Craniosynostosis

Primary craniosynostosis, the premature closure of one or more cranial sutures, occurs in up to one in 2,500 births. Secondary causes of craniosynostosis include disorders that lead to failure of brain development and conditions that influence bone metabolism. The craniosynostoses result in skull deformities and increased intracranial pressure as the developing brain is compressed. Clinical signs and symptoms depend on which suture or combination of sutures are involved. The etiology of craniosynostosis is believed to concern genetic abnormalities in fibroblast growth factors or their receptors, and the craniosynostoses can be associated with other congenital syndromes. The most important differential diagnoses are molding that occurs during birth in young infants, and positional molding that occurs as a result of excessive time spent in the supine position in older infants. The diagnosis may be confirmed by plain films of the skull, and surgical treatment is usually indicated to prevent neurologic complications.