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Overview
Craniosynostosis (kray-nee-o-sin-os-TOE-sis)
is a birth defect in which one or more of the
fibrous joints between the bones of your baby's
skull (cranial sutures) close prematurely (fuse),
before your baby's brain is fully formed. Brain
growth continues, giving the head a misshapen
appearance.

Normally, during infancy the sutures remain

Cranial sutures and
flexible, giving your baby's brain time to grow. fontanels
In the front of the skull, the sutures meet in the
large soft spot (fontanel) on top of the head.
The anterior fontanel is the soft spot you feel
just behind your baby's forehead. The next
largest is at the back (posterior). Each side of
the skull has a tiny fontanel.
Children's Center
Craniosynostosis usually involves premature At Mayo Clinic, medical
fusion of a single cranial suture, but can involve and surgical specialists
more than one of the sutures in your baby's from over 70 areas are
skull (multiple suture craniosynostosis). In rare available to collaborate in
cases, craniosynostosis is caused by certain the treatment of your
genetic syndromes (syndromic child.
craniosynostosis).
Treating craniosynostosis involves surgery to Read about the Mayo Clinic does not endorse companies or
Children's Center. products. Advertising revenue supports our no
correct the shape of the head and allow for
for-profit mission.
normal brain growth. Early diagnosis and
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treatment allow your baby's brain adequate space to grow and develop.
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Although neurological damage can occur in severe cases, most children

have normal cognitive development and achieve good cosmetic results after
surgery. Early diagnosis and treatment are key.
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Symptoms
The signs of craniosynostosis are usually noticeable at birth, but they'll
become more apparent during the first few months of your baby's life. Signs
and severity depend on how many sutures are fused and when in brain
development the fusion occurs. These can include:

A misshapen skull, with the shape depending on which of the sutures

are affected

An abnormal feeling or disappearing fontanel on your baby's skull

Development of a raised, hard ridge along affected sutures

Slow or no growth of the head as your baby grows

Types of craniosynostosis

There are several types of craniosynostosis. Most involve the fusion of a

single cranial suture. Some complex forms of craniosynostosis involve the
fusion of multiple sutures. Most cases of multiple suture craniosynostosis
are linked to genetic syndromes and are called syndromic craniosynostosis.

The term given to each type of craniosynostosis depends on what sutures

are affected. Types of craniosynostosis include:
 Sagittal (scaphocephaly). Premature fusion of the sagittal suture that
runs from the front to the back at the top of the skull forces the head to
grow long and narrow. Sagittal craniosynostosis results in a head
shape called scaphocephaly and is the most common type of
craniosynostosis.

Coronal. Premature fusion of one of the coronal sutures (unicoronal)

that run from each ear to the top of the skull may cause the forehead to
flatten on the affected side and bulge on the unaffected side. It also
leads to turning of the nose and a raised eye socket on the affected
side. When both coronal sutures fuse prematurely (bicoronal), the head
has a short and wide appearance, often with the forehead tilted
forward.

Metopic. The metopic suture runs from the top of the bridge of the
nose up through the midline of the forehead to the anterior fontanel and
the sagittal suture. Premature fusion gives the forehead a triangular
appearance and widens the back part of the head. This is also called
trigonocephaly.

Lambdoid. Lambdoid synostosis is a rare type of craniosynostosis that

involves the lambdoid suture, which runs along the back of the head. It
may cause one side of your baby's head to appear flat, one ear to be
higher than the other ear and tilting of the top of the head to one side.

Other reasons for a misshapen head

A misshapen head doesn't always indicate craniosynostosis. For example, if

the back of your baby's head appears flattened, it could be the result of
spending too much time on one side of his or her head. This can be treated
with regular position changes, or if significant, with helmet therapy (cranial
orthosis) to help reshape the head to a more normal appearance.

When to see a doctor

Your doctor will routinely monitor your child's head growth at well-child visits.
Talk to your pediatrician if you have concerns about your baby's head
growth or shape.

Request an Appointment at Mayo Clinic

Causes
Often the cause of craniosynostosis is not known, but sometimes it's related
to genetic disorders.
 Nonsyndromic craniosynostosis is the most common type of
craniosynostosis, and its cause is unknown, although it's thought to be
a combination of genes and environmental factors.

Syndromic craniosynostosis is caused by certain genetic

syndromes, such as Apert syndrome, Pfeiffer syndrome or Crouzon
syndrome, which can affect your baby's skull development. These
syndromes usually also include other physical features and health
problems.

Complications
If untreated, craniosynostosis may cause, for example:

Permanent head and facial deformity

Poor self-esteem and social isolation

The risk of increased pressure inside the skull (intracranial pressure) from
simple craniosynostosis is small, as long as the suture and head shape are
fixed surgically. But babies with an underlying syndrome may develop
increased intracranial pressure if their skulls don't expand enough to make
room for their growing brains.

If untreated, increased intracranial pressure can cause:

Developmental delays

Cognitive impairment

No energy or interest (lethargy)

Blindness

Eye movement disorders

Seizures

Death, in rare instances

By Mayo Clinic Staff

Craniosynostosis care at Mayo Clinic

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 Diagnosis & treatment

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Related
Cranial sutures and fontanels

Craniosynostosis

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CT scan

Genetic testing

X-ray

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