Craniosynostosis

4 January 2018

After birth, a baby’s brain develops very rapidly in the first 3 years. In the first 9 months it is expected to grow up to two times and in the third year it is expected to grow to almost three times. In order for the brain to adapt to the rapid growth of the skull, the skull bones remain separate from each other for 12-18 months with flexible fibrous tissues called “sutures” (fontanel). During birth, when necessary, the sutures overlap without causing damage to the brain. It protects the brain from trauma during the postnatal period and produces bone for the cranial bones to adapt to growth during brain growth. Small skull disorders that occur during birth are normal and recover very quickly.

If one or more of the sutures between the skull bones closes early and loses its elasticity and the ability to produce new bones before the brain development is completed, developmental disorders will occur in the skull and the face. In principle, if the pouch between the two bones closes early, growth will stop in the bones parallel to it, and in the horizontal bones, there will be overgrowth to compensate for brain growth (Virchow’s law). For this reason, the shape of the skull will differ from the suture that closes.

In addition to the problem of formal development in the skulls, permanent headache, eye problems, intelligence and learning problems, neurological problems can be seen during the development of the child. These problems are caused by the increase in intracranial pressure due to the inability of the head bones to adapt to brain development.

The diagnosis of craniosynostosis can usually be made by a clinical examination of the pediatrician and computerized tomography. On average 1 case is diagnosed between 1000 and 3000 births. The occurence is higher in males. The exact cause is not known, but genetics play a role.

Craniosynostosis patients are divided into two groups according to the presence of other congenital anomalies which are syndromic and non-syndromic. While there are only isolated craniosynostosis in the non-syndromes, different anomalies are observed in the other parts of the body in the syndromic ones and they are named accordingly. The vast majority of craniosynostoses (80-90%) are not syndromic. Syndromic ones are very rare. The most common ones are;

Crouzon syndrome – affects 1 in 60,000 children, disrupting the growth of the skull and facial bones, causing skull and facial deformity.
Apert syndrome – affects 1 in 100,000 births and causes head, hand, foot and face deformities by disrupting the normal growth of the bones starting before birth.

Pfeiffer syndrome – affects 1 in 100,000 children. Disrupts bone growth and causes head and face deformities as well as causing large toes, large fingers, hands and feet.

Saethre-Chotzen – a genetic syndrome seen 1 in 50,000 births causing a variety of deformities, including facial imperfections

Posted in Blog, Oral and Maxillofacial Surgery, Skull Surgery by Prof. Dr. Kemal UĞURLU

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