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Craniosynostosis : Symptoms, Causes, and Treatment

Craniosynostosis is a condition of birth defects when the fontanel closes faster. As a result, the baby's head develops abnormally and causes the shape of the baby's head to appear imperfect.

Initially, the skull bone is not a single 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 the baby's brain can develop. Then, the crown will close and form a solid skull bone.

In babies with craniosynostosis, the fontanel closes faster before the baby's brain is fully formed. This condition makes the brain push the skull bones, so 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 trigger serious conditions, such as blindness to death.

Symptoms of Craniosynostosis

Craniosynostosis is a rare disease. Early in life, the development of the human brain is very rapid. In the first 6 months, it will reach twice its initial size and in the first year of life, it will reach 4 times its initial size.

At the age of 2 years, the baby's brain size reaches 80% the size of an adult brain. The developing brain needs a flexible skull to compensate for its development.

Symptoms that can arise are:




  • abnormal head shape.
  • hard lines along the sutura area.
  • loss of baby fontanelle.
  • The baby's head growth is not proportional to the growth of his body.
  • increased ICP (intracranial pressure) or pressure in the skull. For example, headaches, visual disturbances, sudden decline in children's academic abilities, vomiting.

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When to see a doctor

Keep the recommended immunization schedule. Pediatricians will monitor the child's growth and development during immunizations, including the growth of the child's head. If you suspect there is an oddity in the development or shape of the child's head, do not delay to immediately see a doctor.

Please note, an abnormal baby's head shape does not always indicate craniosynostosis. This condition can occur due to the baby sleeping too often on one side of the body, without changing positions. Therefore, a doctor's examination is needed to confirm this.

Causes of Craniosynostosis

Craniosynostosis is caused by the closure of the skull sutures that are too fast, so the growth of skull bones in these areas is limited. This condition can be divided into:

  • non-syndromic, not accompanied by other birth defects and generally do not know the cause.
  • Syndrome, caused by various syndromes that are generally caused by genetic disorders - such as Apert Syndrome, Crouzon Syndrome, Pfeiffer Syndrome, Saether-Chotzen.

Some other things that are thought to cause this situation:

  • endocrine: hyperthyroidism, hypophosphatemia, vitamin D deficiency, hypercalcemia, and so on
  • blood disorders that cause bone marrow hyperplasia - for example, sickle cells, thalassemia
  • lack of brain growth, for example, microcephaly

Risk Factors for Craniosynostosis

The risk of a baby suffering from craniosynostosis will be higher in women who suffer from thyroid disease or undergo treatment for thyroid disease during pregnancy. The same risk can also be experienced by women who use fertility drugs, such as clomifene, before becoming pregnant.

Diagnosis of Craniosynostosis

In the process of diagnosing craniosynostosis, the doctor will examine to find any abnormal head shape. Physical examination is done to see the baby's condition. The doctor will also take measurements of the baby's head and the condition of the suture and fontanel.

Follow-up examinations - such as CT, MRI, or X-rays, will be done to confirm the diagnosis. The aim is to see the Sutura network. While genetic testing will be done if there is a suspicion of genetic disorders that causes this condition.

Read Also : Common eye problems affect baby

Craniosynostosis Treatment

Craniosynostosis with mild or moderate severity does not require special treatment. The doctor will advise patients to use special helmets, to improve the shape of the skull and help brain development. However, the most serious cases of craniosynostosis are treated with surgery.

Surgery for craniosynostosis depends on the severity of craniosynostosis and whether there is an underlying genetic disorder. Surgery aims to reduce and prevent the emergence of pressure on the brain, make space in the skull so that the brain can develop, and improve the shape of the skull.

Two types of operations can be performed to treat craniosynostosis, namely:

  • Endoscopic Surgery

This surgery is performed on infants under 6 months of age. This procedure requires only one 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 three to four days of hospitalization and blood transfusion.

Complications of Craniosynostosis

Mild craniosynostosis left without treatment can cause permanent deformation of the head and face. As a result, sufferers of craniosynostosis feel ashamed to mingle with the community.

Patients with severe craniosynostosis are at risk of experiencing increased intracranial pressure (pressure inside the head cavity). If left untreated, an increase in intracranial pressure can trigger the following serious conditions:

  • Developmental disorders
  • Impaired eyeball movement
  • Cognitive disorders (learning and thinking)
  • Convulsions
  • Blindness



References

References

  1. Jung, et al. (2018). Craniosynostosis. Advances in Clinical Neuroscience and Rehabilitation.
  2. American Association of Neurological Surgeons (2019). Craniosynostosis and Craniofacial Disorders.
  3. Centers for Disease Control and Prevention (2017). Birth Defects. Craniosynostosis.

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Health and clinical interests include all aspects of infectious diseases

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