Craniosynostosis: Imaging and Surgical Implications

Craniosynostosis is characterized by the early fusion of cranial sutures, leading to abnormal skull shapes, potentially increased intracranial pressure (ICP), and, in some cases, neurocognitive challenges. Craniosynostosis may occur in isolation or as part of a genetic syndrome, with the majority of cases being nonsyndromic. Genetic mutations, such as those affecting the <i>FGFR</i> and <i>TWIST</i> genes and numerous signaling pathways, play a pivotal role in the etiopathogenesis of craniosynostosis. Secondary craniosynostoses related to systemic diseases and bone dysplasia are also common. Imaging is integral to the evaluation of craniosynostosis, allowing accurate diagnosis, differentiating mimics, facilitating surgical planning, and enabling postoperative monitoring. Technical advances in US, low-dose CT, and black bone MRI have enhanced diagnostic accuracy while minimizing pediatric patients' exposure to radiation. Surgical intervention is often required to ensure deformity correction, maintain adequate intracranial volume for early brain development, and prevent immediate and delayed complications. Timely intervention and systematic follow-up are critical for better long-term outcomes and decrease the risk of repeat surgeries in pediatric patients with craniosynostosis. The authors provide a comprehensive overview of craniosynostosis, beginning with the embryologic development and anatomic organization of cranial sutures. The multifactorial causes and the classification of this disorder are discussed, with an emphasis on syndromic and nonsyndromic craniosynostoses. Critical imaging findings and a structured presurgical imaging approach are described and illustrated. In addition, surgical planning, timing, and techniques; postoperative imaging appearances; and long-term follow-up considerations critical to multidisciplinary care are reviewed. ^©RSNA, 2026 Supplemental material is available for this article.