Craniosynostosis Part I: Pathophysiology and Patient Evaluation

Abstract

Craniosynostosis is the premature fusion and obliteration of one or more cranial sutures. As a result, cranial growth can only occur parallel to the involved suture(s) resulting in predictable head shape morphologies. More importantly, the restricted cranial growth can also have a deleterious effect on the growing brain with increased intracranial pressures leading to abnormal neurocognitive development, blindness, and death. Craniosynostosis can either be syndromic or non-syndromic; and therefore, patient evaluation should be performed in a multi-disciplinary team setting for comprehensive care. Physical exam focuses on head shape morphology, associated anomalies, and signs of intracranial pressure. Imaging with 3D CT is gold standard at confirming diagnosis and extent of suture involvement. Early diagnosis is key for planning optimal intervention. This review contains 14 figures, 4 tables and, 42 references. Keywords: craniosynostosis, virchow’s law, plagiocephaly, trigonocephaly, scaphocephaly, brachycephaly, Apert Syndrome, Crouzon Syndrome, Pfeiffer Syndrome, intracranial pressure