Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome

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ORPHA:73246Q87.8
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Overview

Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome (sometimes called VBFDD syndrome) is a very rare genetic condition that affects multiple parts of the body at the same time. The name describes its main features: problems with the nerves that control the internal organs (visceral neuropathy), structural differences in the brain, distinctive facial features, and delays in reaching developmental milestones like talking and walking. The condition affects the nervous system in two important ways. First, the nerves that control the digestive system and other internal organs do not work properly, which can cause serious gut problems such as severe constipation, bloating, and difficulty moving food through the intestines. Second, the brain itself may be formed differently, which contributes to intellectual disability and developmental delays. Children with this syndrome often have a characteristic appearance to their face, though this varies from person to person. Because this syndrome is so rare, treatment focuses on managing symptoms rather than curing the underlying cause. Care usually involves a team of specialists working together to support the child's development, manage digestive problems, and address any neurological needs. Early intervention with therapies such as speech, occupational, and physical therapy can make a meaningful difference in quality of life.

Key symptoms:

Severe constipation or inability to pass stool normallyBloating and abdominal pain due to poor gut movementDifficulty feeding in infancyIntellectual disability or learning difficultiesDelayed speech and language developmentDelayed motor milestones such as sitting, standing, or walkingDistinctive facial features such as widely spaced eyes or a flat nasal bridgeStructural differences in the brain seen on imagingWeak muscle tone (hypotonia)Seizures in some individualsPoor weight gain or failure to thrive in infancy

Clinical phenotype terms (25)— hover any for plain English
Intestinal pseudo-obstructionHP:0004389Fetal megacystisHP:0010956
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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome.

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No actively recruiting trials found for Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome at this time.

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No specialists are currently listed for Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay 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 Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

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Family & Caregiver Grants

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene caused this condition in my child, and what does that mean for other family members?,What is the best way to manage my child's bowel problems, and when would surgery be considered?,What therapies should my child start right away, and how often should they attend?,Are there any clinical trials or research studies we could participate in?,What signs should prompt me to go to the emergency room?,What educational support and services should I be requesting for my child?,Are there other families or support groups who have experience with this condition?

Common questions about Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome

What is Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome?

Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome (sometimes called VBFDD syndrome) is a very rare genetic condition that affects multiple parts of the body at the same time. The name describes its main features: problems with the nerves that control the internal organs (visceral neuropathy), structural differences in the brain, distinctive facial features, and delays in reaching developmental milestones like talking and walking. The condition affects the nervous system in two important ways. First, the nerves that control the digestive system and other inter

How is Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome inherited?

Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome typically begin?

Typical onset of Visceral neuropathy-brain anomalies-facial dysmorphism-developmental delay syndrome is neonatal. Age of onset can vary across affected individuals.