Craniosynostosis

Craniosynostosis, or simply synostosis, is the early growing together (or fusion) of two or more bones of the skull. A newborn's skull is made up of many separate bones that are not yet fused together. Because the brain grows quickly in the first two years of life, it is important that the skull bones remain open. In fact, complete fusion of the bones normally occurs late in the teen years. Synostosis interferes with normal growth of the brain and skull.

What Is Normal Development?

A suture is a hinge of bony edges that are united by a thin layer of soft tissue. During normal development, interdigitations (folds of the membranes) develop between the bones and form a definitive suture. Later, the open cranial and facial sutures close by forming bony bridging.

At birth, the open sutures allow a lot of flexibility in craniofacial molding of bones to allow the newborn to pass through the birth canal. This molding usually normalizes within one to two weeks after birth. Normal craniofacial growth occurs through two processes: bone displacement and bone remodeling.

Shape of the Head

An abnormal head shape (plagiocephaly) can occur as a result of abnormal forces on the skull before or after birth. This can happen before birth:

  • When the baby descends into the pelvis
  • If the mom has an abnormally shaped uterus
  • If the fetus is in an odd position
  • If the mom is having twins (or triplets, etc.)

After birth, abnormal head shape is most commonly a result of gravity, when the patient lies in one position for long periods of time. 

Plagiocephaly synostotic vs. deformational.

Most of these problems will "fix themselves" within the first few months after birth due to rapid brain growth or with frequent repositioning of the baby.

Molding helmets may be used for those newborns who are not showing improvement in head shape with repositioning techniques. It is important to distinguish positional plagiocephaly (a non-surgical condition) from lambdoid synostosis and unilateral coronal synostosis, which require surgery to correct the problem. 

Positional plagiocephaly vs. lamboid synostosis.

There are several types of synostosis.

Types of craniosynostosis.

Single-Suture Synostosis (Primary)

One suture synostosed. Examples:

  • Sagittal: Head long and narrow (scaphocephaly)
  • Coronal: Flattening of the forehead (anterior plagiocephaly)
  • Lambdoid: Flattening at the back of the skull and the ear (posterior plagiocephaly)
  • Metopic: Triangular shaped head (trigonocephaly)

Double-Suture Synostosis

More than one suture synostosed. Examples:

  • Bicoronal: Skull is wider than normal (anterior brachycephaly)
  • Bilambdoid: Skull is wider than normal (posterior brachycephaly)
  • Sagittal plus metopic: Head is long and narrow (scaphocephaly)

Complex Multisuture Synostosis

More than one suture synostosed. Examples:

  • Bicoronal, sagittal, metopic: Head is short and wide and / or pointed at the top (turribrachycephaly)
  • Multisuture: Skull is shaped like a cloverleaf (Kleeblattschädel)

Secondary

Craniosynostosis secondary to known disorder. Examples:

  • Hyperthyroidism, rickets

Syndromic

Craniosynostosis and other anomalies. Examples:

  • Apert syndrome, Carpenter syndrome

Suture Closure in Humans   

Age Closure Begins 

Metopic 2
Sagittal 22
Coronal 24
Lambdoid26
Squamosal35-39
Sphenofrontal22
Sphenoparietal29
Sphenotemporal 28-32
Maso-occipital 26-30

Sagittal Synostosis (scaphocephaly)

This is the most common type of synostosis. It produces a boat-shaped head that is longer than it is wide, and is associated with frontal bossing and a palpable ridge along the closed suture.

Unilateral Coronal Synostosis

Unilateral craniosynostosis is typically characterized by the harlequin eye (almond shaped) and a flattened forehead.

Bilateral coronal synostosis (brachycephaly) is characterized by a shortened skull in the antero-posterior dimension (brachycephaly) with vertical elongation (turribrachycephaly). This type can present with Apert's syndrome and Crouzon's syndrome or as an isolated finding.

Lambdoid Synostosis

In lambdoid synostosis, there is a flattening at the back of the skull and the ear is towards the back of the head.

Metopic Synostosis (trigonocephaly)

This form of synostosis is relatively uncommon (less than 10 percent of cases) and is characterized by a bony ridge in the midline of the forehead, a triangularly shaped head, a narrow forehead and eyes that are positioned close together. 

Several syndromes are associated with synostosis.

Crouzon's Syndrome

This is the most common of the syndromes. It is associated with bilateral coronal craniosynostosis, midfacial abnormalities, forward protrusion of the eyes and airway obstruction. About 30 percent of patients will have associated hydrocephalus. There generally are no limb abnormalities seen and intelligence is often normal. This syndrome occurs in one out of 25,000 births. A mutation that may be responsible for Crouzon's syndrome has been localized of the FGFR2 gene; however up to one third of the cases occur spontaneously.

Apert's Syndrome

This syndrome is also characterized by bilateral coronal craniosynostosis, with a tall and shortened head. Other forms of synostosis may also be seen with this condition. The skull base is frequently fused as well. Additionally, hand, elbow, hip and knee deformities may be present. Like Crouzon's syndrome, the face has a classical appearance with protrusion and wide spacing of the eyes, beaked nose and down-slanting eyelids. It occurs in about one in 100,000 births. This is usually an inherited pattern. Developmental delay is common, and mental retardation is seen in 50-85 percent of cases.

Carpenter's Syndrome

This syndrome is commonly associated with lambdoid and sagittal synostosis with limb abnormalities that may include extra digits on the feet. It is also associated with abnormalities of the heart. A genetic location for this syndrome has not yet been identified.

Pfeiffer's Syndrome

This syndrome is also characterized by craniosynostosis and limb deformities. Unicoronal craniosynostosis is common as well as mid-facial deformities, protruding eyes and hearing loss. Hydrocephalus is common. Three types have been described, with types 2 and 3 being the more severe forms.

Saethre-Chotzen Syndrome

This is a congenital syndrome with commonly-associated craniosynostosis and limb deformities. The genetic location of this syndrome is different than the other syndromes. Characteristic features include coronal synostosis (usually unilateral) with restriction in growth of the anterior cranial base, a low set frontal hairline and facial asymmetry, and mild to moderate developmental delay.

Doctors haven't found all the causes for synostosis. In some case the cause appears to be genetic.

Complex Multisuture Synostosis 

Secondary Synostosis 

Hematologic disorders Congenital hemolytic icterus 
Polycythemia vera 
Sickle cell anemia 
Thalassemia
Iatrogenic disordersHydrocephalus with shunt 
Malformations Encephalocele 
Holoprosencephaly
Microcephaly
Metabolic disordersHyperthyroidism
Rickets (various forms)
Mucopolysaccharidoses and related disordersD-mannosidase deficiency
ß-glucuronidase deficiency
Hurler syndrome
Morquio syndrome
Mucolipidosis III
TeratogensAminopterin
Cyclophosphamide
Diphenylhydantoin (various forms)
Fluconazole
Retinoidse
Valproate

Craniosynostosis causes a change in the normal shape of the head. If a suture − the seam between two skull bones − is fused, it cannot grow, and the bones with open sutures then grow more than usual to allow enough room for brain growth. In many children, the only symptom may be an irregularly shaped head.

Some children will experience unusually high pressure on the brain, initially causing headaches. If left untreated, some children may then develop learning disabilities or problems with their eyesight. Early in life the brain is spared damage from this pressure by the other bones growing to accommodate the growing brain. Doctors cannot predict which children will develop this pressure problem; however, with more than one suture fused, the likelihood of increased pressure is higher.

Children who have pancraniosynostosis, in which all the large sutures in the head are fused, have a very high risk of increased pressure resulting in headaches, progressive loss of eyesight and developmental delays.

There may be reasons other than synostosis that your child's head is misshapen, so your child's doctor may order X-rays or a CT scan to screen for this condition. The doctor may refer your child to a neurosurgeon to obtain an opinion regarding the problem.

Craniosynostosis is treated by surgery that opens the fused sutures creating space for brain growth. A variety of surgical procedures may be used. The surgeon will discuss the options available for your child and choose the most appropriate procedure depending on the sutures involved and your child's age when he or she is diagnosed. Often a neurosurgeon and a plastic surgeon work together for more complex types of craniosynostosis.

At Cincinnati Children's Hospital Medical Center, we provide a multidisciplinary team to manage complex cases of syndromic craniosynostosis through our Craniofacial Center. The team includes:

Stereotactic Image Guided Endoscopic Craniosynostosis Repair

Real-time Stereotactic-Endoscopic Craniectomy: This new, minimally invasive technique can be used to treat patients with unicoronal, bicoronal and metopic synostosis. It is reserved only for young infants (less than 3 months of age); it is therefore important that patients be referred to the craniofacial team as soon as synostosis is suspected.

Surgery requires making small incisions on the scalp. A small camera (endoscope) is used that is registered to the patient's radiologic images (CT) to ensure precise removal of the pathologic suture and release of the other bones to permit normal bone growth. This is done while minimizing blood loss, decreasing hospital stay and the possibility of complications.

Endoscopic Strip Sagittal Craniectomy: An endoscopic strip sagittal craniectomy is also minimally invasive and is used with younger infants with sagittal craniosynostosis. Small incisions are made in the front and back of the head. A strip of bone encasing the fused suture approximately two inches wide is removed under endoscopic guidance.

Your child will spend the first night in the intensive care unit. Children are usually discharged on the second day after surgery if they are feeding well. The small incisions have resulted in less blood loss compared to conventional procedures and less swelling around the head and eyes.

Standard Craniosynostosis Surgery

Strip Sagittal Craniectomy: This technique is used for children who are not candidates for endoscopic procedures. An incision is made over the top of the head from ear to ear and the scalp is moved forward. A strip of bone encasing the fused suture approximately two inches wide is removed from behind the "soft spot" to the back of the head. The child will spend the first night in the intensive care unit and moved to a regular room the next morning. Children are discharged on the third or fourth day if they are feeding well.

Frontal-Orbital Advancement (FOA): This procedure is recommended for older infants with severe metopic synostosis or coronal synostosis. An incision is made over the top of the scalp from ear to ear. The bone of the skull is removed from above the eyes to behind the forehead. The bone is reformed and then replaced.

Children spend the first night in the intensive care unit. If the blood counts are stable the child is moved to a regular room the following day. Their eyes will become swollen over the first 12 to 24 hours and may be swollen shut for two to three days. As soon as the eyes are open, the blood counts are stable, there is no fever and the child is eating well they may go home. The length of hospital stay is usually five to seven days.

Frontal-Occipital Reversal (FOR): This procedure is advised for children 6 months of age or older who have fusion of the sagittal suture or have multiple sutures that are fused. An incision is made over the top of the scalp from ear to ear. The scalp is drawn back and the skull bone is removed from above the eyes to the back of the head. The bone is divided into several pieces, which are reshaped and then replaced over the crown of the head. Tiny plates and screws are used to fix the bones into proper position. These children spend the first night in the intensive care unit. Significant swelling is expected around the face and eyes, which lasts for several days.

Before surgery, your child will be scheduled for an exam with the Craniofacial Center team, including neurology and plastic surgery. To help us prepare for a successful surgery, share with us your child's full medical history, including any:

  • Bleeding disorders within the family
  • Medications your child is currently taking
  • Any allergies to medication, foods, latex (rubber) or the environment

Also make sure to let us know of any unexplained problems with surgery, watery eyes, sneezing or wheezing while playing with balloons may be due to a latex allergy. Anyone who has had multiple surgical procedures might have such an allergy.

Schedule a Tour

When your find out that your child is scheduled to have surgery, contact Cincinnati Children's to schedule a tour for an important preoperative program to help you and your child prepare for surgery. Call 513-636-8298 to make reservations for this educational program.

Day of Surgery

On the day of the procedure, a doctor called an anesthesiologist will discuss with you methods of pain control appropriate to your child's size and age.

The anesthesiologist will obtain a thorough history of your child's past anesthetics as well as family reactions to general anesthesia. On the day of the surgery, please be prepared to offer this information; it is very important in preventing possible reactions to anesthetic agents that may be used during the surgery.

Before coming to the hospital, please read the pre-operation checklist. If your child is old enough to understand, please explain what will happen before, during and after surgery using familiar terms.

Pre-Operation Checklist

  1. Mark your calendar clearly with the date, time and arrival time for your child's surgery. Surgery for patients who arrive late may have to be rescheduled. Please make arrangements for reliable transportation.
  2. Be sure you understand the eating and drinking instructions. If you don't follow these directions, surgery may be postponed.
  3. Inform us as soon as possible if your child develops a rash, fever, flu, cold or diarrhea or has been exposed to any communicable diseases like chicken pox, measles, mumps, etc. Surgery may have to be postponed if exposure was recent.
  4. To avoid potential bleeding complications, do not give your child aspirin, Motrin, Advil or ibuprofen for two weeks prior to surgery, except as prescribed by your child's surgeon. If, by mistake, your child does take any of the medications listed, please let us know; because these medicines interfere with the blood's ability to clot, your child's surgery may have to be rescheduled. Your child may take Tylenol, an aspirin-free medicine, for pain or fever reduction.
  5. Once your child's surgery has been scheduled, schedule a pre-operative physical with your pediatrician or family doctor for one to three days prior to surgery. This ensures that your child has no illnesses that may complicate surgery or anesthesia, and it also allows your child's doctor to know when your child is having surgery. The doctor must complete the preoperative history and physical form that you received when you and your child last visited us in neurosurgery. Bring this completed form with you on the day of your child's surgery.
  6. On the day before surgery, a nurse or technologist will take a sample of your child's blood for a complete assessment. You must take the Pre-operative blood work form with you. Appointments are not necessary. The hours of operation are Monday - Friday, 7 am to 8:30 pm and Saturday 8:30 am to 4:30 pm. If you have questions, please call the blood center at 513-636-4461. Blood transfusions routinely are necessary for children who have surgery for craniosynostosis. If your child's neurosurgeon has told you that your child will need a blood transfusion for surgery, you have the option of using the blood provided through Hoxworth Blood Center or of donating through Hoxworth's Directed Donor Program. Hoxworth believes that the blood obtained through regular donations is as safe as blood secured through directed donors. This is especially true if the directed donor is not the parent or immediate relative. Women who have given birth within the past six months cannot donate blood. If you are interested in the Directed Donor program, please call Hoxworth at 513-451-0910 or 800-830-1091.
  7. On the morning of the surgery, have your  child shower with soap and water and wash his/her hair with baby shampoo.  
  8. Arrive at the hospital one hour and a half before the scheduled time of surgery and bring your child's overnight bag, the form completed by your child's pediatrician and your insurance card. If you plan on spending the night with your child while he or she recovers with us, remember your own overnight bag.

Children Younger than 12 Months

  • Stop solid baby foods, cereal and formula six hours before surgery, and restrict your child's diet to clear liquids
  • Stop clear liquids and breastfeeding four hours before surgery

Children Older than 12 Months

  • Stop solid foods, milk, juices, candy and gum after midnight the night before surgery
  • Stop clear liquids four hours before surgery. Clear liquids are fluids you can see through:
    • Water
    • Pedialyte
    • Jell-O
    • Sprite
    • Popsicles
    • Clear broth
    • Breast milk (but not formula)

Afternoon Surgery

If your child is younger than 12 months and is having surgery in the afternoon, he or she may have a light breakfast (cereal, toast or a clear liquid) up to eight hours before surgery. Do not give your child meat or fried or fatty food for breakfast.

Taking Medication

If your child takes daily medication for the heart, asthma or seizures, he or she needs to take the medication the morning of surgery. Your child should take medicine with a small sip of clear liquid. Do not give antibiotics the morning of the surgery as they can cause nausea and vomiting.

If you have questions about your child's medication, please call us at 513-636-4726.

No matter the type of surgery your child has, he or she will go directly to our pediatric intensive care unit (PICU) right after surgery. Your child's head will be fully wrapped with a turban dressing and will be elevated. Sticky pads on your child's chest give readings to monitor the heart. A red light on your child's finger or toe tells if the blood is getting enough oxygen. Through the IV that was placed before the surgery, your child will get needed fluids and medications. Every six hours for up to two days, a blood sample will be taken to make sure your child is recovering as well as expected.

Your child will be sleepy the first day and will be able to take a bottle the evening of the surgery. Two days after surgery the head dressing will be removed.

When the blood counts are stable, your child will move from PICU into a regular room, and you'll be able to hold your child even though the head and face swell after surgery. The worst swelling occurs two to three days after surgery. Often the eyes swell shut, which may frighten your child. Parents' voices and familiar music and sounds help comfort your child.

Going Home

You can take your child home on the fourth or fifth day after surgery. Head and face swelling will be improved, but the swelling around the eyes will come and go for two to three more weeks.

If your child sleeps face down, he or she will awaken with the eye swollen shut. The incision will appear red and swollen. After one or two hours, this swelling will go away. In order to reduce or prevent swelling, we recommend that you put your child to sleep on his or her back for several weeks after surgery. If you notice that he or she rolls over, reposition the child onto his or her back and keep the head up.

Once home, your child may have the days and nights confused. To lessen the effects of this adjustment, provide periods of play during the day in a bright room.

Pain 

If your child is under four years of age, administer regular children's Tylenol as directed for his or her pain and irritability. Children older than four years may be sent home with a prescription for Tylenol with codeine.

Diet 

Once your child is home, resume a regular diet. There are some restrictions immediately following surgery, but since your child will be spending several nights with us, we will restrict the diet as needed during that time.

Wound Care 

You should wash your child's incision each day with a mild shampoo (see home care instructions for a child with cranial incision lines). Do not use cream rinse or any lotions on the head until the skin heals completely.

Cover your child's head when going outside; the incision gets sunburned very easily.

Activity 

Your child may return to usual activity levels when you go home. Remove low-lying furniture with sharp edges such as coffee tables to prevent possible head injuries.

We recommend that you keep your child home from school or daycare until the first follow-up visit.  

Medication 

No medications are prescribed routinely following surgery. If your child complains of pain at the site of the incision, give Tylenol.

Bathing

Your child should shower with mild baby soap or shampoo daily. A sponge bath with mild soap followed by rinsing with clean water is fine for smaller children. Do not allow the wound to soak in the bath tub.

Follow-Up Care

Make sure you have your follow-up clinic appointment scheduled for seven to 10 days after the surgery. Six weeks after surgery your child will have a follow-up appointment with the surgeon. You may also want to schedule other appointments with members of the craniofacial team such as plastic surgery and speech pathology.

These appointments should be scheduled before you take your child home from Cincinnati Children's. If they are not, please call us at 513-636-4726.

If your child has a fever or is showing extreme irritability, especially when you laying down, he or she may have an ear infection. Ear infections are a very common result of surgery for craniosynostosis. Call the doctor at 513-636-4726 if you suspect an ear infection or are concerned with you child's progress after surgery.

Please call if your child experiences:

  • Temperature greater than 101 degrees Fahrenheit
  • Bleeding or drainage from the incision
  • Confusion or excessive sleepiness
  • Severe headache that does not stop with Tylenol and rest
  • Excessive vomiting (when nothing stays down)

Our office hours are 8 am to 4:30 pm Monday-Friday. On evenings and weekends, please call.


Last Updated 09/2012