Phthisis bulbi is the end-stage condition of the eye that has irreversibly atrophied after severe trauma, infection, chronic inflammation, or other insults. The term originates from the Greek word phthisis (wasting or consumption). It is characterized by softening, shrinkage, calcification, and ossification of the eyeball, with complete loss of visual function.
Ocular atrophy occurs in stages. The following three stages are thought to precede complete phthisis bulbi.
| Stage | Name | Findings |
|---|---|---|
| Stage 1 | Atrophy without shrinkage | Internal structures degenerate but globe shape is preserved |
| Stage 2 | Atrophy with shrinkage | Shrinkage of the globe begins |
| Stage 3 | Phthisis bulbi | Complete softening, shrinkage, calcification, and ossification |
The main causative diseases are listed below. Tripathy et al.’s clinicopathological review shows that three factors—hypotony, breakdown of the blood-ocular barrier, and chronic inflammation—converge into a common final pathology¹.
Phthisis bulbi is an irreversible condition, and the visual function of the atrophied eye cannot be restored. The goal of treatment is pain relief and cosmetic improvement (for prosthetic eye fitting); there is no treatment aimed at restoring vision.
Symptoms of phthisis bulbi are broadly classified into two types based on the presence or absence of pain. The distinction between “painful phthisis bulbi” and “painless phthisis bulbi” is the most important factor in determining the treatment strategy.
External Appearance
Shrinkage and softening of the eyeball: Intraocular pressure is markedly decreased (sometimes unmeasurable), and the eyeball feels distinctly smaller and softer.
Corneal findings: Corneal opacity and vascular invasion (pannus formation) occur, and transparency is lost.
Enophthalmos: The entire orbit appears sunken due to atrophy of orbital fat.
Scleral thinning: The sclera becomes softened and thinned, sometimes appearing bluish.
Intraocular Findings
Anterior segment changes: The anterior chamber disappears, and the iris atrophies and deforms. The lens becomes displaced, opacified, and calcified.
Fundus and intraocular changes: The choroid and retina atrophy and lose function. White calcified plaques appear within the eye.
Ossification findings: In the final stage, heterotopic bone formation (osseous metaplasia) occurs. Intraocular calcification and ossification are confirmed on CT.
Ciliary body changes: The ciliary body becomes fibrotic and completely loses its aqueous humor production function.
There are two types: painful and painless. In painless phthisis bulbi, there is no pain, and the basic approach is cosmetic improvement with a prosthetic eye and follow-up observation. In painful phthisis bulbi, chronic eye pain is the main issue, and if it is resistant to standard analgesics, surgical treatments such as enucleation are considered. Even in the same patient, the condition may transition from painless to painful over time.
Phthisis bulbi is not a single disease but an end-stage condition resulting from various severe eye diseases or injuries.
The diagnosis of phthisis bulbi is based on a combination of medical history and clinical findings. An important differential diagnosis, especially in children, is retinoblastoma.
| Examination method | Purpose | Main findings |
|---|---|---|
| Intraocular pressure measurement | Quantification of low intraocular pressure | Markedly low intraocular pressure (<5 mmHg) or unmeasurable |
| B-mode ultrasound | Evaluation of intraocular structures | Intraocular calcification, presence of retinal detachment, and eyeball size |
| CT | Confirmation of calcification and ossification | Quantification of intraocular calcification (bone formation) and reduced eyeball size |
| MRI | Evaluation of soft tissues | Structural degeneration inside the eyeball and exclusion of tumors |
| Anterior segment examination (slit lamp) | Evaluation of cornea, iris, and lens | Corneal opacity, shallow anterior chamber, iris atrophy |
Congenital microphthalmos is a developmental anomaly in which the eyeball is small from birth, and its etiology differs from phthisis bulbi, which is acquired atrophy of the eyeball. The key point in differentiation is whether there is a history of severe acquired disease or trauma. In phthisis bulbi, atrophic findings such as corneal opacity, calcification, and low intraocular pressure are observed, and advanced degeneration of intraocular structures is confirmed by ultrasound or CT. In congenital microphthalmos, degenerative findings of internal structures are often mild. In addition, when calcification is observed in pediatric phthisis bulbi, differentiation from retinoblastoma is also an important issue.
The treatment strategy for phthisis bulbi is largely divided based on the presence or absence of pain.
For painless phthisis bulbi, observation and cosmetic improvement with a prosthetic eye are the basics. Aggarwal et al. classified 50 cases of phthisis bulbi into 4 categories and proposed guidelines for prosthetic eye use, concluding that cosmetic rehabilitation is the only treatment option⁴.
For painful phthisis bulbi where chronic pain is a problem, the following treatment options are available.
| Treatment | Indication | Features/Precautions |
|---|---|---|
| Enucleation | Standard treatment for painful phthisis bulbi | Most reliable method for pain relief. Orbital implant (e.g., hydroxyapatite, porous polyethylene) is inserted simultaneously. Histopathological examination is possible. |
| Evisceration | Cases desiring scleral preservation | Surgical technique is relatively simple. If there is a history of endophthalmitis, it is generally contraindicated due to the risk of sympathetic ophthalmia |
| Retrobulbar injection (ethanol) | Cases where surgery is difficult due to poor general condition | Symptomatic pain relief. The effect may be temporary, and repeated administration may be necessary |
When phthisis bulbi occurs in childhood, it affects the growth of the orbital bones and eyelids.
Removal is not always necessary. For painless phthisis bulbi, the basic approach is to wear a prosthesis and monitor the condition. On the other hand, for painful phthisis bulbi with persistent chronic pain, enucleation is the most reliable method of pain relief. Removal is also indicated when there is a strong desire for cosmetic improvement or when a malignant tumor is suspected that can only be diagnosed pathologically by enucleation. The decision to remove the eye is made by considering the patient’s wishes and medical necessity together.
Phthisis bulbi is a convergent pathology that leads from various initial events to a common final state.
The histological changes in phthisis bulbi are as follows.
In an eye that has become phthisical, uveal antigens are usually “sequestered,” and the risk of inducing sympathetic ophthalmia in the contralateral eye is low. However, it cannot be completely ruled out. If inflammatory signs (redness, photophobia, floaters, etc.) appear in the contralateral eye during follow-up of a phthisical eye, it is important to promptly consult an ophthalmologist.
If intraocular tissue remains after evisceration, antigenic stimulation may theoretically persist. Therefore, in cases with a history of sympathetic ophthalmia or high risk, enucleation is preferred.
In orbital reconstruction after enucleation, improvements in implant materials are ongoing. Bioceramics (hydroxyapatite, porous polyethylene) promote fibrovascular ingrowth, and good motility due to tissue integration is expected. Research on patient-specific optimized implants using 3D printing technology is also progressing, and it is anticipated that precise custom-made orbital implants tailored to the orbital shape will become possible in the future.
In pediatric phthisis bulbi, impaired orbital bone growth is a concern. Research is underway on expandable implants that can be incrementally upsized during the growth period, attracting attention as a means to promote symmetrical orbital growth. Autologous dermis-fat grafts are also considered useful for maintaining orbital volume and promoting growth in children. A review by Jovanovic et al. reported that primary dermis-fat grafts showed a good eyelid position maintenance rate (83.3%), particularly useful in children, complex orbits, and scarred sockets⁵.
Research on QOL assessment and psychological support for patients with phthisis bulbi or after enucleation is gaining attention. Tools are being developed to comprehensively evaluate changes in appearance, loss of visual function, and impact on social life. Improvements in ocular prosthesis quality (precise iris pattern matching, reproduction of vascular patterns) also contribute to cosmetic QOL enhancement. Establishing psychological support programs for patients and a collaborative system between ocularists and ophthalmologists are important future tasks.
Tripathy K, Chawla R, Temkar S, Sagar P, Kashyap S, Pushker N, Sharma YR. Phthisis Bulbi-a Clinicopathological Perspective. Semin Ophthalmol. 2018;33(6):788-803. PMID: 29902388
Adewara BA, Badmus SA, Olugbade OT, Ezeanosike E, Adegbehingbe BO. Distribution of phthisis bulbi and status of fellow eyes at a tertiary eye-care centre in Nigeria: a ten-year review. Afr Health Sci. 2021;21(1):395-402. PMID: 34394326
Bui K, Tomaiuolo M, Carter K, et al. Sympathetic Ophthalmia in Patients with Enucleation or Evisceration: Pathology Laboratory and IRIS® Registry Experience. Ocul Oncol Pathol. 2023;9(5-6):138-146. PMID: 38089175
Aggarwal H, Singh RD, Kumar P, Gupta SK, Alvi HA. Prosthetic guidelines for ocular rehabilitation in patients with phthisis bulbi: a treatment-based classification system. J Prosthet Dent. 2014;111(6):525-528. PMID: 24423459
Jovanovic N, Carniciu AL, Russell WW, Jarocki A, Kahana A. Reconstruction of the Orbit and Anophthalmic Socket Using the Dermis Fat Graft: A Major Review. Ophthalmic Plast Reconstr Surg. 2020;36(6):529-535. PMID: 32134765
Taha H, Amer HZ, El-Zomor H, et al. Phthisis bulbi: clinical and pathologic findings in retinoblastoma. Fetal Pediatr Pathol. 2015;34(4):223-232. PMID: 25839785
