Imaging Modalities in Craniosynostosis: A Systematic Review and Proposal of the ARCANA Protocol for Multimodal Radiation-Free Assessment.

Background/

Objective

Craniosynostosis, the premature fusion of one or more cranial sutures, is the second most common craniofacial defect and poses significant diagnostic and therapeutic challenges. Our objective was to systematically evaluate current diagnostic imaging modalities for craniosynostosis and to propose a novel radiation-free ARCANA Protocol as an alternative to conventional screening.

Methods

Following PRISMA guidelines, we conducted a systematic review of the literature using PubMed and Cochrane databases from 2015 onwards, restricted to English-language and full-text articles. Inclusion criteria encompassed studies evaluating diagnostic accuracy, radiation exposure, and neurocranial outcomes associated with imaging modalities in craniosynostosis. Quality assessment was performed using QUADAS-2. To evaluate the certainty of evidence supporting each imaging modality, we applied the GRADE framework. Given the extensive number of included studies (n = 70), findings were categorized by diagnostic modality rather than individual studies.

Results

Analysis of 70 selected studies demonstrated a continued reliance on 3D computed tomography (3DCT) as the diagnostic gold standard, despite recognized risks of cumulative radiation exposure in pediatric populations. Alternative radiation-free imaging techniques including high-resolution ultrasonography (US), three-dimensional stereophotogrammetry (3DSPG), and advanced magnetic resonance imaging (MRI) have emerged, offering substantial benefits such as eliminating ionizing radiation and providing comprehensive neurocranial assessments. 3DCT demonstrates approximately 90% sensitivity and 90-100% specificity for detecting suture closure; ultrasound achieves 71-100% sensitivity and 86-100% specificity, while advanced MRI techniques such as GA-VIBE report up to 97% sensitivity and 96% specificity.

Conclusions

The proposed ARCANA Protocol integrates clinical assessment, 3DSPG, US, and advanced MRI sequences into a unified multimodal framework that eliminates radiation exposure while ensuring comprehensive evaluation of cranial and intracranial anatomy. The protocol emphasizes patient safety and diagnostic accuracy. The main limitations of this study are the heterogeneity of the included studies and the lack of prospective validation, which is essential to confirm diagnostic and clinical effectiveness and to support a potential paradigm shift toward radiation-free assessment of craniosynostosis.