Blepharoptosis-myopia-ectopia lentis syndrome

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ORPHA:1259OMIM:110150Q15.8
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Overview

Blepharoptosis-myopia-ectopia lentis syndrome is an extremely rare inherited eye condition that affects several structures of the eye. The name describes its three main features: blepharoptosis (drooping of the upper eyelids), myopia (nearsightedness), and ectopia lentis (displacement of the lens inside the eye from its normal position). These eye problems are present from birth or early childhood and can significantly affect vision if not managed properly. The drooping eyelids can partially block vision, especially if they cover the pupil. Nearsightedness in this condition tends to be moderate to severe, meaning distant objects appear very blurry. The displaced lens can cause additional vision problems, including blurred or double vision, and may increase the risk of other eye complications such as glaucoma or retinal detachment over time. Because this syndrome is so rare, there is limited information about its full range of symptoms and long-term outcomes. Treatment is mainly focused on managing each eye problem individually. This may include glasses or contact lenses for nearsightedness, surgery to correct drooping eyelids (ptosis repair), and careful monitoring or surgical management of the displaced lens. Early diagnosis and regular follow-up with eye specialists are important to preserve vision and prevent complications.

Key symptoms:

Drooping upper eyelids (ptosis)Severe nearsightedness (myopia)Displaced lens in the eyeBlurred visionDifficulty seeing distant objectsPossible double visionReduced visual sharpnessRisk of increased eye pressure (glaucoma)Risk of retinal detachment

Clinical phenotype terms (10)— hover any for plain English
Ectopia lentisHP:0001083Abnormal helix morphologyHP:0011039Palpebral edemaHP:0100540Fingernail dysplasiaHP:0100798
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Blepharoptosis-myopia-ectopia lentis syndrome.

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No actively recruiting trials found for Blepharoptosis-myopia-ectopia lentis syndrome at this time.

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No specialists are currently listed for Blepharoptosis-myopia-ectopia lentis syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 Blepharoptosis-myopia-ectopia lentis syndrome.

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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 the lens displacement, and does it need surgical treatment now?,Will my child need eyelid surgery, and if so, when is the best time?,How often should eye exams be scheduled to monitor for glaucoma or retinal problems?,Are there genetic tests that could help confirm the diagnosis or identify the cause?,What visual aids or school accommodations would you recommend?,Is there a risk that other family members could be affected?,Are there any activities or sports my child should avoid because of the displaced lens?

Common questions about Blepharoptosis-myopia-ectopia lentis syndrome

What is Blepharoptosis-myopia-ectopia lentis syndrome?

Blepharoptosis-myopia-ectopia lentis syndrome is an extremely rare inherited eye condition that affects several structures of the eye. The name describes its three main features: blepharoptosis (drooping of the upper eyelids), myopia (nearsightedness), and ectopia lentis (displacement of the lens inside the eye from its normal position). These eye problems are present from birth or early childhood and can significantly affect vision if not managed properly. The drooping eyelids can partially block vision, especially if they cover the pupil. Nearsightedness in this condition tends to be modera

How is Blepharoptosis-myopia-ectopia lentis syndrome inherited?

Blepharoptosis-myopia-ectopia lentis syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Blepharoptosis-myopia-ectopia lentis syndrome typically begin?

Typical onset of Blepharoptosis-myopia-ectopia lentis syndrome is childhood. Age of onset can vary across affected individuals.