Pectus excavatum-macrocephaly-dysplastic nails syndrome

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ORPHA:2835OMIM:600399Q87.5
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8Treatment centers

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Overview

Pectus excavatum-macrocephaly-dysplastic nails syndrome is an extremely rare genetic condition that affects several parts of the body at the same time. The three main features are pectus excavatum (a sunken or caved-in chest), macrocephaly (an unusually large head), and dysplastic nails (abnormally shaped or formed fingernails and toenails). This syndrome was first described in a small number of patients, and very little is known about it in the medical literature. People with this condition are typically born with or develop a noticeable indentation of the breastbone and chest wall, which can range from mild to severe. The large head size is usually present from birth or early infancy. The nail abnormalities may include nails that are thin, ridged, brittle, or unusually shaped. Some affected individuals may also have other skeletal or developmental differences. Because this syndrome is so rare, there is no specific cure or targeted treatment. Management focuses on addressing each symptom individually. For example, severe pectus excavatum may be treated with surgery if it causes breathing or heart problems. Nail care and monitoring of head growth are also part of ongoing management. Genetic counseling is recommended for affected families to understand the risk of passing the condition to future children.

Also known as:

Key symptoms:

Sunken or caved-in chest (pectus excavatum)Unusually large head (macrocephaly)Abnormally shaped or brittle fingernails and toenailsPossible skeletal abnormalitiesPossible growth differencesChest wall deformity visible from the outsideNails that may be thin, ridged, or poorly formed

Clinical phenotype terms (15)— hover any for plain English
Nail dysplasiaHP:0002164Hypoplasia of the zygomatic boneHP:0010669
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 Pectus excavatum-macrocephaly-dysplastic nails syndrome.

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No actively recruiting trials found for Pectus excavatum-macrocephaly-dysplastic nails syndrome at this time.

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No specialists are currently listed for Pectus excavatum-macrocephaly-dysplastic nails 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 Pectus excavatum-macrocephaly-dysplastic nails syndrome.

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Community

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Latest news about Pectus excavatum-macrocephaly-dysplastic nails syndrome

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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.How severe is my child's pectus excavatum, and does it need surgical correction?,Is the large head size causing any pressure on the brain, and how will you monitor this?,Should we pursue genetic testing to look for an underlying cause?,What specialists should be part of our care team?,Are there any activity restrictions my child should follow?,What signs or symptoms should prompt an emergency visit?,Is there a risk of passing this condition to future children?

Common questions about Pectus excavatum-macrocephaly-dysplastic nails syndrome

What is Pectus excavatum-macrocephaly-dysplastic nails syndrome?

Pectus excavatum-macrocephaly-dysplastic nails syndrome is an extremely rare genetic condition that affects several parts of the body at the same time. The three main features are pectus excavatum (a sunken or caved-in chest), macrocephaly (an unusually large head), and dysplastic nails (abnormally shaped or formed fingernails and toenails). This syndrome was first described in a small number of patients, and very little is known about it in the medical literature. People with this condition are typically born with or develop a noticeable indentation of the breastbone and chest wall, which ca

At what age does Pectus excavatum-macrocephaly-dysplastic nails syndrome typically begin?

Typical onset of Pectus excavatum-macrocephaly-dysplastic nails syndrome is neonatal. Age of onset can vary across affected individuals.