BNAR syndrome

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ORPHA:217266OMIM:608980Q87.8
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Overview

BNAR syndrome, also known as Blepharophimosis-Nystagmus-Anal atresia-Radial defect syndrome, is an extremely rare genetic condition that affects multiple body systems from birth. The name comes from the combination of its main features: blepharophimosis (narrowing of the eye openings), nystagmus (involuntary eye movements), anal atresia (the anal opening is closed or absent at birth), and radial defects (problems with the radius bone in the forearm and sometimes the thumb). Because this syndrome involves several different parts of the body, affected children may need care from many different specialists starting in the newborn period. Babies born with BNAR syndrome typically need surgical correction of the anal atresia shortly after birth, as this is a life-threatening condition if not treated. The eye abnormalities can affect vision and may require surgical or supportive treatment. The limb abnormalities involving the radius bone and thumb can range from mild to severe and may affect hand function. Because so few cases have been described in the medical literature, the full range of symptoms and the best treatment approaches are still being understood. There is currently no cure for BNAR syndrome, and treatment focuses on managing each individual symptom through surgery, therapy, and supportive care.

Also known as:

Key symptoms:

Narrow eye openings (blepharophimosis)Involuntary eye movements (nystagmus)Absent or closed anal opening at birth (anal atresia)Underdeveloped or missing forearm bone (radial defect)Thumb abnormalities, including absent or underdeveloped thumbVision problemsPossible feeding difficulties in infancyPossible growth delaysLimb length differencesReduced hand grip or hand function

Clinical phenotype terms (7)— hover any for plain English
Bifid noseHP:0011803Short lingual frenulumHP:0000200Anteriorly placed anusHP:0001545Anal stenosisHP:0002025Abnormal fifth toe morphologyHP:0010322Abnormal respiratory system morphologyHP:0012252
Inheritance

Autosomal recessive

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for BNAR syndrome.

View clinical trials →

No actively recruiting trials found for BNAR syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the BNAR syndrome community →

No specialists are currently listed for BNAR 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 BNAR syndrome.

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Community

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Latest news about BNAR syndrome

No recent news articles for BNAR syndrome.

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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.What is the full extent of my child's radial and thumb abnormalities, and what surgeries might be needed?,How will the nystagmus and narrow eye openings affect my child's vision long-term?,What bowel management plan should we follow after the anal atresia repair?,Should we pursue genetic testing, and what might it tell us about recurrence risk for future children?,What therapies (occupational, physical) should we start, and how often?,Are there any other organ systems we should screen for problems?,Can you connect us with other families or support groups for children with similar conditions?

Common questions about BNAR syndrome

What is BNAR syndrome?

BNAR syndrome, also known as Blepharophimosis-Nystagmus-Anal atresia-Radial defect syndrome, is an extremely rare genetic condition that affects multiple body systems from birth. The name comes from the combination of its main features: blepharophimosis (narrowing of the eye openings), nystagmus (involuntary eye movements), anal atresia (the anal opening is closed or absent at birth), and radial defects (problems with the radius bone in the forearm and sometimes the thumb). Because this syndrome involves several different parts of the body, affected children may need care from many different s

How is BNAR syndrome inherited?

BNAR syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does BNAR syndrome typically begin?

Typical onset of BNAR syndrome is neonatal. Age of onset can vary across affected individuals.