Overview
Kandori fleck retina (also known as Kandori's fleck retina or fleck retina of Kandori) is a rare inherited retinal dystrophy first described by the Japanese ophthalmologist Fumio Kandori in 1959. It is characterized by the presence of large, irregular, yellowish-white flecks located predominantly in the midperipheral and peripheral regions of the retina, typically sparing the macula. These flecks are thought to represent abnormal deposits at the level of the retinal pigment epithelium (RPE). The condition primarily affects the eyes and is generally considered a stationary or very slowly progressive disorder. Most affected individuals maintain good central visual acuity throughout life, though some patients may experience mild difficulties with dark adaptation (night vision disturbances or nyctalopia). Electroretinography (ERG) findings are typically normal or show only mild abnormalities. The fundoscopic appearance of the flecks is distinctive and helps differentiate this condition from other flecked retina syndromes such as fundus flavimaculatus (Stargardt disease), fundus albipunctatus, or familial drusen. There is currently no specific treatment for Kandori fleck retina. Management is primarily supportive, involving regular ophthalmologic monitoring to track any changes in visual function or retinal appearance. Given the generally benign course of the disease, the visual prognosis is favorable for most patients. Genetic counseling may be offered to affected families. The condition remains extremely rare, with only a limited number of cases reported in the medical literature, predominantly from Japan.
Autosomal recessive
Passed on when both parents carry the same gene change; often skips generations
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Kandori fleck retina.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Kandori fleck retina.
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Common questions about Kandori fleck retina
What is Kandori fleck retina?
Kandori fleck retina (also known as Kandori's fleck retina or fleck retina of Kandori) is a rare inherited retinal dystrophy first described by the Japanese ophthalmologist Fumio Kandori in 1959. It is characterized by the presence of large, irregular, yellowish-white flecks located predominantly in the midperipheral and peripheral regions of the retina, typically sparing the macula. These flecks are thought to represent abnormal deposits at the level of the retinal pigment epithelium (RPE). The condition primarily affects the eyes and is generally considered a stationary or very slowly progr
How is Kandori fleck retina inherited?
Kandori fleck retina follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.