courtesy of Robert W. Hered, MD.)

Palpebral Fissure Slants

In the normal eye, the eyelids are generally positioned so that the lateral canthus is approximately 1 mm higher than the medial canthus. Slight upward or downward slanting of palpebral fissures normally occurs on a familial basis or in certain ethnic groups (eg, Asians). However, certain craniofacial syndromes frequently cause palpebral fissures to have a characteristic upward or downward slant (eg, downward slant in Treacher Collins syndrome; see Chapter 18, Fig 18-9).