Craniosynostosis, Philadelphia type

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ORPHA:1527OMIM:185900Q87.0
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8Treatment centers

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Overview

Craniosynostosis, Philadelphia type is an extremely rare genetic condition in which one or more of the joints (called sutures) between the bones of a baby's skull close too early, before the brain has fully grown. This early closure is known as craniosynostosis. In the Philadelphia type specifically, craniosynostosis is combined with other skeletal abnormalities, particularly syndactyly, which means some fingers or toes are fused or webbed together. The condition is present at birth and can affect the shape of the head, the development of the face, and the hands and feet. Because the skull bones fuse prematurely, the growing brain may be under pressure, which can lead to problems with brain development if not treated. The abnormal skull shape is usually noticeable at birth or shortly after. Children with this condition may also have short stature and other bone-related findings. The severity can vary from person to person. Treatment typically involves surgery to release the fused skull sutures and allow the brain to grow normally. Additional surgeries may be needed to correct hand or foot abnormalities and to improve the appearance and function of affected areas. A team of specialists, including craniofacial surgeons, neurosurgeons, and orthopedic surgeons, usually manages care. Early diagnosis and intervention are important to reduce the risk of complications such as increased pressure inside the skull.

Key symptoms:

Abnormal head shape due to early skull bone fusionFused or webbed fingers (syndactyly of the hands)Fused or webbed toes (syndactyly of the feet)Raised pressure inside the skullFlattened or unusual facial featuresShort staturePossible developmental delaysBulging or tense soft spot on the headRidging along skull suture linesPossible vision problems from skull pressure

Clinical phenotype terms (3)— hover any for plain English
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 Craniosynostosis, Philadelphia type.

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No actively recruiting trials found for Craniosynostosis, Philadelphia type at this time.

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No specialists are currently listed for Craniosynostosis, Philadelphia type.

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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 Craniosynostosis, Philadelphia type.

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Community

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Caregiver Resources

NORD Caregiver Resources

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Mental Health Support

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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.Which skull sutures are affected, and how severe is the fusion?,When should surgery be performed, and what type of surgery is recommended?,What are the risks and expected outcomes of the surgery?,Will my child need additional surgeries for the hands or feet?,Should we pursue genetic testing, and what would the results mean for our family?,What signs of increased intracranial pressure should I watch for at home?,What developmental support or therapies should we start early?

Common questions about Craniosynostosis, Philadelphia type

What is Craniosynostosis, Philadelphia type?

Craniosynostosis, Philadelphia type is an extremely rare genetic condition in which one or more of the joints (called sutures) between the bones of a baby's skull close too early, before the brain has fully grown. This early closure is known as craniosynostosis. In the Philadelphia type specifically, craniosynostosis is combined with other skeletal abnormalities, particularly syndactyly, which means some fingers or toes are fused or webbed together. The condition is present at birth and can affect the shape of the head, the development of the face, and the hands and feet. Because the skull bo

How is Craniosynostosis, Philadelphia type inherited?

Craniosynostosis, Philadelphia type follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Craniosynostosis, Philadelphia type typically begin?

Typical onset of Craniosynostosis, Philadelphia type is neonatal. Age of onset can vary across affected individuals.