Early-onset lamellar cataract

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ORPHA:441452OMIM:600881H26.8
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Overview

Early-onset lamellar cataract is a type of childhood cataract where a cloudy layer forms in a specific shell-like zone (called a lamella) within the lens of the eye. This condition appears early in life, typically during infancy or early childhood, and can affect one or both eyes. The cloudy area in the lens blocks or scatters light as it enters the eye, which can lead to blurred vision or reduced visual sharpness. If left untreated, it may cause amblyopia (sometimes called 'lazy eye'), where the brain starts to favor one eye over the other, potentially leading to permanent vision problems in the affected eye. Lamellar cataracts are one of the most common forms of congenital or infantile cataracts. The severity can vary widely — some children have only mild cloudiness that does not significantly affect vision, while others have dense opacities that require surgical removal of the lens. Early detection and treatment are very important to allow normal visual development during the critical early years of life. Treatment typically involves cataract surgery to remove the cloudy lens, followed by optical correction with glasses, contact lenses, or in some cases an intraocular lens implant. After surgery, children often need ongoing vision therapy and patching to treat or prevent amblyopia. With timely treatment and follow-up, many children achieve good functional vision, though lifelong eye care is usually needed.

Key symptoms:

Cloudy or white appearance in the pupil of the eyeBlurred or reduced visionDifficulty seeing clearly at a distance or up closeEyes that do not align properly (strabismus or crossed eyes)Lazy eye (amblyopia)Sensitivity to bright lightNystagmus (involuntary eye movements)Difficulty tracking objects with the eyesSquinting or closing one eyeHolding objects very close to the faceDelayed visual development milestones

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Early-onset lamellar cataract.

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No actively recruiting trials found for Early-onset lamellar cataract at this time.

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No specialists are currently listed for Early-onset lamellar cataract.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Early-onset lamellar cataract.

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Community

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Caregiver Resources

NORD Caregiver Resources

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Mental Health Support

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Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is my child's cataract, and is surgery needed now or can we monitor it?,What are the risks and benefits of cataract surgery at my child's current age?,Will my child need an intraocular lens implant, or will they use glasses or contact lenses after surgery?,How often will my child need follow-up eye exams after treatment?,What is the risk of developing glaucoma or other complications after surgery?,Should we pursue genetic testing to understand the cause and implications for future children?,What can I do at home to support my child's visual development and amblyopia treatment?

Common questions about Early-onset lamellar cataract

What is Early-onset lamellar cataract?

Early-onset lamellar cataract is a type of childhood cataract where a cloudy layer forms in a specific shell-like zone (called a lamella) within the lens of the eye. This condition appears early in life, typically during infancy or early childhood, and can affect one or both eyes. The cloudy area in the lens blocks or scatters light as it enters the eye, which can lead to blurred vision or reduced visual sharpness. If left untreated, it may cause amblyopia (sometimes called 'lazy eye'), where the brain starts to favor one eye over the other, potentially leading to permanent vision problems in

At what age does Early-onset lamellar cataract typically begin?

Typical onset of Early-onset lamellar cataract is infantile. Age of onset can vary across affected individuals.