Pterygium colli-intellectual disability-digital anomalies syndrome

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ORPHA:2988OMIM:600159Q87.0
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Overview

Pterygium colli-intellectual disability-digital anomalies syndrome is an extremely rare genetic condition that affects multiple parts of the body. The name describes its three main features: pterygium colli (a webbed or broad neck caused by extra skin folds), intellectual disability of varying degrees, and digital anomalies (unusual features of the fingers and toes). Children born with this syndrome may have a short or webbed neck that can resemble features seen in Turner syndrome or Noonan syndrome, but this condition is distinct. The intellectual disability can range from mild to moderate, affecting learning, speech development, and daily functioning. Digital anomalies may include short fingers (brachydactyly), unusually shaped fingers, or other hand and foot differences. Some affected individuals may also have additional features such as short stature, facial differences, or skeletal abnormalities. Because this syndrome is so rare, there is no specific cure or targeted treatment. Management focuses on supportive care, including special education services, speech therapy, occupational therapy, and orthopedic care when needed. Early intervention programs can help children reach their full developmental potential. A team of specialists typically works together to address the various aspects of this condition.

Also known as:

Key symptoms:

Webbed or broad neck (pterygium colli)Intellectual disabilityAbnormal fingers or toesShort fingers (brachydactyly)Short statureDelayed speech and language developmentLearning difficultiesUnusual facial featuresLow-set earsLimited range of motion in jointsDelayed motor milestones

Clinical phenotype terms (16)— hover any for plain English
Epicanthus inversusHP:0000537Enlarged interphalangeal jointsHP:0006247Proximal placement of thumbHP:0009623Abnormality of the epiphysis of the distal phalanx of the thumbHP:0009662Broad distal phalanx of fingerHP:0009836Plantar edemaHP:0025537Palmar edemaHP:0025538
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 Pterygium colli-intellectual disability-digital anomalies syndrome.

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No actively recruiting trials found for Pterygium colli-intellectual disability-digital anomalies syndrome at this time.

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No specialists are currently listed for Pterygium colli-intellectual disability-digital anomalies syndrome.

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 Pterygium colli-intellectual disability-digital anomalies syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific evaluations does my child need to confirm this diagnosis?,Should we pursue genetic testing such as whole exome sequencing?,What therapies should we start right away to support my child's development?,How often should my child be seen by each specialist?,Are there any other conditions we should rule out that look similar?,What educational resources and support services are available for my child?,Is there any ongoing research or clinical trials related to this condition?

Common questions about Pterygium colli-intellectual disability-digital anomalies syndrome

What is Pterygium colli-intellectual disability-digital anomalies syndrome?

Pterygium colli-intellectual disability-digital anomalies syndrome is an extremely rare genetic condition that affects multiple parts of the body. The name describes its three main features: pterygium colli (a webbed or broad neck caused by extra skin folds), intellectual disability of varying degrees, and digital anomalies (unusual features of the fingers and toes). Children born with this syndrome may have a short or webbed neck that can resemble features seen in Turner syndrome or Noonan syndrome, but this condition is distinct. The intellectual disability can range from mild to moderate, a

How is Pterygium colli-intellectual disability-digital anomalies syndrome inherited?

Pterygium colli-intellectual disability-digital anomalies syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Pterygium colli-intellectual disability-digital anomalies syndrome typically begin?

Typical onset of Pterygium colli-intellectual disability-digital anomalies syndrome is neonatal. Age of onset can vary across affected individuals.