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Phthisis bulbi..elias t
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- 2. OUTLINE • Definition • Causes •Grading • Pathophysiology • Diagnosis • Management
- 3. 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.
- 4. CAUSES • Trauma • Infection •Inflammations • Surgery • RD • Malignancy • Vascular causes
- 5. GRADING Yanoff and Fineproposed 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
- 6. ATROPHIA BULBI WITHOUT SHRINKAGE. •The size and shape of the eye are maintained despite the atrophy of intraocular tissues. • The following structures are most sensitive to loss of nutrition • The lens, which becomes cataractous; • The retina,which atrophies and becomes separated from RPE by serous fluid accumulation; • The aqueous outflow tract, where anterior and posterior synechiae develop
- 7. ATROPHIA BULBI WITH SHRINKAGE •The 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
- 8. PHTHISIS BULBI • Thesize 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
- 10. PATHOPHYSIOLOGY • The majorfactors 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
- 11. DIAGNOSIS Physical examination • Phthisicaleyes can usually be easily detected by inspection of the patient’s face • Diagnosis is simplified due to the unilaterality of the disease • With asymmetry of the eyeballs and interpalpebral fissures.
- 12. 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
- 13. SYMPTOMS • vision loss •blurry vision • floaters • photophobia • ocular pain • redness • periorbital edema • visual loss
- 14. MANAGEMENT • Treatment approachfor a phthisic eye is often futile, • Mainly aims at alleviating ocular pain and at cosmetic rehabilitation of the affected eye • Not 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.
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Editor's Notes
- #4 Phthisis bulbi is defined as atrophy, shrinkage, and disorganization of the eye and intraocular contents. Not all eyes rendered sightless by trauma become phthisical. If the nutritional status of the eye and near-normal intraocular pressure (IOP) are maintained during the repair process, the globe will remain clinically stable. However, blind eyes are at high risk for repeated trauma, with cumulative destructive effects. Slow, progressive functional decompensation may also prevail
- #5 Etiology Phthisis bulbi is an end stage ocular disease which can result from a variety of ocular insults including. Common causes include trauma, surgery, infection, inflammation, malignancy, retinal detachment, and vascular lesion[2] Traumatic Open globe injury Closed globe injury Chemical injury —alkali burn Radiation Infections Keratitis Endophthalmitis Panophthalmitis Chronic retinal detachment Postoperative Anterior segment surgeries (cataract surgery via anterior route and lensectomy, glaucoma surgery, corneal surgeries) Posterior segment surgeries (vitrectomy for endophthalmitis, retained intraocular foreign bodies, suprachoroidal hemorrhage, silicone oil removal, vitreoretinal surgery for retinal detachment with complex proliferative vitreoretinopathy, after penetrating trauma, combined rhegmatogenous, & tractional retinal detachment in diabetics, retinectomy for intractable glaucoma pneumatic retinopexy, suprachoroidal hemorrhage drainage) Inflammation Intermediate uveitis is the most common inflammatory cause of end-stage eye disease. In the advanced stages of intermediate uveitis, cyclitic membranes may form leading to hypotony and phthisis. JIA & Childhood-onset Behçet disease Sympathetic ophthalmia Vogt-Koyanagi-Harada syndrome Autosomal dominant neovascular inflammatory vitreoretinopathy. Vascular disorders Ocular ischemic syndrome Coat's disease Retinopathy of prematurity (ROP) Proliferative diabetic retinopathy (PDR) Retinal capillary hemangioblastoma Congenital Causes Persistent hyperplastic primary vitreous Peters’ anomaly type 2 Norrie disease Familial exudative vitreoretinopathy Incontinentia pigmenti Osteoporosis-pseudoglioma syndrome (OPPG) Tumors Retinoblastoma Choroidal melanoma Rare tumors including adenoma of the nonpigmented ciliary epithelium, double pseudo-neoplastic proliferation of the RPE, primary retinal glial tumor with features of myxopapillary ependymoma, osteogenic sarcoma, concurrent benign teratoid medulloepithelioma and pineoblastoma, and conjunctival spindle cell carcinoma. Medications Cidofovir Miscellaneous Parry–Romberg syndrome Uveal effusion syndrome Sickle cell disease Neurofibromatosis
- #6 STAGES OF OCULAR DEGENERATION Many blind eyes pass through several stages of atrophy and disorganization before progressing to the end stage of phthisis bulbi: • Atrophia bulbi without shrinkage. In this initial stage, the size and shape of the eye are maintained despite the atrophy of intraocular tissues. The following structures are most sensitive to loss of nutrition: the lens, which becomes cataractous; the retina, which atrophies and becomes separated from the retinal pigment epithelium (RPE) by serous fluid accumulation; and the aqueous outflow tract, where anterior and posterior synechiae develop. A B C Figure 1-10 Types of multinucleated giant cells. A, Langhans (circle). Note the peripheral arrangement of nuclei. B, Touton giant cell (circle). Note the central eosinophilic cytoplasm and annulus of nuclei surrounded by a foamy, lipid-filled pale outer ring. C, Foreign body giant cell (circle). Note the haphazardly arranged nuclei. CHAPTER 1: Introduction to Part I ● 15 C A B * * Figure 1-11 Phthisis bulbi. A, Gross photograph of a whole globe. Note the squared-off shape of the globe (arrow), resulting from hypotony and the force of the 4 rectus muscles on the sclera. B, Gross photograph of a phthisical globe that has been opened. Note the irregular contour, cataractous lens with calcification (asterisk), cyclitic membrane with adherent retina (arrowheads), and bone formation (between green arrows). C, Photomicrograph demonstrating the histopathologic correlation with the gross photograph shown in part B. In addition, organized ciliochoroidal effusions are apparent histologically (blue arrows). (Part A courtesy of Ralph C. Eagle, MD; parts B and C courtesy of Robert H. Rosa Jr, MD.) • Atrophia bulbi with shrinkage. In this stage, the 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 rectus muscles. 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 (Fig 1-11). In this end stage, the size of the globe shrinks from a normal average diameter of 23–25 mm to an average diameter 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
- #10 Figure 1-11 Phthisis bulbi. A, Gross photograph of a whole globe. Note the squared-off shape of the globe (arrow), resulting from hypotony and the force of the 4 rectus muscles on the sclera. B, Gross photograph of a phthisical globe that has been opened. Note the irregular contour, cataractous lens with calcification (asterisk), cyclitic membrane with adherent retina (arrowheads), and bone formation (between green arrows). C, Photomicrograph demonstrating the histopathologic correlation with the gross photograph shown in part B. In addition, organized ciliochoroidal effusions are apparent histologically (blue arrows).
- #11 History The term phthisis bulbi derives from the Greek word phthiein or phthinein , meaning shrinkage or consuming, and was first used by Galen. Over the last 200 years, the clinical interpretation of phthisis bulbi has often been modified according to the underlying disease and structural changes; a clear distinction from ocular atrophy was often difficult and controversial. Hogan and Zimmerman were the first ones who stated that both terms – atrophy and phthisis bulbi – refer to consecutive stages in the degeneration process of a severely damaged eye. Their descriptive classification system including three different stages – (1) ocular atrophy without shrinkage; (2) with shrinkage; and (3) with shrinkage and disorganization. General Pathology Microscopic features include internal disorganization, inflammatory reaction, a reactive proliferation of various cells, calcification, and ossification.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".[3] Pathophysiology The major factors associated with the pathogenesis of phthisis are ocular hypotony, deranged blood-ocular barriers, and intraocular inflammation.[2] Hypotony causes alteration of oxygenation, nutrition and metabolism, while the breakdown of the ocular blood barrier of the plasma proteins and release of cytokines, chemotactic & angiogenetic factors
- #15 Differential diagnosis Although the underlying diseases and the clinical course of phthisis bulbi are quite variable, the end-stage disease is rarely missed because of characteristic clinical features (i.e., small, soft, atrophic eyes), which are often associated with decreased or lost vision. However, clinicians should be aware of any potential disease entity which, if not treated properly, may result in a blind, often painful phthisical eye. Intraocular malignancies (i.e., retinoblastoma, malignant uveal melanoma) should be taken into consideration if the ocular history is limited and an obvious cause for phthisis is missing. In addition, congenital abnormalities like microphthalmos and microcornea should be kept in the differential diagnosis of phthisis bulbi. Management General treatment Treatment approach for a phthisic eye is often futile, and mainly aims at alleviating ocular pain and at cosmetic rehabilitation of the affected eye, not 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.