Doyne Honeycomb Macular Dystrophy

Background and History: 

Robert Walter Doyne, an ophthalmologist in England, first described this disorder in 1899.  It is sometimes called Malattia Leventinese after it was observed among families living in the Leventine valley in southern Switzerland.

Clinical Correlations: 

This rare disorder is limited to the retina of the eye where round white lesions appear in a radial fashion around the macula, an area we use for our fine vision since it has a dense concentration of cells that respond to light and transmit images to the brain.  In the early stages, vision is minimally impacted but with time slow vision loss takes place.  Later, significant vision loss is present.  Sometimes, new blood vessels may appear in the area and when these hemorrhage, sudden vision loss occurs.  This may result in scarring which leads to severe, permanent vision loss.

Genetics: 

This is an autosomal dominant disorder in most cases, meaning that the inheritance pattern is vertical as parents can directly transmit the disease to their children.

Diagnosis and Prognosis: 

No treatment is available for the genetic disease but low vision aids are often helpful in restoring near normal visual function for many years.

Additional Information
Inheritance/Pedigree: 
Autosomal dominant