Macrosomia-microphthalmia-cleft palate syndrome

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ORPHA:2432OMIM:248110Q87.0
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Overview

Macrosomia-microphthalmia-cleft palate syndrome is a very rare condition that affects several parts of the body at the same time. It is sometimes called Froster-Iskenius syndrome or Froster syndrome. The name describes three of its main features: macrosomia (being larger than normal at birth), microphthalmia (having unusually small eyes), and cleft palate (an opening in the roof of the mouth that is present from birth). These features appear together and are noticeable from the time a baby is born. Because the eyes are smaller than normal, children with this syndrome often have significant vision problems. The cleft palate can make feeding, speaking, and breathing more difficult in early life. Being large at birth (macrosomia) may also come with other physical differences. Some children may also have intellectual disability or developmental delays, though the full range of how this condition affects each person can vary. Treatment focuses on managing each symptom individually. Surgery can help repair the cleft palate, and vision specialists can support children with eye problems. There is no cure for this syndrome, and care is provided by a team of different specialists working together to improve quality of life.

Also known as:

Key symptoms:

Larger than normal body size at birth (macrosomia)Unusually small eyes (microphthalmia)Opening in the roof of the mouth (cleft palate)Vision problems or reduced visionDifficulty feeding as a newbornSpeech and language delaysIntellectual disability or developmental delaysDistinctive facial features

Clinical phenotype terms (11)— hover any for plain English
Abnormal cranial suture/fontanelle morphologyHP:0000235Abnormal calvaria morphologyHP:0002683Median cleft palateHP:0009099
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 Macrosomia-microphthalmia-cleft palate syndrome.

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No actively recruiting trials found for Macrosomia-microphthalmia-cleft palate syndrome at this time.

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No specialists are currently listed for Macrosomia-microphthalmia-cleft palate 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 Macrosomia-microphthalmia-cleft palate syndrome.

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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.What genetic tests should we do to confirm the diagnosis and understand the cause?,What specialists does my child need to see, and how often?,When should cleft palate surgery be done, and what should we expect during recovery?,What vision supports are available for my child, and will their vision get worse over time?,Are there early intervention or therapy programs you recommend starting right away?,What are the chances that future children could be affected by this condition?,Are there any research studies or registries we can join to help advance understanding of this syndrome?

Common questions about Macrosomia-microphthalmia-cleft palate syndrome

What is Macrosomia-microphthalmia-cleft palate syndrome?

Macrosomia-microphthalmia-cleft palate syndrome is a very rare condition that affects several parts of the body at the same time. It is sometimes called Froster-Iskenius syndrome or Froster syndrome. The name describes three of its main features: macrosomia (being larger than normal at birth), microphthalmia (having unusually small eyes), and cleft palate (an opening in the roof of the mouth that is present from birth). These features appear together and are noticeable from the time a baby is born. Because the eyes are smaller than normal, children with this syndrome often have significant vi

How is Macrosomia-microphthalmia-cleft palate syndrome inherited?

Macrosomia-microphthalmia-cleft palate syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Macrosomia-microphthalmia-cleft palate syndrome typically begin?

Typical onset of Macrosomia-microphthalmia-cleft palate syndrome is neonatal. Age of onset can vary across affected individuals.