Craniosynostosis, Causes, Diagnosis, Treatment, Prevention, Complications

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metopic craniosynostosis, lambdoid craniosynostosis, craniosynostosis surgery, long term effects of craniosynostosis
Craniosynostosis, Causes, Diagnosis, Treatment, Prevention, Complications

Craniosynostosis is a birth defect in which the fontanel closes prematurely. 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.

In babies with craniosynostosis, the fontanel closes more quickly 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 inside the head cavity can increase and lead to serious conditions, such as blindness and death.

Causes and Risk Factors of Craniosynostosis

It is not known what causes craniosynostosis, but the 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 for the baby to develop craniosynostosis is higher in women who have thyroid disease or are taking thyroid medication during pregnancy. The same risk can also be experienced by women who use fertility drugs, such as clomifen, before becoming pregnant.

Symptoms of Craniosynostosis

Signs of craniosynostosis usually appear at birth, and become more pronounced 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
  • The shape of the 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 babies his age
  • Abnormal head shape, for example elongated and flat, or looks flat on one side

When to go to the doctor

Follow 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 there is an abnormality in the development or shape of the child's head, do not delay to immediately see a doctor.

It should be noted that 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 be sure.

Diagnosis of Craniosynostosis

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

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

Craniosynostosis Treatment

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

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

Surgery for craniosynostosis depends on the severity of the craniosynostosis and whether there is an underlying genetic abnormality. 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 done to treat craniosynostosis, namely:

  • Endoscopic Surgery
  • This surgery is performed on infants 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 surgery
  • This procedure is performed on infants over 6 months of age. Open surgery requires 3-4 days of hospitalization and blood transfusions.

Complications of Craniosynostosis

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 mingle with society.

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

  • Developmental disorders
  • Eye movement disorders
  • 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, routine pregnancy checks 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. It aims to treat as early as possible if the baby is suspected of having a brain development disorder.

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