Craniosynostosis occurs when the sutures, edges of the skull bones, in an infant’s skull close too early. Normally, these sutures close between the ages of two and three. The head stops growing in the places where the sutures have fused, causing the skull and facial bones to expand abnormally.
Craniosynostosis often occurs by chance, but can be linked to certain genetic disorders such as:
- Carpenter syndrome
- Crouzon syndrome
- Pfeiffer syndrome
- Saethre-Chotzen syndrome
These disorders are either autosomal recessive, needing two copies of the gene for the condition to expressed, or autosomal dominant, needing one gene for the condition to be expressed. It is important for families to be tested to uncover a genetic cause for craniosynostosis.
If left untreated, craniosynostosis can lead to:
- Developmental Delay
- Head deformity
- Increased pressure on the brain
Types of Craniosynostosis
There are several types of craniosynostosis. They are named for the suture or sutures involved. The three most common types of craniosynostosis are scaphocephaly, plagiocephaly, and trigonocephaly.
Scaphocephaly is the most common type of craniosynostosis. It involves the closing of the sagittal suture which runs down the middle of the top of the head. This fusion causes a long, narrow skull and a broad forehead. Scaphocephaly is more common in boys than in girls.
Plagiocephaly is the next most common type of craniosynostosis and is most common in girls than boys. It involves the closing of the sutures that run from the top of the head toward either ear. There are two types of plagiocephaly, anterior and deformational.
Anterior plagiocephaly occurs when either the right or left side of the coronal suture fuses. Also called coronal synostosis, this condition causes the forehead to stop growing and produces a flattening of the brow and forehead on the affected side and a prominent forehead on the opposite side.
Deformational plagiocephaly, which is a more common type of plagiocephaly, occurs when the head becomes misshaped due to repeated pressure to the same area of the head. Not actually craniosynostosis, this condition does not require surgery. The increase in the number of infants with deformational plagiocephaly may be linked to the “Back to Sleep” initiative meant to help prevent sudden infant death syndrome (SIDS). Treatment for this type of plagiocephaly depends on the severity of the child’s condition.
Trigonocephaly, a rare form of craniosynostosis, is a fusion of the forehead suture which runs from the middle of the forehead toward the nose. This type of fusion could lead to a ridge running down the middle of the forehead. In these cases, the forehead can appear pointed and the eyes may be placed close together. Trigonocephaly is more common in boys than girls.
Diagnosis of Craniosynostosis
Abnormal changes in the shape of an infant’s head or face are the most noticeable signs of craniosynostosis. Other symptoms include:
- Bony ridges along the affected sutures
- Bulging eyes and/or fontanel (soft spot)
- Developmental delays
- Facial abnormalities
- High-pitched crying
- Increased head circumference
- Increased irritability
- Poor feeding or projectile vomiting
Cranionsynostosis can be observed at birth or identified later after a physical exam and diagnostic testing. During the visit, the doctor may ask about the infant’s prenatal and birth history and family history of craniosynostosis or facial abnormalities, and developmental delays. Measurements of the child’s head will also be taken at this time. Your physician may also conduct:
- Diagnostic imaging tests, such as CT scans, to check for fusions
- Genetic tests, to identify any syndromic causes
- X-rays, which also check for fused sutures
Treatment of Craniosynostosis
The goal of treatment for craniosynostosis is to relive the pressure inside the skull so that the brain can grow properly and correct any deformities. Specific treatment options rely on: the child’s age and health, the severity and type of craniosynostosis, the child’s tolerance to medications, therapies, and procedures, the parent’s preference, and the overall expectations.
Surgical and non-surgical treatment options are available for children with craniosynostosis.
Surgery for Craniosynostosis
Surgery is typically recommended for craniosynostosis treatment. Because the bones are still soft, the best time to perform surgery is before the age of one and as early as one month, depending on the severity of the condition.
There are two types of surgeries used to correct craniosynostosis, traditional surgery and minimally invasive endoscopic surgery.
Traditional Surgery – Calvarial Vault Remodeling
Traditional surgery is also referred to as calvarial vault remodeling. With this procedure, the shape of the baby’s head is corrected by moving the abnormally or prematurely fused area of the skull in order to help achieve a rounded shape. This surgery can take up to eight hours and is recommended for those over six months of age because of the expected blood loss.
Post-surgery, babies usually spend one night in the ICU and up to five days in the hospital for monitoring. There may be temporary swelling of the face. Follow-up visits may be required one month, six months, and 12 months post-surgery. With traditional surgery, there are no additional treatments necessary, unless a recurrence of craniosynostosis is found.
Minimally Invasive Endoscopic Surgery
Endoscopic surgery is usually performed on babies less than three months old. Using an endoscope, the surgeon opens the abnormally or prematurely fused sutures allowing the baby’s brain to grow normally. This procedure takes about an hour and the patient is usually released to go home after an overnight stay.
After this procedure, the baby wears a molding helmet. Follow-up visits with the neurosurgeon occur every three months for the first year after surgery to monitor progress.
Nonsurgical Treatment for Craniosynostosis – Helmet Molding Therapy
Helmet molding therapy, cranial orthosis, is used to correct the abnormal shape of the skull found with deformational plagiocephaly, brachycephaly, or scaphocephaly in patients younger than one year old. The helmet has a hard exterior and a foam lining. This therapy allows for growth in the flat regions while restricting growth in the prominent areas.
To be effective, the helmet must be worn 23 hours a day and adjusted by the doctor every two weeks to allow for the baby’s rapid head growth. The length of the treatment depends largely on the age at which the therapy begins and the severity of the condition.
Frequently Asked Questions for Parents
What is craniosynostosis?
Craniosynostosis is the abnormally premature fusion of the skull. When craniosynostosis occurs the head grows in a manner which causes an abnormal shape of the head.
When can possible abnormalities be detected?
Deformities can usually be seen soon after birth. Once detected by the parents or pediatrician, a referral to a specialist can be made.
When should my child see a craniofacial specialist?
It is best to have your child see a specialist as soon as possible, within the first few weeks of life. The necessary treatment options can be discussed at this time. With treatments such as minimally invasive endoscopy, the optimal time for treatment is before three months of age when the bones are still moldable.
What is helmet molding therapy?
Helmet molding therapy is a treatment used to correct the shape of an abnormal skull using a helmet. This therapy discourages the baby from resting on the flattened side, allowing it to regain its normal shape.
Will my baby have to wear the helmet all day?
Yes. It is recommended that the baby wear the helmet 23 hours a day.
How long will my baby wear a helmet?
The average treatment with a helmet is anywhere from nine months to one year. Treatment duration depends largely on the severity of the child’s condition.
Brachycephaly: A birth defect characterized by the disproportionate shortness of the head. It is caused by a premature fusing of the coronal suture. Brachycephaly is commonly associated with a number of syndromes and can also occur from infant positioning during sleep.
Craniosynostosis: The abnormally/premature fusing of the skull bones.
Crouzon’s syndrome: A birth defect characterized by abnormalities in the skull and facial bones, caused by a fusing of both sides of the coronal suture which causes the skull to be short in the front and the back. Flat cheek bones and a flat nose are also typical.
Pfeiffer’s syndrome: A birth defect which causes abnormalities of the skull, hands, and feet. This syndrome results in wide-set, bulging eyes, an underdeveloped upper jaw, and a beaked nose due to the head being unable to grow normally.
Plagiocephaly: An asymmetrical shape of the head with flatness on the back of the head or forehead.
Saethre-Chotzen syndrome: A birth defect which causes an unusually short or broad head. The eyes may be spaced far apart with droopy eyelids, and fingers may be abnormally short and webbed.
Scaphocephaly: Scaphocephaly most commonly results from early fusion of the sagittal suture, which runs front to back, down the middle of the top of the head. It causes a head shape that is long and narrow.
Trigonocephaly: A triangular configuration of the skull caused by the premature fusion of the two halves of the frontal bones at the metopic suture, which extends down the middle of the forehead toward the nose.
If you would like more information about this craniofacial anomaly, please contact the Craniofacial Team of Texas by calling 512-377-1142 or toll free 877-612-7069 to schedule an appointment or complete an online appointment request.