Craniosynostosis

What is Craniosynostosis?

Craniosynostosis occurs in one out of every 2,000 live births.

Craniosynostosis is when an infant’s sutures fuse too early and it affects the growth of the brain. Often, the end result is an infant with an abnormally shaped head. An infant’s brain begins to grow before birth and continues to grow throughout the infant’s first three years of life. At birth, an infant has 40% of his/her adult brain volume and this increases to 80% by three years of age, and to 90% by seven years of age. An infant’s skull consists of bones that are separated by soft joints called "sutures". The "soft spot", or fontanelle, is where the sutures meet on the top of the forehead. As an infant’s brain continues to grow, the sutures continue to grow. Throughout an infant’s first three years of life, an infant’s sutures will close or "fuse" in sequence. Craniosynostosis may involve the premature closure of a single suture or multiple sutures in the case of a syndrome.

Craniosynostosis, if left untreated, can cause severe and permanent damage. An infant may have increased intracranial pressure, seizures, neurological deficiencies, eye problems, malalignment of the spine, and cognitive and developmental delays.

The key to treating an infant with Craniosynostosis is accurate early diagnosis and medical treatment. Be sure to bring your infant to well-child visits, so your pediatrician can routinely chart the growth of your infant's head over time. This may help identify the problem early, if it occurs. Please keep in mind that well-child visits are just one part of an overall good health plan. Remain proactive and be informed.

There are four common types of Craniosynostosis:

	Unicoronal - coronal sutures run from each ear to the sagittal suture at the top of the head; the fusion of one suture
	Bicoronal - coronal sutures run from each ear to the sagittal suture at the top of the head; the fusion of both sutures
	Metopic - fusion of the metopic suture which runs from a baby’s nose to its sagittal suture
	Sagittal - fusion of the sagittal suture which runs from the front of the head to the back of the skull

Lambdoid Craniosynostosis is the rarest form of Craniosynostosis. CLICK HERE to read more.

Here are some more examples courtesy of MedicalModeling, LLC and David A. Staffenberg, MD:

Normal Infant Cranium (Skull)

The cranium becomes long and narrow when the sagittal suture fuses (indicated by the line).

Coronal Craniosynostosis involves one of the sutures on either side of the forehead. The forehead on the same side becomes flat, and the nose and eyes also become increasingly asymmetric.

A triangular head shape is caused by Craniosynostosis of the metopic suture which runs from the fontanel, down the middle of the forehead, to the nose. The eyes become close set and the temples become "pinched in."

CLICK HERE to learn more about Dr. David A. Staffenberg.

What Causes Craniosynostosis?

The cause of Craniosynostosis is unknown. It is sporadic. It can be present at birth (congenital). It can be hereditary. The hereditary form often occurs with other defects.

However, most cases of Craniosynostosis occur in a family with no history of the condition. Children with Craniosynostosis are otherwise healthy and have normal intelligence.

How will I know if my infant has Craniosynostosis?

Your doctor can do a physical examination. The doctor may:

Measure the width of the infant's head
Take x-rays of the skull
Perform an MRI
Perform a Computed Tomography (CT) scan of the head
Genetic testing can also be performed on the parents

Craniosynostosis vs. Plagiocephaly

Plagiocephaly, or "flat head," is common. It is usually caused by external forces and is called Deformational Plagiocephaly. While this condition may improve over the first few months, a few simple maneuvers can improve this result. Unilateral Coronal Craniosynostosis may also cause Plagiocephaly and must be considered in each case. The physical findings allow proper diagnosis in most cases. Babies with Craniosynostosis need to be identified early and referred to a Craniofacial team.

Are there symptoms of Craniosynostosis?

Some symptoms can include:

An absence of the normal feeling of a "soft spot" on the newborn's skull
Early disappearance of the "soft spot"
A raised hard ridge along the affected sutures
Unusual head shape or decreased head circumference
Slow or no increase in the head size over time as the baby grows
Scalp veins may be very noticeable
Seizures
Bulging eyes
Developmental delays

What if my infant is diagnosed with Craniosynostosis?

The main treatment for Craniosynostosis is reconstructive surgery.

The goals of the reconstructive surgery are:

Relieve any pressure on the brain
Make sure there is enough room in the skull to allow the brain to properly grow
Improve the appearance of the child's head

What else can I do for my child?

It is critically important to be evaluated and treated by a multidisciplinary craniofacial team. As you go along, you may encounter emotional and social challenges. Remember, do not be afraid to ask questions and ask for help. There are support organizations available to help you.

Courtesy of The Children's Craniofacial Association (CCA):

The following booklet publications were generously shared by the Children's Craniofacial Association

**Please note that these booklets are for informational purposes only.

A Guide To Understanding Craniosynostosis

A Guide To Understanding Craniosynostosis (Spanish)

A Parent's Journey To Acceptance

Anthropology and Craniofacial Anomalies

Apert Feet

Cleidocranial Dysplasia

Parents: You Are The Offical Care Manager

What To Bring To The Hospital

What To Expect When Your Child Goes To Surgery

Empowering Children To Cope With Teasing

Consumer Bill of Rights and Responsibilities

CLICK HERE to view the Consumer Bill of Rights and Responsibilities featured at www.hcqualitycommission.gov.

Interested in learning more?

**Please note that we do not recommend these sites. They are strictly for informational purposes only. These links should not be viewed as advice on the treatment and/or diagnosis of Craniosynostosis or any other medical condition. Advice on the treatment or care of a child suffering from Craniosynostosis should be obtained only through consultation with a physician who has examined that child or is familiar with that child's medical history.

Children's Hospital of Wisconsin

Mayo Clinic

CAPPS Kids

New York-Presbyterian

Children's Craniofacial Association (CCA)

The Children's Hospital at Montefiore

NYU Langone Medical Center

National Institute of Neurological Disorders and Stroke (NINDS)

Medline Plus

WebMD

Seattle Children's Hospital

Texas Department of State Health Services