Understanding Craniosynostosis: Causes, Symptoms, and Management

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Craniosynostosis

by Alike Medical Team ∙ Updated on June 13, 2023

Additional names

This group contains additional names: - Craniostenosis - Deformity of the skull - Early fusion of cranial sutures - Crouzon syndrome - Goldenhar syndrome - Hypertelorism - Macrocephaly - Pierre Robin syndrome - Robin sequence - Robin syndrome - Treacher collins syndrome

General

Craniosynostosis is a congenital defect in which one or more of the fibrous sutures between the bones in the baby’s skull fuse, before the brain finishes growing. Because the baby’s brain continues to grow, the head is given a misshapen appearance. In most of the kids, the sutures between the skull bones remain open and meet in soft spots- the fontanels.Craniosynostosis can be caused by some genetic syndromes such as Apert syndrome, Pfeiffer syndrome or Crouzon syndrome. Therefore it is important to look for other abnormalities in the baby’s limbs, ears or heart. In most of the cases, the reason for craniosynostosis is unknown. Craniosynostosis does not usually cause neurological damage or developmental problems if diagnosed and treated early. Treatment is by surgery. There are several types of craniosynostosis, depending on the suture that is fused; Sagittal is the most common type of craniosynostosis and it results in a head shape called scaphocephaly- long and narrow. Coronal suture fusion may cause a flattened forehead, turning nose and raised eye socket. Metopic suture fusion gives the forehead a triangular appearance and the back of the head is widened. Lambdoid synostosis is rare and it may cause one side of the head to look flat, and one ear seems higher than the other.

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Signs & symptoms

Craniosynostosis symptoms are usually visible right after birth and they become even more noticeable in the first few months. The severity of the condition depends on the number and timing of sutures fusion. Symptoms may include a misshapen skull, disappearance of the baby’s fontanel, appearance of hard ridges in the area along affected sutures, slowly growing head circumference, sleepiness, noticeable scalp veins, irritability, high-pitched cry, poor feeding, vomiting and developmental delay.

Diagnosis

Diagnosis is done upon findings in physical examination, imaging studies such as CT or MRI of the baby’s head and sometimes genetic tests to identify the cause.

Treatment

Some cases of craniosynostosis are left untreated and work out only with a helmet therapy that helps reshape the baby's skull. However, in most of the cases, surgery in the treatment of choice- which its timing and type are determined by the suture that is fused, and the reason for the condition. Before surgery, imaging studies or done to create a surgical plan and customize the template needed for the procedure. The surgery is performed by a neurosurgeon and can be done in either an open or a minimally invasive approach.

Note

☝ We provide information on prescription and over-the-counter medicines, diagnosis, procedures and lab tests. This material is provided for educational purposes only and is not medical advice, diagnosis or treatment.

National Institutes of Health ∙ World Health Organization ∙ MedlinePluse ∙ Centers for Disease Control and Prevention

☝ All information has been reviewed by certified physicians from Alike

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