WARNING: This page of the SickKids web site contains graphic images of surgical procedures.
What is Sagittal Synostosis?
It is the most common type of premature suture fusion approximately 1 in every 5000 births with a 3:1 male:female ratio. Sagittal synostosis is the premature closure of the sagittal suture.
Why is It Called Sagittal Synostosis or Scaphocephaly?
The premature fusion causes a characteristic scaphocephaly which is Greek for "Keel shaped" or boat shaped skull.
What Does Sagittal Synostosis Look Like?
You will notice that your child's head shape will be typically narrow from side to side and lengthened from front to back. The sagittal suture itself may be easily visible in the newborn as a prominent bony ridge extending from the 'soft spot' to the back of the head. Also, children with this type of synostosis will often have a very prominent forehead and occipital (back of the head) region.
child with sagittal synostosis
Does Sagittal Synostosis Affect the Brain?
This form of craniosynostosis is rarely associated with increased intracranial pressure and is thus of little threat to normal brain growth and development . However, your child should be assessed by a team of specialists particularly those involved in the fields of Neuro-Ophthalmology, Neurosurgery and Craniofacial Surgery to test and evaluate whether increased intracranial pressure is a concern your child's case or not.
Does My Child Need X-Rays?
Typically, most children do not need x-rays to diagnose sagittal synostosis. A skilled clinician should be able to diagnose this condition with careful assessment of the skull. However, a CT-Scan with 3D reconstruction will be used to:
- Verify the diagnosis of sagittal synostosis.
- Assess brain growth and development.
- Evaluate if there is evidence of increase intracranial pressure
- Assist in surgical planning if indicated.
CT scans of a child with sagittal synostosis
What Happens in the Craniofacial Clinic?
You and your child will meet the Craniofacial Surgeon and the Nurse Coordinator. The Craniofacial Surgeon will confirm or establish diagnosis and discuss the options available to you. Then, a plan will be formulated that will involve you meeting specific members of the team including but not limited to Genetics, Neurosurgery, Neuro ophthalmology, Occupational Therapy and Social Work. A CT Scan will also be arranged. Once these assessments have taken place you will once again meet with the Craniofacial Surgeon who will discuss any further questions or findings, outline surgical options and expectations and/or discuss conservative management.
Once a decision for surgery has been made then the Nurse Coordinator will discuss with you the preoperative planning including preoperative physical assessments, bloodwork, directed blood donation, length of stay, ICU stay, pain management, preoperative preparation of your child and programs available, expected post operative recovery course and follow up. These will be discussed with you in clinic and once again when a surgical date has been secured with the Craniofacial Surgeon and the corresponding Neurosurgeon that your child had seen.
The Nurse coordinator will liase with any and all services both inside and outside of the hospital to ensure that all aspects of your child's preoperative work up is completed and incorporated in the plan. The Nurse Coordinator will also be able to help you connect with other families who have recently gone through similar circumstances and surgery after their child was diagnosed with this type of synostosis.
Will My Child Need Surgery?
The decision for surgery is based on severity of the synostosis, the findings of the interdisciplinary team and parental involvement. For the more severe cases surgery is almost always indicated. For mild to moderate cases surgical management and treatment is offered. For the cases that are considered mild often conservative (no treatment) management with yearly follow up is indicated. All families are offered the option of surgical management and treatment. The team is always available should families wish to revisit surgical correction in the future if and when their child's head asymmetry becomes problematic particularly from a psychological and or social perspective.
Surgery With Diagnosis Before 6 Months of Age
There is a close relationship between skull growth and facial growth. Children with abnormal skull growth will have abnormal facial growth. Since the molding and shaping of the skull is directly dependent on the growth of the brain the best cosmetic and functional results are obtained with early surgery. Surgical correction of this type of synostosis is best performed at 4-6 months of age with an extended strip craniectomy and subsequent molding helmet therapy. The extended strip craniectomy involves the removal of the fused sagittal suture and the placement of cuts along the bones of the skull to allow for appropriate brain growth. The surgery is generally 5 hours in length. Approximately 7-10 days following surgery the child will be fitted for a customized molding helmet. Molding helmet therapy ensures that the skull will mold into a more symmetrical and aesthetic shape. The molding helmet is custom fitted to the shape of the new skull and is designed to do two things. Firstly, it will act as a protective cushion to prevent undue pressure on any one side of the head and allow for better distribution of the pressure along the bones of the skull. Secondly, the helmet is fitted in such a way as to allow for the bones of the skull to expand in the previously constricted areas in response to the natural growth of the brain.
Molding helmet therapy following extended strip craniectomy generally lasts 4-6 months. During this time the molding helmet is being modified, to allow for the constant and continued growth of the brain and skull, every three weeks. The molding helmet is to be worn 23 hours a day with an hour a day allotted for washing, incision line assessment/care and break.
Click here to learn more about molding helmets
Surgery With Diagnosis After 6 Months of Age
With diagnosis after 6 months of age the surgical correction of sagittal synostosis involves a procedure called a Total Cranial Vault Reshaping. As it sounds it is a more complex and lengthy (8-9.5 hours) surgery than the strip craniectomy. This surgery involves the removal and reconstruction of the bones in the front, back and sides of the skull. Bone pieces are held in place with plates and screws (usually bio-resorbable ones). Generally, this surgery is performed between 10-12 months of age when children are old and strong enough to undergo this procedure. It is during this time frame that the bones of the skull are thick enough and yet still malleable to reconstructive efforts. Usually, there is not enough bone to reconstruct the entire skull and several "soft spots"' remain. These will heal spontaneously over time. The brain is still growing rapidly and any holes/defects left after the reconstruction will be filled in naturally by new bone being laid down. This phenomenon takes place until approximately 18-24 months of age.
For those children older than 2 years of age a second procedure is often needed to address any bony defects left after a Total Cranial Vault Reshaping.
Blood Transfusion. Is It Necessary? Can We Donate?
Your child will require 2-4 units of blood to be available for this surgery. You will have the opportunity to be a directed donor of your blood if you or your spouse are
- Compatible with your child
- Meet the criteria of the Canadian Blood Services.
If you are not we will utilize the blood from the volunteer blood supply from The Canadian Blood Services. The Nurse Coordinator will speak to you more about this and the process involved in getting screened and tested and what is required to donate blood for your child. Once donated, blood has a shelf life of approximately 35 days. Thus most parents will be donating units within one and two weeks of the surgical date
Following surgery, infants and children generally stay overnight in our Pediatric Intensive Care Unit. From there they will be transferred the following day to the Plastic Surgery Unit (8C) where they will generally stay for 3-5 days. Before discharge all children will have a postoperative CT Scan. Children and families will then be followed up by the Craniofacial Surgeon in the Craniofacial Clinics one week after discharge.
x months after extended strip craniectomy