Can an eye in phthisis be rehabilitated? A case of improved vision with 1-year follow-up.

P hthisis bulbi implies, in clinical terminology, a shrunken globe, usually from ceased aqueous humor formation (phthisis meaning “wasting away”). The intraocular pressure approaches 0 mm Hg. As a consequence, the cornea becomes distorted and can develop edema and scarring, and the lens develops cataracts. Both seem to occur due to the lack of nutrition by the aqueous. In addition, edema can develop in the macula and the optic nerve head and vision suffers accordingly. Finally, cyclitic membranes and proliferative vitreal retinopathy can develop, resulting in total retinal detachment and scar formation. The cause of phthisis is often uveitis, either long-term or following trauma, surgery, or endstage, heavily treated glaucoma. Treatment of phthisis is generally considered hopeless. Aggressive treatmentof intraocularinflammation in uveitis or avoiding too much cyclodestructioninglaucomaduringthe early stage with hypotony may delay or prevent the final collapse. In some rare instances of chronic cyclodialysisorseverecycliticmembranes,asurgical approach has been reported to beofsomevalue.Ingeneral,however, if the intraocular pressure is close to 0, the eye is collapsed, the cornea is edematous, thelensiscataractous, the ocularvolumeisreducedtohalforone third of the normal volume, and the ocular layers are correspondingly thickened, no treatment has, to our knowledge, been effective in restoring vision. Any proposed treatment of phthisis must be surgical, if for no other reason than to restore the clarity of the cornea. In this respect, standard corneal transplantation is ineffective, since the source of nutrition, a healthy flow of aqueous, is absent. With the advent of more advanced techniques for keratoprosthesis surgery and more long-term, postoperative stability, there may be new hope for eyes going into phthisis. Initial attempts in this direction have been made in the past, but there was no success in restoring vision. In this report, we describe a patient with a chemical burn that led to a shrunken eye, no palpable intraocular pressure, an opaque cornea (due in part to the direct effect of the alkali), and an attached retina, who regained substantial vision following keratoprosthesis surgery and was followed up for 1 year.