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Coats syndrome
Coats syndrome is a rare eye disorder marked by abnormal retinal blood vessels that leak fluid, causing vision loss, retinal detachment, and sometimes blindness, usually affecting one eye in children or young adults
Prevalence
> 1 / 100 000
300–400
US Estimated
400–540
Europe Estimated
Age of Onset
Childhood
ICD-10
H35.02
Inheritance Pattern
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
Rare View
Coats' disease is a rare, non-hereditary eye disorder characterized by abnormal development of the blood vessels in the retina, leading to fluid leakage and potential vision loss. It predominantly affects young males and typically presents unilaterally.
5 Facts you should know
FACT
Most people begin showing symptoms in childhood
FACT
Early signs and symptoms vary but may include vision loss, "crossed eyes" (strabismus), and a white mass in the pupil behind the lens of the eye (leukocoria)
FACT
Over time, Coats disease may also lead to retinal detachment, glaucoma, and clouding of the lens of the eye (cataracts)
FACT
In most cases, only one eye is affected
FACT
The irregular caliber of telangiectatic vessels, aneurysms, subretinal exudates, and lack of calcification on ultrasound differentiates it from retinoblastoma
Interest over time
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Common signs & symptoms
Decreased vision, strabismus, leukocoria (advanced), visual field defects
Fundus: retinal telangiectasia (“light-bulb” aneurysms), hard exudates, subretinal fluid/exudative detachment
Key diagnostic step: exclude retinoblastoma (imaging + exam)
Current treatments
Mainstays: laser photocoagulation and/or cryotherapy to ablate telangiectatic vessels; approach depends on stage and detachment extent.
Adjuncts in selected cases: intravitreal steroids or anti-VEGF (pediatric safety considerations), vitreoretinal surgery for complex detachments
End-stage/painful blind eye or diagnostic uncertainty: enucleation may be considered
Top Clinical Trials
| Title | Description | Phases | Status | Interventions | More Information |
|---|---|---|---|---|---|
| Safety and Efficacy of 18 mm Short Vitrectomy Probe for Pediatric Vitreoretinal Surgeries | This study aims to study the safety and efficacy of using an 18 mm short vitrectomy probe for pediatric vitreoretinal surgeries and to investigate the surgeon's comfort and reliability of using the shorter probe. | Phase 4 | Recruiting | Device: Pars plana vitrectomy with a 18 mm short vitrector | More Info |
| Cardiovascular Risk in Children With Chronic Conditions Study | Children living with chronic health conditions face a higher risk of developing cardiovascular diseases than their peers, largely due to the accelerated aging of the heart and blood vessels. Although experts recognize this elevated risk and recommend close monitoring and early intervention, the unde... | Recruiting | More Info |