Phthisis bulbi oculi

Phthisis bulbi, also known as end-stage eye, is an atrophic scarred and disorganized globe that may result from a variety of severe ocular insults.

Epidemiology

In general, phthisis bulbi involves elderly patients, usually 65-85 years of age . Children and adolescents are only rarely affected, mainly due to ocular trauma and congenital malformations .

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 .

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 :

• small shrunken, deformed, calcified globe with enophthalmos
• abnormal intraocular contents deranged

Signal characteristics :

• 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.

See also

• ocular pathology

Siehe auch:

• Bulbus-oculi-Verkalkungen
• Verkalkungen Auge
• Retinoblastom
• Pathologien des Auges
• persistierender hyperplastischer primärer Glaskörper (PHPV)

und weiter:

• Leukokorie
• Sonographie des Auges