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Craniofacial Program

Metopic Synostosis

What is Metopic Synostosis?

It is the premature fusion of the suture in the middle of the forehead called the metopic suture. The estimated prevalence is 1 in 15,000 live births with a 3:1 male:female ratio. A positive family history is obtained in approximately 5 % of patients.

What Does It Look Like?

synostosis-met1 image

Premature fusion of this suture results in trigonocephaly which is Greek for "triangular shaped" forehead. When viewed from above, the front of the head has the appearance of the bow of a ship. The suture is usually represented by an unsightly ridge down the center of the forehead. This synostosis will also cause the eyes to be spaced closely together (hypotelorism) with some upslanting of the outer corners (upslanted palprabral fissures).

 

 

 



Does Metopic Synostosis Affect the Brain?

Craniosynostosis is known to be a cause of increased intracranial pressure and children with one prematurely fused suture, such as metopic synostosis, demostrate elevated intracranial pressure in >14% of cases. It is for this reason 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.

Children with metopic synostosis usually fall into one of three categories.

  1. No other malformations are present and normal development is expected.
  2. Other malformations such as hypotonia (low tone) and or developmental delay can be or is documented shortly after birth. However no syndrome can be attributed to these findings.
  3. A known syndrome can be identified and cognitive and behavioural development is expected to follow the characteristics of the syndrome.

It is for the above reasons that a full Craniofacial Team assessment is indicated and warranted.

Does My Child Need X-Rays?

synostosis-met2 image

Typically, most children do not need x-rays to diagnose metopic 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:

  1. Verify the diagnosis of metopic synostosis.
  2. Assess brain growth and development.
  3. Evaluate if there is evidence of increased intracranial pressure.
  4. Assist in surgical planning, if indicated.

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. A plan will be formulated that will involve you and your child 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 preoperative planning with you 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 has 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 childs preoperative work up are 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 to Have 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 very 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.

What Does Surgery Entail?

In the moderate to severe cases surgical correction with a Cranio Orbital Reconstruction (approximately 6 hours in length) is indicated. This surgery involves the reconstruction of the frontal bones (forehead) and of the upper orbits (supra orbital rims) into a more aesthetically and functionally correct position.

Via a coronal incision, the skull bones are cut and separated from the brain and reshaped. The synostosed portion of bone is removed and the forehead and the upper bone portions of the eye sockets (supra orbital rim) are reconstructed and moved forward. Bone pieces are held in place with plates and screws (usually bio-resorbable ones). Usually there is not enough bone to reconstruct the entire surgical area and several ‘soft spots’ remain which will heal spontaneously over time

  
CT scan and picture of child after surgery for metopic synostosis

Blood Transfusion. Is It Necessary? Can We Donate?

Your child will require 2 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

  1. Compatible with your child
  2. Healthy
  3. 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.

After Surgery

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.