Pediatric approach to craniosynostosis.

Abstract

1. Gregory S. Liptak, MD, MPH* 2. Joseph M. Serletti, MD† 1. 2. *Associate Professor of Pediatrics. 3. 4. †Associate Professor of Surgery, University of Rochester Medical Center, Rochester, NY. Asymmetry of the head is a common occurrence in infants and may have a number of etiologies, including positional deformity, simple craniosynostosis, complex craniofacial synostosis, metabolic bone disease, depressed skull fracture, microcephaly due to brain damage, and hydrocephalus. Deformations of the head attributable to prenatal compression usually resolve by the time a child is 2 months of age. However, a number of centers have reported a recent increase in the occurrence of cranial deformations in infants older than 6 months, which is attributed to the current recommendation that children sleep on their backs to avoid sudden infant death syndrome. Synostosis of a single suture may lead to only cosmetic changes, but synostosis of several sutures may be life-threatening and lead to increased intracranial pressure, loss of vision, and severe deformities that affect social interactions. Primary craniosynostosis, which is defined as synostosis without an underlying abnormality of the brain or metabolic defect, occurs in 1 in 2,000 births. Secondary craniosynostosis, such as would be due to cerebral atrophy, is somewhat less common. Figure 1⇓ shows the occurrence of isolated craniosynostosis in a referral center. (“Isolated” in this context means involvement of the sutures only without an identified genetic or chromosomal abnormality such as Apert syndrome.) Involvement of the sagittal suture is the most common form and occurs more frequently in males than in females. Synostosis of a single suture is more common than synostosis of multiple sutures. Figure 1. Occurrence of isolated craniosynostosis in a referral center. Reprinted with permission from Shillito J Jr, Matson DD. Craniosynostosis: a review of 519 surgical patients. Pediatrics. 1968;41:829–853 . Secondary craniosynostosis may be due to a structural cerebral abnormality such as atrophy or hypoplasia and may be related to other conditions such as hyperthyroidism, severe anemia (eg, thalassemia), or a metabolic condition (eg, mucopolysaccharidosis). Primary …