Trigonocephaly: Causes, Symptoms, Diagnosis and Treatment

Did you know that your baby’s skull is not a unique and solid entity? It is made of several bony plates; there are fibrous joints called sutures between these plates.

Usually, sutures in the skull of a developing baby are fused in a gradual process over time. However, sometimes the fusion happens too early. This is a medical problem known as craniosynostosis.

One type of craniosynostosis is called metopic synostosis (trigonocephaly or craniosynostosis of a metopic suture).

A prominent ridge along the forehead is often a normal finding. Still, children with metopic synostosis due to premature fusion of the metopic suture have a triangular shape on the forehead.

To summarize meta topic synostosis:

  • They have a grand ridge along their foreheads.
  • They will also have a pointed, almost triangular shape on the front and top of their skulls and eyes that look very close together.
  • As with any other type of craniosynostosis, the meta topic synostosis may risk other complications.

However, it is essential to remember that each child is different and that the condition can vary widely in its severity.

Here are some more important facts about the metatopic synostosis:

 

  • Metopic synostosis can be pretty mild in some children and quite severe in others.
  • There is no single proven cause for metopic synostosis.
  • Metopic synostosis is often noted at birth, but it may also become evident in older babies.
  • Metopic synostosis and other types of craniosynostosis should not be confused with plagiocephaly, a different condition associated with the baby’s position during sleep.
  • Children with more severe cases of metopic synostosis may experience vision, learning, or behavior problems.
  • Some children with only a mild metopic crest or synostosis do not need medical treatment. For those who do, surgery has proven to be a successful approach.

What causes the metopic synostosis?

In most children, the metatopic synovectomy occurs without any identifiable reason. Sometimes, however, the metatopic synostosis occurs as a component of a rare genetic syndrome.

The following disorders have been linked to trigonocephaly:

  • Baller-Gerold syndrome also causes abnormalities in the bones of the arms and hands.
  • Jacobsen syndrome results from the loss of material within a particular chromosome.
  • Muenke syndrome is caused by a mutation in the gene that produces a protein responsible for the health of the brain and bone tissue.
  • Opitz syndrome causes several congenital disabilities that affect the face, heart, and larynx.
  • Say-Meyer syndrome is characterized by delays in development, problems with motor skills, and short stature.

What are the symptoms of trigonocephaly?

Children with trigonocephaly have visible symptoms that include one or all of the following:

  • A grand ridge runs down the middle of the forehead.
  • A triangular shape too narrow on the forehead and the top of the skull.
  • Eyes that look very close together.

Diagnosis

Children with meta topic synostosis usually show visible symptoms at birth, namely:

  • A ridge is running down the front.
  • A narrow and triangular shape on the forehead and the top of the skull.
  • Sometimes, eyes are spaced very close together.
  • A child’s metopic synostosis is diagnosed later in childhood during a routine physical examination.

Once an initial finding of metopic synostosis is made, your doctor can take the following steps to confirm the diagnosis:

  • Obtain a complete medical history of the family.
  • Determine if your child has developmental delays or other problems to meet milestones appropriate for their age.
  • You measure the circumference of your child’s head and plot these measurements on a graph to detect unusual patterns.
  • Order a computed tomography (CT) scan, a non-invasive procedure that uses X-ray equipment and powerful computers to create detailed skull images.

Treatment

Not always necessary, a child with a mild meta topic synostosis may have no symptoms beyond a noticeable ridge in the middle of the forehead. In these cases, doctors may decide that medical treatment is not needed.

Surgery

Many children with moderate to severe metastatic synostosis will require surgical intervention.

It is designed to correct deformities in the facial and cranial bones and often works better in children under one year since the bones are still soft and flexible, which makes them easier to handle.

It can be recommended in much younger infants, including newborns. Depending on the severity of their symptoms, it can also be performed in older children (although the older the child, the more complex the surgery).

It is usually completed after a single surgery (but in some cases, it may be necessary to repeat it over time, depending on the severity of the condition).

Before the operation:

  • Explain the reasons to consider surgery.
  • Explain the steps involved in the process.
  • Review aftercare instructions, including warning signs of possible complications.
During surgery, the surgeon and the treatment team:
  • In the case of minimally invasive surgery, use an endoscopy, a long, thin tube with complementary light, and a camera to release the sutures that are abnormally fused.
  • In the case of reconstructive surgery, reposition the bones of the skull to allow average growth and development.

Monitoring and follow-up

After the surgery, your child:

  • You will stay in the hospital for one to five days.
  • You may have visible swelling from a few days to a few weeks.
  • You may need to use a bandage covering your head, protecting the incision site from contamination and infection.
  • You will have to return for checkups and evaluations to measure how well the skull, facial bones, and brain development are.