The absence of the globe and ocular tissue from the orbit.
The majority of cases of anophthalmos are seen following evisceration or enucleation. Congenital anophthalmos is rare and due to the arrest of embryogenesis during formation of the optic vesicle. A genetic, infectious or toxic etiology may be identified. Recent studies point to the role of SOX2 haploinsufficiency, as this variant was found in up to 10% of patients with anophthalmos or severe microphthalmia.
Disfigurement may result from the volume deficit. An orbital implant is typically placed at the time of evisceration or enucleation. An ocular prosthesis is fitted subsequently. In the absence of an implant (or with placement of an undersized implant), post enucleation/evisceration socket syndrome (PESS) may result, manifest as enophthalmos, deep upper eyelid sulcus, ptosis, lower lid laxity, ectropion or entropion, and shallowing of the inferior fornix.
Patients who have had an eye enucleated or removed may have socket related problems including:
- Sensitivity to the ocular prosthesis (false eye)
- Deep superior sulcus deformity ie the eye looks sunken above the upper eyelid
- Shallow fornices ie the groove between the lids and the deeper tissues is too shallow to support an ocular prosthesis
- Contracted socket (a more severe deficiency of volume and surface area which makes retention of an ocular prosthesis difficult)
What treatments are there for anophthalmic socket?
Depending on the problem, the following treatment may be prescribed:
- Anti-allergy eyedrops
- Surgery to increase the orbital volume and/or reconstruction of the fornices. Examples: secondary orbital implants or mucous membrane grafting (i.e. dermis fat graft)