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Costello syndrome (CS)

Costello syndrome is a rare condition that affects multiple parts of the body. It often causes developmental delays, intellectual disability, distinctive facial features, loose skin on the hands and feet, and flexible joints

Prevalence

0.1/100,000

150–300

US Estimated

250–500

Europe Estimated

Age of Onset

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ICD-10

Q87.1

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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5 Facts you should know

FACT

1

Musculoskeletal abnormalities are common and include joint laxity, ulnar deviation of the wrists, scoliosis, and tight Achilles tendons, which may lead to delayed motor milestones and require orthopedic intervention

FACT

2

Characteristic craniofacial features include coarse facial appearance, full lips, nasal papillomas, and macrocephaly, often accompanied by loose, soft skin and deep palmar/plantar creases

FACT

3

Feeding difficulties, failure to thrive, and developmental delay are common early signs, with most children requiring nutritional support in infancy

FACT

4

Cardiac abnormalities are frequent, particularly hypertrophic cardiomyopathy, arrhythmias (especially multifocal atrial tachycardia), and structural defects such as pulmonary valve stenosis

FACT

5

There is an elevated risk of malignancy, especially rhabdomyosarcoma, neuroblastoma, and transitional cell carcinoma of the bladder, requiring ongoing surveillance throughout childhood

Costello syndrome is also known as...

Costello syndrome is also known as:

  • Faciocutaneoskeletal Syndrome

 

What’s your Rare IQ?

What is a hallmark sign often seen in infants with Costello syndrome?

Common signs and symptoms

Coarse facial features

Loose skin, especially on hands and feet

Delayed growth and motor development

Feeding difficulties in infancy

Curly, sparse, or fine hair

Intellectual disability

Heart problems

Musculoskeletal issues

Current treatments

There is no cure, but care is supportive and multidisciplinary:

Cardiology

Monitor and treat heart defects

Feeding therapy

Especially in early years

Orthopedics/Physical therapy

For joint and skeletal issues

Dermatology & ENT

For skin and airway issues

Oncology monitoring

Due to increased tumor risk