Craniosynostosis, Herrmann-Opitz type

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Overview

Craniosynostosis, Herrmann-Opitz 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 premature fusion of skull bones is called craniosynostosis. In the Herrmann-Opitz type, craniosynostosis is accompanied by additional features that may include short stature, limb abnormalities (such as brachydactyly, meaning short fingers and toes), and sometimes intellectual disability or developmental delays. Facial features may appear unusual due to the abnormal skull shape, and some affected individuals may have other skeletal anomalies. This condition was first described by Herrmann and Opitz, and it is sometimes referred to as Herrmann-Opitz craniosynostosis syndrome. Because it is so rare, with only a handful of cases reported in the medical literature, much about this condition remains poorly understood. The treatment approach typically involves surgery to correct the skull fusion and relieve pressure on the growing brain, along with supportive therapies to address developmental or skeletal issues. A team of specialists including craniofacial surgeons, geneticists, and developmental pediatricians usually manages care. Early diagnosis and intervention are important to reduce the risk of complications such as increased pressure inside the skull, which can affect brain development.

Key symptoms:

Premature fusion of skull bones (craniosynostosis)Abnormal head shapeShort statureShort fingers and toes (brachydactyly)Developmental delaysIntellectual disabilityUnusual facial featuresSkeletal abnormalitiesPossible increased pressure inside the skullLimb anomalies

Clinical phenotype terms (24)— hover any for plain English
Abnormality of the urethraHP:0000795
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 Craniosynostosis, Herrmann-Opitz type.

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No actively recruiting trials found for Craniosynostosis, Herrmann-Opitz type at this time.

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No specialists are currently listed for Craniosynostosis, Herrmann-Opitz 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, Herrmann-Opitz type.

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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.How severe is my child's craniosynostosis, and when should surgery be done?,What type of surgery is recommended, and what are the risks?,Will my child need more than one surgery as they grow?,What developmental therapies should we start, and how soon?,Is genetic testing available to confirm the specific diagnosis?,What signs of complications should I watch for at home?,Are there other family members who should be tested or evaluated?

Common questions about Craniosynostosis, Herrmann-Opitz type

What is Craniosynostosis, Herrmann-Opitz type?

Craniosynostosis, Herrmann-Opitz 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 premature fusion of skull bones is called craniosynostosis. In the Herrmann-Opitz type, craniosynostosis is accompanied by additional features that may include short stature, limb abnormalities (such as brachydactyly, meaning short fingers and toes), and sometimes intellectual disability or developmental delays. Facial features may appear unusual due to the abnormal skull shap

How is Craniosynostosis, Herrmann-Opitz type inherited?

Craniosynostosis, Herrmann-Opitz type follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Craniosynostosis, Herrmann-Opitz type typically begin?

Typical onset of Craniosynostosis, Herrmann-Opitz type is neonatal. Age of onset can vary across affected individuals.