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Cornea: endothelial dystrophy
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.
Presenting signs
- Blue-white appearance of eyes (corneal edema).
- Visual deficits.
- Initially non-painful but, as disease progresses, painful superficial corneal ulcers can develop.
Acute presentation
- Focal corneal edema, usually located temporally
.
Age predisposition
- Variable.
- Dogs as early as 2 up to dogs of 13 years of age but predominantly older.
Gender predisposition
- Females may be overrepresented.
Breed/Species predisposition
- Boston terrier Boston Terrier (age range 5-9 years, mean age 7.5 years).
- Chihuahua Chihuahua - Smooth Coat (age range 6-13 years, mean age 9.5 years).
- Dachshund Dachshund.
- Boxer Boxer.
- Miniature Schnauzer Schnauzer: miniature.
- Wire-haired Fox Terrier Fox Terrier - Wire.
- Basset Hound Basset Hound.
- Miniature Poodle Poodle: miniature.
Cost considerations
- Symptomatic treatment with topical hyperosmotic agents is moderately expensive.
- Recurrent corneal ulceration occurs with severe and progressive disease and may require repeated minor surgical treatments.
Special risks
- Recurrent corneal ulceration may require surgical intervention and a general anesthesia be therefore required.
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.