Contributor: Gordon K. Klintworth
Ablepharia (ablepharon, cryptophthalmos, cryptophthalmia) is a very rare developmental anomaly in which the anterior surface of the eye is fused with the overlying skin. The condition is usually bilateral, but is sometimes unilateral. Multiple ocular anomalies are commonly present. The anomaly is most often isolated and complete, but 20% of cases are partial. Under such circumstances the lateral part of the eyelids are normal, but the facial skin is fused to an ectatic and vascularized cornea [neovascularization - cornea] medially. Usually the eyebrow and eyelashes are totally or partially absent. A small horizontal line may be present at the site of the usual palpebral fissure. Where the eyelids fail to form the skin of the eyelids is keratinized and the dermis is fused to fibrovascular tissue, which replaces the cornea. Hair follicles, meibomian glands, accessory lacrimal glands, and glands of Zeis are all absent. The orbicularis oculi muscle is present and a bright light provokes its constriction.
The conjunctiva, superior lacrimal punctum, and the lacrimal gland are absent and xerophthalmia occurs. The anterior chamber may be small or non-existent. The crystalline lens may be present, absent or consist of a a few abortive remnants. The iris is absent or adherent to the back of the cornea and the ciliary body is atrophic. The choroid and retina may be normally developed or severely disorganized. ERG and flash-VEP may be normal. Rarely microphthalmos is present and sometimes with colobomatous cysts [cyst - colobomatous]. Ablepharia may be associated with systemic abnormalities (Fraser syndrome).