65 year old man who had CT scan for stoke evaluation
Right sided hemiplegia
Small, partially calcified bulb, left eye.
Phthisis bulbi defines the end stage appearance of the globe after any multitude of insults (i.e. injury or infection). The intraocular structures are not easily identifiable, by physical exam or with imaging, and there is often calcification within the eye. The globe is small, shrunken, and anatomically disfigured. In clinical terminology, a phthisis eye represents a shrunken globe, usually from ceased aqueous humor formation. Some ophthalmic pathologists further restrict the term phthisis to shrunken eyes that also show generalized disorganization of the intraocular contents. In phthisis bulbi, calcium may be deposited within keratopathy, lens, metaplasia, retinopathy of prematurity, sclera, retina, or optic nerve.
Phthisis bulbi has varied etiologies, from ocular injury, radiation, infection, diffuse inflammation, or autoimmune disease (Behcet's Disease). Initial damage to intraocular structures either from penetrating trauma or from inflammation can eventually lead to widespread atrophy and disorganization of the eye. In the case of retinoblastoma, phthisis bulbi can occur as the result of tumor necrosis.
In a review of retinoblastoma patients by Mullaney et al, they found that 2.7% of patients with retinoblastoma had concomitant phthisis bulbi. There are also reports of phthisical eyes found to have malignant tumors such as sarcoma and melanoma. This is important to consider, as the treatment for phthisis bulbi is often enucleation followed by placement of an ocular prosthesis.
Phthisis bulbi patients are blind and it was previously thought this blindness was permanent. A case report from Dohlman and D'Amico describes a successful vision recovery surgery, using keratoprosthesis, on a phthisis eye. They describe the only known case of a patient with a severely opaque cornea and phthisis eye who had substantial vision restored.
Enophthalmos is posterior displacement of the eye. The anterior projection of the eye is usually measured relative to the outer edge of the orbit, the orbital rim, but it also may be assessed relative to the frontal and maxillary prominences, or the contralateral eye. Secondary enophthalmos, as in this case, is due to an acquired change in the volumetric relationship between the rigid bone cavity, the orbit, and its contents (predominantly the orbital fat and the eye). Expansion of the orbital cavity without change in the volume of the orbital contents leads to enophthalmos. Alternatively, scarring contracture of the orbital fat and extraocular muscles may decrease soft tissue volume, making the orbital cavity less full and causing enophthalmos.
Microphthalmos refers to acquired diminution or congenital underdevelopment in the size of the globe. Microphthalmos refers to an eye that has an axial length <21 mm in adults or <19 mm 1-year-old. Microphthalmos in adults can occur as result of similar processes that lead to phthisis bulbi such as trauma, surgery, inflammation, radiation or infection.