Undifferentiated cells between these osteogenic bone fronts form the cranial vault sutures, which function to keep the suture patent while allowing rapid and continual bone formation at the edges of the bone front until brain growth is complete. 8, 9 Differentiating osteoblasts accumulate on the leading edges of cranial vault bones as the brain expands during prenatal and early postnatal growth. The bones of the cranial vault ossify directly from undifferentiated mesenchyme. The intent is to improve pediatric care providers’ recognition and timely referral for craniosynostosis and their differentiation of synostotic from deformational and other nonoperative head shape changes. The report ends with a brief discussion of microcephaly as it relates to craniosynostosis as well as fontanelle closure. This is followed by a description of each type of primary craniosynostosis (metopic, unicoronal, bicoronal, sagittal, lambdoid, and frontosphenoidal) and their resultant head shape changes, with an emphasis on differentiating conditions that require surgical correction from those (bathrocephaly, deformational plagiocephaly/brachycephaly, and neonatal intensive care unit-associated skill deformation, known as NICUcephaly) that do not. As an introduction, the physiology and genetics of skull growth as well as the pathophysiology underlying craniosynostosis are reviewed.

The purpose of this clinical report is to review the characteristic head shape changes, as well as secondary craniofacial characteristics, that occur in the setting of the various primary craniosynostoses and deformations. Pediatric care providers, pediatricians, pediatric subspecialty physicians, and other health care providers should be able to recognize children with abnormal head shapes that occur as a result of both synostotic and deformational processes.

Misshapen ear