ISSN 2398-2969      

Cornea: endothelial dystrophy

icanis

Synonym(s): Corneal endothelial degeneration; Bullous keratopathy


Introduction

  • Cause: inherited or degenerative (mechanism unknown) abnormality of corneal endothelial cells.
  • Signs: spontaneous, progressive corneal edema.
  • Diagnosis: examination, signalment.
  • Treatment: topical hyperosmotics, palliative thermokeratoplasty, corneal grafting.
  • Prognosis: guarded to poor for vision.

Pathogenesis

Etiology

  • May be a similar disease to Fuchs dystrophy in man.
  • Fuchs dystrophy inherited as an autosomal dominant trait with incomplete penetrance.

Predisposing factors

General
  • Sex (female).
  • Breed (Boston Terrier, Chihuahua, Dachshund, Boxer, Miniature Poodle, Miniature Schnauzer, Basset Hound).

Pathophysiology

  • There is bilateral, progressive degeneration of the corneal endothelial cells.
  • There is a reduction in the number of cells and the endothelium demonstrates fibrous metaplasia.
  • Leads to progressive corneal edema.

Timecourse

  • The initial focal lesion of corneal edema progresses slowly over months to years to involve the entire cornea.
  • Both eyes are typically affected, often initially asymmetrically.

Epidemiology

  • Relates to breed and age predisposition and distribution in a given population.

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Michau T M, Gilger B C, Maggio F et al (2003) Use of thermokeratoplasty for treatment of ulcerative keratitis and bullous keratopathy secondary to corneal endothelial disease in dogs: 13 cases (1994-2001). JAVMA 222, 607-612 PubMed.
  • Bergmanson J P, Sheldon T M & Goosey J D (1999) Fuchs' endothelial dystrophy: a fresh look at an aging disease. Ophthalmic and Physiologic Optics 19 (3), 210-222 PubMed.
  • Adamis A P, Filatov V, Tripathi B J et al (1993) Fuchs' endothelial dystrophy of the cornea. Survey of Ophthalmology 38 (2), 149-168 PubMed.
  • Gwin R, Polack F, Warren J et al (1982) Primary canine corneal endothelial dystrophy: specular microscopic evaluation, diagnosis and therapy. JAAHA 18 (3), 471-479 VetMedResource.
  • Martin C & Dice P (1982) Corneal endothelial dystrophy in the dog. JAAHA 18 (2), 327-336 VetMedResource.

Other sources of information

  • Ledbetter E C & Gilger B C (2013)diseases and Surgery of the Cornea and Sclera.In:Veterinary Ophthalmology(5th edition. Ed Gelatt KN, Gilger BC & Kern TJ) pp 976-1049.

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