Familial omphalocele syndrome with facial dysmorphism

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Overview

Familial omphalocele syndrome with facial dysmorphism is an extremely rare genetic condition that is present at birth. The hallmark feature is an omphalocele, which is a birth defect where the baby's intestines or other abdominal organs stick out through the belly button area, covered by a thin membrane. In this particular syndrome, the omphalocele occurs alongside distinctive facial features (facial dysmorphism), which may include unusual spacing of the eyes, a broad or flat nasal bridge, low-set ears, or other subtle differences in facial structure. Because this condition runs in families, it is considered a familial (inherited) form rather than an isolated birth defect. The condition is typically identified at birth or even before birth through prenatal ultrasound. The omphalocele requires surgical repair, usually shortly after delivery. The facial features may become more or less noticeable as the child grows. Because this syndrome is so rare, there is limited published medical literature, and the full range of possible symptoms and long-term outcomes is not completely understood. Management involves a team of specialists who address the abdominal wall defect, monitor growth and development, and evaluate for any additional associated abnormalities. Genetic counseling is strongly recommended for affected families to understand the inheritance pattern and recurrence risk in future pregnancies.

Key symptoms:

Abdominal organs protruding through the belly button area at birth (omphalocele)Unusual facial featuresWide-set or closely-set eyesBroad or flat nasal bridgeLow-set earsSmall jawPossible feeding difficulties in infancyPossible developmental delaysPossible additional abdominal wall abnormalities

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Familial omphalocele syndrome with facial dysmorphism.

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No actively recruiting trials found for Familial omphalocele syndrome with facial dysmorphism at this time.

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No specialists are currently listed for Familial omphalocele syndrome with facial dysmorphism.

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 Familial omphalocele syndrome with facial dysmorphism.

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Community

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Latest news about Familial omphalocele syndrome with facial dysmorphism

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Caregiver Resources

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Family & Caregiver Grants

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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 size and severity of my child's omphalocele, and what surgical approach do you recommend?,Are there any other organ systems affected that we should be monitoring?,What genetic testing is available, and should our family members also be tested?,What is the chance this condition could occur again in a future pregnancy?,What developmental milestones should we watch for, and when should we seek early intervention?,Are there any long-term complications we should be aware of after surgical repair?,Can you refer us to a genetic counselor and any relevant support organizations?

Common questions about Familial omphalocele syndrome with facial dysmorphism

What is Familial omphalocele syndrome with facial dysmorphism?

Familial omphalocele syndrome with facial dysmorphism is an extremely rare genetic condition that is present at birth. The hallmark feature is an omphalocele, which is a birth defect where the baby's intestines or other abdominal organs stick out through the belly button area, covered by a thin membrane. In this particular syndrome, the omphalocele occurs alongside distinctive facial features (facial dysmorphism), which may include unusual spacing of the eyes, a broad or flat nasal bridge, low-set ears, or other subtle differences in facial structure. Because this condition runs in families, i

At what age does Familial omphalocele syndrome with facial dysmorphism typically begin?

Typical onset of Familial omphalocele syndrome with facial dysmorphism is neonatal. Age of onset can vary across affected individuals.