Cardiocranial syndrome, Pfeiffer type

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:2872OMIM:218450Q87.8
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Cardiocranial syndrome, Pfeiffer type (also known as Pfeiffer cardiocranial syndrome) is an extremely rare genetic condition that affects both the heart and the skull. This syndrome was first described by Pfeiffer and involves a combination of heart defects (congenital heart disease) and abnormalities in the shape and structure of the skull (craniosynostosis or other cranial malformations). Affected individuals may also have additional features such as abnormalities of the limbs, facial differences, and developmental delays. Because the heart and skull are both affected from birth, this condition is typically recognized in newborns or during infancy. Heart defects can range from mild to severe and may include structural problems such as septal defects or other malformations. The skull abnormalities can lead to an unusual head shape and may sometimes increase pressure inside the skull if the bones fuse too early. Treatment is focused on managing the specific symptoms each person has. Heart surgery may be needed to correct structural heart defects, and skull surgery (cranial surgery) may be required if there is premature fusion of skull bones. Developmental support, physical therapy, and regular monitoring by a team of specialists are important parts of care. Because this condition is so rare, there is limited published information, and management is highly individualized based on each patient's needs.

Also known as:

Key symptoms:

Heart defects present at birthAbnormal skull shapeEarly fusion of skull bones (craniosynostosis)Unusual facial featuresLimb abnormalitiesGrowth delaysDevelopmental delaysShort statureWidely spaced eyesDepressed nasal bridge

Clinical phenotype terms (34)— hover any for plain English
Deep palmar creaseHP:0006191Plantar flexion contractureHP:0008112Contracture of the proximal interphalangeal joint of the 2nd fingerHP:0009540Small hypothenar eminenceHP:0010487Cutaneous syndactyly of toesHP:0010621Abnormal hair whorlHP:0010721Sagittal craniosynostosisHP:0004442Temporomandibular joint ankylosisHP:0012478
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 Cardiocranial syndrome, Pfeiffer type.

View clinical trials →

No actively recruiting trials found for Cardiocranial syndrome, Pfeiffer type at this time.

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

Search ClinicalTrials.gov ↗Join the Cardiocranial syndrome, Pfeiffer type community →

No specialists are currently listed for Cardiocranial syndrome, Pfeiffer type.

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 Cardiocranial syndrome, Pfeiffer type.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Cardiocranial syndrome, Pfeiffer typeForum →

No community posts yet. Be the first to share your experience with Cardiocranial syndrome, Pfeiffer type.

Start the conversation →

Latest news about Cardiocranial syndrome, Pfeiffer type

No recent news articles for Cardiocranial syndrome, Pfeiffer type.

Follow this condition to be notified when news becomes available.

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.How severe is my child's heart defect, and will surgery be needed?,Does my child have craniosynostosis, and if so, when should surgery be considered?,What developmental milestones should I watch for, and when should I be concerned?,Are there genetic tests that can help confirm the diagnosis or identify the cause?,What specialists should be part of my child's care team?,What are the signs of an emergency that I should watch for at home?,Is genetic counseling available for our family regarding future pregnancies?

Common questions about Cardiocranial syndrome, Pfeiffer type

What is Cardiocranial syndrome, Pfeiffer type?

Cardiocranial syndrome, Pfeiffer type (also known as Pfeiffer cardiocranial syndrome) is an extremely rare genetic condition that affects both the heart and the skull. This syndrome was first described by Pfeiffer and involves a combination of heart defects (congenital heart disease) and abnormalities in the shape and structure of the skull (craniosynostosis or other cranial malformations). Affected individuals may also have additional features such as abnormalities of the limbs, facial differences, and developmental delays. Because the heart and skull are both affected from birth, this condi

How is Cardiocranial syndrome, Pfeiffer type inherited?

Cardiocranial syndrome, Pfeiffer type follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Cardiocranial syndrome, Pfeiffer type typically begin?

Typical onset of Cardiocranial syndrome, Pfeiffer type is neonatal. Age of onset can vary across affected individuals.