Craniosynostosis - Symptoms, causes and treatment

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Craniosynostosis - Symptoms, causes and treatment
Craniosynostosis - Symptoms, causes and treatment
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Craniosynostosis is a birth defect when the fontanel closes too quickly. As a result, the baby's head develops abnormally and causes the baby's head to look imperfect

At first, the skull bone is not a single whole bone that stands alone, but a combination of several bones connected by the crown. The crown will remain open until the baby is 2 years old so that the baby's brain can develop. After that, the crown will close and form a solid skull bone.

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In babies with craniosynostosis, the fontanel closes faster before the baby's brain is fully formed. This condition makes the brain push the skull bone so that the shape of the baby's head becomes disproportionate.

If left untreated, craniosynostosis can cause permanent changes in the shape of the head and face. The pressure in the cavity of the head can increase and lead to serious conditions, from blindness to death.

Causes and Risk Factors of Craniosynostosis

It is not known what causes craniosynostosis, but this condition is thought to be related to genetic and environmental factors. Craniosynostosis is also thought to be triggered by conditions that can affect a child's skull development, such as Apert syndrome, Pfeiffer syndrome and Crouzon syndrome.

The risk of having a baby with craniosynostosis is higher in women who have thyroid disease or are taking treatment for thyroid disease during pregnancy. The same risk can also be experienced by women who use fertility drugs, such as clomifen, before becoming pregnant.

Craniosynostosis Symptoms

Signs of craniosynostosis usually appear when the baby is born, and become more obvious after a few months. These signs include:

  • The crown or soft part of the baby's head is not felt
  • The forehead looks like a triangle, with a wide back of the head
  • Forehead that is partly flat and partly prominent
  • The position of one ear is higher than the other ear
  • The shape of the baby's head is smaller than the baby's age
  • Head shape is abnormal, for example, elongated and flat, or looks flat on one side

When to see a doctor

Keep the recommended immunization schedule. The pediatrician will monitor the child's growth and development during immunization, including the growth of his head. If you suspect that there is an abnormality in the development or shape of your child's head, don't wait to see a doctor immediately.

Please note, an abnormally shaped baby's head does not always indicate craniosynostosis. This condition can occur because the baby sleeps on one side of the body too often without changing positions. Therefore, a doctor's examination is needed to confirm it.

Diagnosis of Craniosynostosis

To confirm craniosynostosis, the doctor will perform the following tests:

  • Examination of the baby's head by checking the crown and abnormalities on the head
  • Scan with CT scan to see skull bones in more detail
  • Genetic test to determine the type of genetic disorder suspected to be the cause

Craniosynostosis Treatment

Craniosynostosis with mild or moderate severity does not require special treatment. Doctors simply advise patients to wear special helmets to improve the shape of the skull and help brain development.

While in most cases of serious craniosynostosis, treatment must be carried out with surgery.

Surgery for craniosynostosis depends on the severity of craniosynostosis and whether there is an underlying genetic disorder. The goal is to reduce and prevent pressure on the brain, make room in the skull for the brain to expand, and improve the shape of the skull.

There are two types of surgery that can be performed to treat craniosynostosis, namely:

  • Endoscopic surgeryThis surgery is performed on babies under 6 months of age. This procedure requires only 1 day of hospitalization and does not require a blood transfusion. After this procedure, therapy with a special helmet can be done to improve the shape of the skull.

  • Open surgeryThis procedure is performed on babies over 6 months old. Open surgery requires 3-4 days of hospitalization and blood transfusion.

Craniosynostosis Complications

Mild craniosynostosis left untreated can cause permanent changes in the shape of the head and face. As a result, people with craniosynostosis feel ashamed to blend in with society.

Patients with severe craniosynostosis are at risk for increased intracranial pressure (pressure inside the head cavity). If left untreated, increased intracranial pressure can lead to the following serious conditions:

  • Developmental disorders
  • Eye movement disorder
  • Cognitive impairment (learning and thinking)
  • Seizure
  • Blindness

Prevention of Craniosynostosis

Craniosynostosis is difficult to prevent, because this condition is an inherited disorder due to genetic factors. However, regular prenatal check-ups can be done to detect early onset of this condition in the fetus.

Then, babies born with craniosynostosis need regular check-ups with the doctor to monitor brain development and function. This is aimed at handling as early as possible if the baby is suspected of having a brain development disorder.

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