Craniosynostosis and related syndromes

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The term craniostenosis was coined by Virchow1 in 1851. He suggested that when premature fusion of the cranial vault occurs, there is an inhibition of the normal growth of the skull in the direction perpendicular to the suture which is fused. This results in a compensatory growth in a direction parallel to the fused suture.

Because not much is known about the pathogenesis of craniostenosis, there is much confusion concerning the need for, the timing of, and the forms of treatment. Treatment is directed usually at the closed suture. This is not the origin of the problem but the result. Because of this lack of understanding, the cranial sutures often reclose prematurely. Many attempts have been made to prevent this occurrence. No procedure is universally successful; hence the great number of variations. These include painting the dura with Zenker’s solution, applying polyethylene film to the suture line, and removing the suture. It is our impression that reclosure following craniosynostectomy can be prevented. Attention must be given to the underlying pathology which will be discussed.

The normal cranial sutures are closed in a fibrous union by 6 months of age. By 20 months, the anterior fontanelle has closed. At approximately 8 years of age, the bones are completely ossified. Craniosynostosis by definition is present shortly after birth.

Craniosynostosis is not an uncommon problem. The incidence is approximately 5 per 10,000 births.2 Matson3 reported on 519 cases collected between 1930 and 1966. Sagittal synostosis, the most common form, is much more . . .



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