Phthisis bulbi, also known as end-stage eye, is an atrophic scarred and disorganized non-functioning globe that may result from a variety of severe ocular insults.
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Epidemiology
In general, phthisis bulbi involves elderly patients, usually 65-85 years of age 7. Children and adolescents are only rarely affected, mainly due to ocular trauma and congenital malformations 7.
Clinical presentation
Typical clinical symptoms and signs include chronic ocular hypotension, a shrunken globe, pseudoenophthalmos, intraocular tissue fibrosis and scarring, visual loss, recurrent episodes of intraocular irritation, and pain, and swelling in and around the eye 7,8.
Pathology
The globe is reduced in size (usually <20 mm) with a thickened/folded posterior sclera. Dystrophic calcification is common, and osseous metaplasia sometimes occurs, forming what is called "intraocular bone".
Etiology
Causes of this end-stage damage include:
- trauma (causes ciliochoroidal effusion and cyclitic membrane)
- infection
- other inflammatory processes
- radiation
- chronic retinal detachment
- persistent hyperplastic primary vitreous
- retinoblastoma
Radiographic features
CT
- small and shrunken globe with foci of calcium deposits and ossification in the sclera, cornea, lens, retina, and optic nerve
- distortion of globe components with challenging to separate and identify structures
- fibrotic scarring with irregular globe contour and diffusely increased attenuation
MRI
General features ref:
- small shrunken, deformed, calcified globe with enophthalmos
- abnormal intraocular contents deranged
Signal characteristics ref:
- T1: often with heterogeneous areas of increased signal, depending on the degree of calcification and hemorrhage
- T2: mixed vitreous dark filling defects due to coarse calcifications
- FLAIR: usually with increased signal
Treatment and prognosis
The globe is non-functioning, thus the patient is blind in that eye. Enucleation +/- prosthesis insertion is performed if there is associated chronic pain or for cosmetic reasons.
Differential diagnosis
The differential includes other causes of calcification of the globe.
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