Enlarged parietal foramina

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ORPHA:60015OMIM:168500Q75.8
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Overview

Enlarged parietal foramina (EPF), also known as foramina parietalia permagna or parietal foramina with cleidocranial dysplasia, is a rare condition where two small openings (holes) in the back of the skull, called parietal foramina, are larger than normal. In most people, these openings close before or shortly after birth. In people with EPF, they stay open and enlarged throughout life. The condition is usually present from birth and can be seen on imaging of the skull. For many people, enlarged parietal foramina cause no symptoms at all and are discovered by accident during a brain scan done for another reason. However, some people experience headaches, scalp tenderness over the openings, or in rare cases, seizures. The skin and tissue over the openings may feel soft or pulsate. In some cases, EPF is linked to other brain or skull differences. Treatment is usually not needed for people without symptoms. Those who do have symptoms, such as seizures or headaches, are treated for those specific problems. Because this condition is genetic, family members may also be checked. The outlook for most people with EPF is very good, especially when no other health problems are present.

Also known as:

Key symptoms:

Enlarged openings in the back of the skull visible on imagingHeadachesScalp tenderness or sensitivity over the skull openingsSoft or pulsating feeling on the scalp at the back of the headSeizures (in some cases)No symptoms at all in many people (found by accident)

Clinical phenotype terms (20)— hover any for plain English
Parietal foraminaHP:0002697Abnormal posterior cranial fossa morphologyHP:0000932Venous malformationHP:0012721Scalp tendernessHP:0100809Short claviclesHP:0000894Occipital encephaloceleHP:0002085MyelomeningoceleHP:0002475Multiple exostosesHP:0002762Aplasia cutis congenita of scalpHP:0007385Congenital craniofacial dysostosisHP:0008497Abnormal cerebral vein morphologyHP:0012480EncephalomalaciaHP:0040197
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

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 Enlarged parietal foramina.

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No actively recruiting trials found for Enlarged parietal foramina at this time.

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No specialists are currently listed for Enlarged parietal foramina.

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 Enlarged parietal foramina.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Do I need any regular monitoring or follow-up imaging for this condition?,Should I or my child wear a helmet or protective headgear during sports or activities?,What are the signs that something has gone wrong and I need to go to the emergency room?,Should other family members be tested for this condition?,Is genetic counseling recommended if I am planning to have children?,Are there any activity restrictions I should follow?,What should I tell my other doctors and emergency providers about this condition?

Common questions about Enlarged parietal foramina

What is Enlarged parietal foramina?

Enlarged parietal foramina (EPF), also known as foramina parietalia permagna or parietal foramina with cleidocranial dysplasia, is a rare condition where two small openings (holes) in the back of the skull, called parietal foramina, are larger than normal. In most people, these openings close before or shortly after birth. In people with EPF, they stay open and enlarged throughout life. The condition is usually present from birth and can be seen on imaging of the skull. For many people, enlarged parietal foramina cause no symptoms at all and are discovered by accident during a brain scan done

How is Enlarged parietal foramina inherited?

Enlarged parietal foramina follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Enlarged parietal foramina typically begin?

Typical onset of Enlarged parietal foramina is neonatal. Age of onset can vary across affected individuals.