Definition and introduction Phthisis bulbi is defined as atrophy, shrinkage, and disorganization of the eye and intraocular contents Clinical condition representing end-stage ocular response to severe eye injury or disease damage Not all eyes rendered sightless by trauma become phthisical A mnemonic rule used to describe phthisis bulbi is ''7S" referring to : Soft - Shrunken - Shapeless - Sightless - Structureless - Squared - Sore.
Causes Trauma Infection Inflammations Surgery RD Malignancy Vascular causes
Grading Yanoff and Fine proposed the following descriptive grading system based on clinicopathological features: Grade I : Atrophy bulbi without shrinkage Grade II : Atrophia bulbi with shrinkage Grade III : Atrophia bulbi with shrinkage and disorganization (phthisis bulbi) Grade IV : Phthisis bulbi with intraocular ossification Grade V : Phthisis bulbi with intraocular calcium deposition
Atrophia bulbi without shrinkage. T he size and shape of the eye are maintained despite the atrophy of intraocular tissues. The following structures are most sensitive to loss of nutrition T he lens, which becomes cataractous; T he retina,which atrophies and becomes separated from RPE by serous fluid accumulation; T he aqueous outflow tract, where anterior and posterior synechiae develop
Atrophia bulbi with shrinkage T he eye becomes soft because of ciliary body dysfunction and progressive reduction of IOP. The globe becomes smaller and assumes a squared-off configuration as a result of the influence of the 4 EOM The anterior chamber collapses. Associated corneal endothelial cell damage initially results in corneal edema, followed by opacification with degenerative pannus, stromal scarring, and vascularization. Most of the remaining internal structures of the eye will be atrophic but recognizable histologically
Phthisis bulbi T he size of the globe shrinks from normal average diam of 23–25 mm to an average diam of 16–19 mm. Most of the ocular contents become disorganized. In areas of preserved uvea the RPE proliferates, and nodular drusen may develop. In addition, extensive dystrophic calcification of the Bowman layer, lens, retina, and drusen usually occurs. Osseous metaplasia of the RPE with bone formation may be a prominent feature. Finally, the sclera becomes markedly thickened, particularly posteriorly
Pathophysiology The major factors associated with the pathogenesis of phthisis are ocular hypotony, deranged blood-ocular barriers, and intraocular inflammation. Hypotony causes alteration of oxygenation, nutrition and metabolism, Breakdown of the ocular blood barrier of the plasma proteins and release of cytokines, chemotactic & angiogenetic factors
Diagnosis Physical examination Phthisical eyes can usually be easily detected by inspection of the patient’s face D iagnosis is simplified due to the unilaterality of the disease W ith asymmetry of the eyeballs and interpalpebral fissures .
Signs Microphthalmos Enophthalmos Lagophthalmos Reduced eyelid fissure Strabismus Conjunctival chemosis and hyperemia Corneoscleral scarring, thickening, vascularization, dystrophic calcification and shrinkage Flattening of the anterior chamber with a narrow to closed chamber angle Hypotony Neovascularization of the iris and chamber angle (rubeosis iridis) Intraocular inflammation (uveitis/endophthalmitis) Synechia (peripheral/posterior) Cyclitic/epiretinal membranes (fibrous/fibrovascular) Cataract formation Phacodonesis (anterior displacement of the ciliary body) Choroidal or/and retinal detachment Epiretinal membranes Choroidal/ciliary body, retinal, and optic nerve degeneration/atrophy Intraocular hemorrhages Dystrophic calcification and heterotopic ossification
Symptoms vision loss blurry vision floaters photophobia ocular pain redness periorbital edema visual loss
Management Treatment approach for a phthisic eye is often futile, M ainly aims at alleviating ocular pain and at cosmetic rehabilitation of the affected eye N ot to restore vision. It can be removed, a procedure called enucleation of the eye. Sometimes, though, it is possible to transplant only parts of the eye, and some vision can be restored.