Craniosynostosis

Medical specialist	Pediatrician, pediatric neurosurgeon, reconstructive plastic surgeon
Symptom	The fontanel is not felt, the shape of the head is abnormal, and the development of the head is not in accordance with the age
risk factor	Mother with thyroid disease, a history of taking certain drugs
How to diagnose	Medical interviews and supporting examinations (genetic tests, imaging tests such as X-rays of the head, CT scan, MRI)
Treatment	Helmet use, surgery
Complications	Developmental and cognitive disorders, abnormal head shape, visual disturbances, increased intracranial pressure
When to see a doctor?	Immediately when there is a change in head shape, or inappropriate head development, the fontanel is not palpable

Understanding

Craniosynostosis is a congenital disorder that occurs in the baby’s skull.

In craniosynostosis, the skull bones close too quickly, causing the baby’s head to develop abnormally or underdeveloped.

The human skull is basically made up of several bones, where the bones are separated by sutures, which are strong elastic tissues.

During the growth period, sutures are needed so that the skull is flexible following the development of the baby’s brain.

Now, as development progresses, the sutures will close completely and in adults produce hard skull bones.

Unfortunately, this does not happen to babies with craniosynostosis.

Meanwhile,  craniosynostosis is divided into several types, depending on which type of suture is involved in the rapid closure of the skull bones.

The types of  craniosynostosis  are:

1. Metopic

The metopic suture extends from the nasal passages through the midline of the forehead to the crown and is connected to the sagittal suture at the top of the skull.

This accelerated the closing of the metopic suture causes the forehead to form a triangle in which the back of the head widens.

2. Sagittal

Sagittal craniosynostosis is the most common type of craniosynostosis.

In this condition, the sagittal suture that runs from the front to the back of the upper skull closes too quickly. As a result, the head grows elongated and flat.

3. Coronal

The coronal suture runs from the right and left ears to the top of the skull.

When this suture closes too quickly, it will result in an unbalanced shape of the forehead, such as uneven and protruding parts.

4. Lambdoid

This type of craniosynostosis is the least common. The lambdoid suture runs from right to left at the back of the skull.

The occurrence of too-fast closing of these sutures causes some sides of the head to look flat.

In some cases, the position of one ear will also appear higher.

Reason 

The cause of craniosynostosis is not yet known with certainty. However, on the other hand, this condition is thought to be related to genetic disorders.

The causes of  craniosynostosis  are generally divided into two, namely:

1. Non-syndromic Craniosynostosis

In this condition,  craniosynostosis has no known cause. It is suspected that craniosynostosis occurs due to genetic and environmental factors.

2. Craniosynostosis syndrome

Craniosynostosis syndrome is caused by various genetic disorders, for example, Apert Syndrome, Crouzon Syndrome, Pfeiffer Syndrome, and Saethre-Chotzen.

3. Risk Factors for Craniosynostosis

Based on reports from the CDC, there are several risk factors that increase the occurrence of  craniosynostosis,  including:

4. Mothers with Thyroid Disease

Pregnant women who have thyroid disease are at higher risk of giving birth to babies with craniosynostosis.

5. Medicines Consumed by Mother

Women who take the drug clomiphene citrate (a fertility drug) before or early in pregnancy have a greater chance of having a baby with craniosynostosis.

Symptom

Signs of craniosynostosis are generally visible when the baby is born and are increasingly evident in the several months of life.

Some of the symptoms  of craniosynostosis  that arise include:

• The fontanel on the baby’s head is not felt
• The growth of the baby’s head is not proportional to the growth of his body
• Abnormal head shape, such as being elongated, looking flat, or flat on one side
• Increased intracranial pressure or pressure within the skull. For example,  headaches, sudden vomiting, visual disturbances, and fuss

Diagnosis

In the process, the diagnosis of craniosynostosis is not only handled by pediatricians.

Collaboration with other specialist doctors is also needed, such as pediatric neurosurgery and reconstructive plastic surgery.

The doctor will carry out a series of examinations, ranging from medical interviews to supporting examinations to find any abnormal head shape. Here’s the explanation.

1. Physical Examination

A physical examination is carried out to see the condition of the baby. The doctor will measure the baby’s head and feel the sutures and fontanel.

2. Scan Examination

Follow-up examinations, such as a CT scan, MRI, or x-rays, are also carried out to confirm the diagnosis of craniosynostosis.

This aims to see the suture network.

3. Genetic Test

Genetic tests will also be carried out if there is a suspicion of a genetic abnormality that is the cause of this condition.

Treatment

Treatment of cases of craniosynostosis depends on the degree of severity. Mild or moderate degrees of craniosynostosis generally do not require special treatment.

Generally, doctors will recommend using a special helmet to help improve the shape of the skull bones and help brain development.

Meanwhile, in severe conditions,  craniosynostosis is treated surgically.

Through the surgical process, it is hoped that the shape of the skull bones can be corrected while reducing and preventing pressure on the brain.

The type of surgery performed depends on the type of craniosynostosis and the underlying genetic syndrome.

However, some cases of this skull bone disorder often require more than one surgery.

Surgery that is usually recommended to treat craniosynostosis  includes  :

1. Endoscopic Surgery

The endoscopic surgical method is a minimally invasive procedure and is generally performed on babies under six months of age.

Because it is minimally invasive, only small incisions or surgical wounds are performed. In general, this procedure requires short treatment and usually does not require a blood transfusion.

On the other hand, the weakness of this endoscopic surgery is that it is only performed on babies who have one problematic suture.

2. Open Surgery

Meanwhile, the open surgical method is performed on babies over six months old.

This surgery can help remove problematic sutures and correct disproportionate skull bones.

Open surgery generally requires a longer treatment time and usually requires blood transfusions.

In severe cases, the doctor may suggest several surgical procedures to help improve the shape of the baby’s head.

Prevention

Craniosynostosis can’t be prevented.

However, with routine checks during pregnancy, this condition can be detected early, especially if there is a history of births with craniosynostosis in the family.

Genetic testing during pregnancy can be considered to help determine the risk of having a baby with craniosynostosis.

Apart from carrying out routine antenatal care during pregnancy, make sure that pregnant women also regularly take pregnancy supplements given by doctors.

Don’t forget to also consult further regarding your risk factors, for example having thyroid disease.

Complications

Craniosynostosis  that is not handled properly will cause complications, such as:

• Permanent abnormal shape of the head and face
• Decreased self-confidence and alienated from society
• Increased intracranial pressure (pressure in the cavity of the head)
• Growth and development disorders
• Cognitive impairment
• seizures
• Headache
• Blindness
• Impaired vision

When to See a Doctor?

Immediately consult a doctor further if the baby:

• There is a change in the shape of the head
• The child’s head development is not appropriate
• Signs appear, such as the baby’s fontanel is no longer palpable and symptoms of increased intracranial (vomiting bursts suddenly, the baby tends to be fussy or weak, to convulsions)