Purpose:The International Society for the Study of Vascular Anomalies (ISSVA) classification is crucial in diagnosing vascular anomalies (VAs), surpassing the International Classification of Diseases 10th Revision. This study aims to reevaluate diagnoses using ISSVA criteria and explore diagnostic patterns.

Methods:Analyzing 138 pediatric VA patients diagnosed via magnetic resonance imaging from 2018 to 2023 at Asan Medical Center, we reviewed clinical, imaging, pathology, and genetic data. Diagnoses were revised per 2018 ISSVA criteria, assessing discrepancies.

Results:Among 133 VA cases, 125 were malformations and eight were tumors, mostly in the head and neck. Clinical and imaging diagnoses disagreed in 51 cases. Some initially complex malformations were simplified. Lymphatic malformation cases shifted to VAs and vascular tumors were identified post-initial diagnosis.

Conclusion:Accurate diagnosis of VAs is essential for prognosis, treatment planning, and predicting outcomes. However, 14.2% of patients showed discordance between clinical diagnoses and imaging findings. Capillary malformations were often overlooked in imaging but became evident with relevant clinical findings. Adopting a multidisciplinary approach and a unified diagnosis based on ISSVA classification is crucial for clearly defining VAs.