Craniosynostosis in Babies – Causes, Symptoms, Diagnosis & Treatment

Craniosynostosis is a birth defect in which the bones in a baby’s skull are joined together. This happens when the baby’s head is not completely formed. This condition can cause vision loss and learning issues in babies. There could be various causes of this condition. Read on to know more about craniosynostosis in babies. Learn about its symptoms, causes, diagnosis, and possible treatment.

What Is Craniosynostosis?

Craniosynostosis is a brain defect that leads to the conjoining of the bones in the baby’s skull, way before time. It is a form of a fused skull that forms deformities in the brain because of the joining of bones. This condition develops before the brain is fully formed and can result in further issues in the structure of the brain as it continues to grow.

To understand the changes that take place when craniosynostosis develops in a baby’s brain, you must first understand how the bone structure formation takes place normally in a baby’s brain. The space present between the skull bones in a baby is filled with a certain material called the sutures. These sutures enable the skull of the baby to expand as the baby’s brain also expands. After a certain span of time, roughly 2 years, these sutures turn into bones, and the bones begin to join. This is a critical stage of joining as the ‘sutures’ start closing. Craniosynostosis begins to develop when more than the required number of sutures close down. And when this condition develops, it can have a harmful effect and hamper the normal growth of the baby’s brain. There comes a time when some part of the brain gets closed for further growth as the sutures shut down, and some other part of the brain continues to grow. This would result in an uneven shape of the brain. All this leads to immense pressure in the skull.

How Common Is It?

Usually, 1 in 2,500 babies is said to be affected by this condition in the US.

Craniosynostosis Types

Craniosynostosis can be categorized into different types based on the sutures affected. Let’s understand some common types of craniosynostosis.

1. Sagittal Craniosynostosis

This type develops in the top region of the baby’s head. It extends from the front to the backside of the soft spot region on the baby’s head. If the sutures, especially around this region, close too early, the head grows to be long and narrow in shape. It is one of the most common types.

2. Coronal Craniosynostosis

The region in which this type develops is on either side of the coronal structures, extending from each ear to the top of the head where the sagittal suture is present. In this case, when the suture closes down before time, a flattened forehead is most likely to develop on the side of the skull.

3. Lambdoid Craniosynostosis

This suture is present in the lambdoid suture at the back of the baby’s head. In this case, too, a flattened structure will be formed if the suture closes early; however, it forms at the backside of the head. This, compared to the previous two, is a rare type of craniosynostosis.

4. Metopic Craniosynostosis

The 4th type in this category occurs in the region extending from the baby’s nose and ending in the sagittal suture placed at the top region of the head. In this case, if the suture closes early, the deformity spotted is a triangular shape on the top of the baby’s head. It is narrower towards the front of the head and then broadens out towards the back. This is also a rare type.

Causes of Craniosynostosis in Babies

Read on to understand the possible causes of this condition in babies. These causes can be further categorized into two types: one is non-syndromic craniosynostosis, which is said to be because of environmental and genetic factors, and the second is syndromic craniosynostosis, which is related to a genetic disorder. When the exact cause is not known, it can be a case of non-syndromic craniosynostosis. In the case of syndromic craniosynostosis, it could be caused due to one of the syndromes:

1. Crouzen Syndrome

This occurs in both the coronal sutures fuse at a premature stage.

2. Carpenter Syndrome

This happens during the fusion of the sagittal and coronal sutures.

3. Saethre-Chotzen Syndrome

A fusion of lambdoid and coronal sutures in the premature stage

4. Pfeiffer Syndrome

This includes the premature fusion of sagittal, coronal, and then sagittal again.

Signs and Symptoms of Craniosynostosis in Babies

The symptoms are usually spotted at birth or within a few months. Here is a list of the symptoms of craniosynostosis that parents should look out for:

• The shape of the skull is uneven and not normal.
• The soft spot, on top of the baby’s head, is abnormal or missing.
• A hardened edge will be spotted along the suture region, which has close way before time.
• The baby’s head will have abnormal growth.
• Depending on the type of brain defect, the baby might have headaches. The eye sockets might be narrowed or widened. The baby may also have learning difficulties and may experience loss of vision.

Complications

If the symptoms are left unidentified and the condition is not treated on time, there might be certain complications, such as:

• Facial deformation may become permanent.
• The baby’s head may be permanently deformed.
• Such deformities might lead to poor self-esteem.
• It might break your confidence and propel you towards social isolation.

Certain pressure in the intracranial region might develop because of the smaller size of the skull, even though the brain might continue to grow. This would give rise to further complications, such as:

• Feeling lazy
• Delayed responses to development
• Unforeseen seizures
• Various eye problems
• Severe impairment of cognitive abilities

Craniosynostosis Diagnosis

If you suspect that your baby might have this condition, you must check with a doctor. The doctor will diagnose the condition and provide the right treatment. The condition may be diagnosed by physical examination and tests.

1. Physical Examination

To diagnose the condition, a paediatrician will touch your baby’s head and try to feel for the ridges in the sutures around the skull.

2. Tests

Computerized tomography (CT) scan and magnetic resonance imaging (MRI) will help in the identification of the skull’s shape and the visualizing of the developed sutures.

3. Genetic Tests

Genetic tests will be done in case the baby shows the symptoms of a genetic syndrome.

How Is Craniosynostosis Treated?

After the condition is diagnosed, the doctor may suggest the following treatment options for the same.

1. Endoscopic Surgery

This procedure is used for babies who are less than 3 months of age, but maybe considered for babies who are 6 months old in case of one suture. Small incisions are made on the scalp to remove sutures. The baby will be required to wear a special helmet for about a year to reshape his skull.

2. Open Surgery

This type is for babies older than 6 months and up until they turn 11 months of age. The bones are removed from the affected area of the skull, reshaped, and placed back. This process causes more blood loss and the recovery period for this is longer as compared to endoscopic surgery.

Craniosynostosis is a condition that can affect any child. The only thing new parents can do is keep an eye out for the symptoms and consult a doctor at the earliest. Early intervention can help avoid complications and keep the baby safe and healthy!

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