Phthisis bulbi

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Disease Entity

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Disease

Phthisis bulbi is a shrunken, non-functional eye that represents an ocular end-stage ocular disease of various causes and is characterized by atrophy, shrinkage, and disorganization of the globe and intraocular contents.[1] This condition is related to a variety of causes that lead to scarring, inflammation, and globe disorganization. Common causes include trauma, surgery, infection, inflammation, malignancy, retinal detachment, and vascular lesions.[2] A phthisical globe presents with a small squared off shape, opaque and thickened cornea, thickened sclera, neovascularization of iris, cataract, cyclitic membrane, ciliochoroidal detachment, and retinal detachment.[2] A mnemonic used to describe phthisis bulbi is '' 7 S ' referring to :

  • soft,
  • shrunken
  • shapeless
  • sightless
  • structureless,
  • squared
  • sore

Etiology

Phthisis bulbi is an end stage ocular disease which can result from a variety of ocular insults including:

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

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 – has been further modified by Yanoff and Fine ( Table 54.1 )

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

Primary prevention

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Diagnosis

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History

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Physical examination

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Signs

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Symptoms

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Clinical diagnosis

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Diagnostic procedures

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Laboratory test

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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

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General treatment

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Medical therapy

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Medical follow up

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Surgery

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Surgical follow up

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Complications

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Prognosis

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Additional Resources

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References

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  1. Themes, U. (2019, August 26). Phthisis BULBI. Retrieved April 25, 2021, from https://entokey.com/phthisis-bulbi/
  2. 2.0 2.1 2.2 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. doi: 10.1080/08820538.2018.1477966. Epub 2018 Jun 14. PMID: 29902388.
  3. Gaillard, F. (n.d.). Phthisis bulbi: Radiology reference article. Retrieved April 26, 2021, from https://radiopaedia.org/articles/phthisis-bulbi