Have you noticed anything different about the shape of your little one's head, especially the forehead, perhaps a triangular shape? It's normal for a mother or father to feel a little worried when they see something like that. To answer the questions that arise in your mind at such times, today we are going to talk about a condition called Metopic Craniosynostosis. Sometimes this is also called Trigonocephaly.
What is Metopic Craniosynostosis?
Simply put, metopic craniosynostosis is a birth defect . It occurs when the two frontal bones in a baby's head, or skull, fuse together prematurely (premature fusion), either in the womb or at birth. Think about it, our skull is not made of a single bone, but rather several pieces of bone. These pieces of bone are connected to each other by something called "sutures." These sutures are like the places where bones grow. As the baby's brain grows, these sutures help the skull grow to accommodate it.
Now, in the case of metopic craniosynostosis, the seam between the two frontal bones, called the "metopic suture," closes before the baby's brain is fully developed. This is when the baby's forehead takes on a triangular shape. This is also called trigonocephaly.
How common is this condition?
Recent studies suggest that metopic craniosynostosis may be the second most common type of craniosynostosis. However, the incidence varies from country to country. In countries like the United States, it is estimated that the condition affects anywhere from one in 700 to one in 15,000 newborns.
What are the symptoms of this?
A baby with metopic craniosynostosis may exhibit the following symptoms:
- Abnormal growth of the baby's head and facial bones .
- The brain is pushed inside the baby's skull, which means increased intracranial pressure .
- Vision problems .
How does the baby's appearance change?
This condition affects the way the baby's facial features develop. It can look like this:
- It has a narrow, pointed, triangular forehead .
- The eyes appear to be close together .
- It looks like my eyebrows are raised .
- The back of the head appears wide .
Imagine, it looks like the baby's forehead is protruding from the middle.
Why is this happening? What are the reasons?
The main cause of metopic craniosynostosis is the fusion of the frontal bones before the brain is fully developed . That is, they fuse prematurely. But doctors still have not been able to find an exact reason for why these bones fuse so quickly. However, research suggests that several factors may play a role:
- Genetic changes (for example, genetic mutations of the FREM1 and SMAD6 genes).
- As a side effect of certain medications taken by the mother during pregnancy (e.g., medications such as valproic acid and clomiphene citrate).
- Mother has thyroid disease .
- The position of the fetus in the uterus .
- As a symptom of another genetic condition, such as Williams syndrome.
Who is most at risk for this?
Your risk of having a child with metopic craniosynostosis may increase if you:
- If you are expecting twins .
- If you have an underlying health condition such as thyroid disease or diabetes mellitus .
- If you take certain medications for a disease like epilepsy.
- If you use tobacco products .
To learn more about the risk of having a baby with a birth defect, see your doctor for preconception counseling. You can get advice that can reduce your risk of having a baby with a birth defect .
What complications can this cause?
Complications of metopic craniosynostosis may not be apparent until the child is older and starts school. The most common complications are:
- Developmental delays (speech and language or motor skills).
- Cognitive development problems .
- Behavioral changes (like irritability).
- Signs of increased intracranial pressure ( including headaches and vision issues).
- The head grows slowly compared to other parts of the body.
How do doctors diagnose this?
Sometimes, a doctor may suspect this during a prenatal ultrasound scan during the second and third trimesters of pregnancy. However, most often, the condition is diagnosed during a newborn physical exam after the baby is born .
The doctor will carefully examine the baby's head from the top (vertex) to the side of the face. If, when viewed from a bird's eye view, the baby's head takes on a triangular or conical shape instead of a round shape, it can be diagnosed as a condition called trigonocephaly.
Tests such as X-rays or CT scans (Computed Tomography - CT scan) are also done to learn more about the shape of the baby's skull bones. The information obtained from these tests helps in planning treatment.
How serious can this situation be?
The severity of metopic craniosynostosis can vary from person to person. The severity is determined by the size of the angle that is created by the rapid fusion of the skull bones. For example:
- In mild cases, there is only a slight change in the shape of the forehead.
- In severe cases, the forehead becomes very pointed, taking on a triangular or conical shape.
- A moderate opportunity lies between these two.
How is this treated?
If metopic craniosynostosis is mild , or if there is only a protruding metopic ridge without significant forehead deformity, medical treatment may not be necessary .
However, if the trigonocephaly is moderate or severe, surgery can be performed to reshape the baby's head and provide the brain with the space it needs to grow . If necessary, surgery can also reduce pressure on the brain.
There are two main types of surgery to treat metopic craniosynostosis:
1. Endoscopic-assisted surgery:
- This surgery is performed on young babies, usually before 4 months of age, and a maximum of 6 months .
- The surgeon makes a small incision on the baby's forehead, where the hairline begins.
- Then, using an endoscope (a thin tube with a camera), a strip of bone above the metopic suture is removed. This endoscope allows the surgeon to see inside the skull and perform the surgery without making a large incision.
2. Open surgery (Open surgery - Cranial Vault Remodeling):
- This surgery is usually performed on babies between 6 and 12 months of age .
- Most of the time, this is done by a neurosurgeon and a plastic surgeon working together.
- The surgeon makes a larger incision in the baby's scalp than in endoscopic surgery .
- Then, the abnormally shaped parts of the skull are removed. Next, the bones are cut, reshaped, and reattached to create the correct head shape . This allows more room for the baby's brain to grow.
Does the baby need to wear a helmet after surgery?
After endoscopic surgery, your baby will need to wear a special helmet until they are about 12 months old. This helmet helps the baby's skull develop into a round shape. A helmet is usually not needed after open surgery , but your surgeon will advise you about it.
Are there any side effects of the treatments?
As with any surgery, there are several risks and side effects that can occur with both of these surgeries:
- Blood loss .
- Infection .
- A brain hemorrhage (Hematoma) .
Your child's surgeon will explain these side effects to you before surgery. Open surgery carries higher risks than endoscopic surgery . Also, the time it takes for surgery and recovery is longer.
Sometimes, if there is bleeding after surgery, the child may need to receive blood transfusions . This is most often seen in open surgeries.
It is uncommon for trigonocephaly to require further surgery to correct the condition, but it may be necessary.
How long does it take to recover after surgery?
Your child may feel much better within a few weeks of surgery. However, it may take two to three months to fully recover . Bone healing and regrowth may take up to a year. Your child's surgeon will talk to you about recovery time and what to expect before surgery.
If my child has metopic craniosynostosis, what should I expect?
Although metopic craniosynostosis is a rare condition, the way it affects each child can vary. Some children may have only very minor changes in head shape and may not need treatment . Others may need surgery to correct the shape of the head and treatment for complications such as developmental delays.
Surgical treatment is successful, and side effects are usually minimal. After endoscopic surgery, the child is kept in the hospital overnight and carefully monitored. They can usually go home within a day or two. With open surgery, the child is kept in the intensive care unit for 24 hours and is monitored by doctors around the clock. After that, they are transferred to another ward of the hospital for a few days or a week to monitor their recovery.
During the hospital stay, the child's head and face may swell. This is normal. The eyes may be swollen and closed for two to three days. After that, the swelling will gradually go down.
Children who have had endoscopic surgery must wear a helmet until they are 12 months old, which can be a bit of a challenge for new parents. Because their child will look different from others until they heal. Many parents decorate the helmet with cute stickers to match their child's personality.
Because your baby looks different, people may ask questions about it. You may find it difficult to share these details with everyone. Many families have found comfort in talking to a mental health professional at times like these.
Can this situation be prevented?
There is currently no way to prevent metopic craniosynostosis. However, you can reduce your risk by talking to your doctor about how to stay healthy before and during pregnancy and ways to prevent birth defects.
When should my child see a doctor?
Once your child is fully recovered, a team of specialists (craniofacial team) will monitor him or her until he or she is older.
If your child is late in reaching developmental milestones appropriate for their age , see a doctor right away. For example, walking or speaking their first words. Although most children with metopic craniosynostosis develop normally, some children may have developmental delays. They may need a little more time than others. A doctor can help your child reach their goals.
After surgery, your surgeon will give you information about things to watch out for, such as infection. If you notice anything unusual, contact your doctor immediately.
What questions should I ask my child's doctor?
- Does my child need surgery?
- What kind of surgery do you recommend?
- Are there any side effects of the treatment?
- When should I come back for check-ups after surgery?
- What do I do if my child is late in reaching developmental milestones?
It can be a shock to see your newborn baby's head shape look different than expected. But don't worry. As soon as your baby is born, your medical team will diagnose metopic craniosynostosis and treat it to prevent complications that could affect your baby's growth and development. Surgery will help your baby's brain and skull develop properly.
Summary (Take-Home Message)
If you notice your baby's forehead taking on a triangular shape, it could be a sign that the metopic suture has closed prematurely. This is called metopic craniosynostosis or trigonocephaly .
Don't worry! If the condition is mild, treatment may not be necessary. If it is more severe, surgery can be performed to correct the shape of the head and allow the brain to grow properly. The surgery is successful, and children recover quickly. You may need to wear a special helmet for a while after the surgery.
The most important thing is to follow the doctor's instructions carefully and to stay in touch with your child's development . If you have any questions or doubts, don't hesitate to ask the doctor. We all want your child to grow up healthy and happy!
` Metopic craniosynostosis, trigonocephaly, infantile skull, forehead shape, birth defects, brain development, surgery
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