Assessing focal liver lesions in Beckwith-Wiedemann Syndrome: Comparison of contrast-enhanced ultrasound and magnetic resonance imaging.

PURPOSE: Beckwith-Wiedemann Syndrome (BWS) predisposes children to hepatoblastoma and other cancers and requires tumor screening with abdominal ultrasound and magnetic resonance imaging (MRI) when lesions are seen. Contrast-enhanced ultrasound (CEUS) may offer an alternative to MRI, which often requires sedation. Our aim was to evaluate the diagnostic performance of CEUS compared to MRI in this group when evaluating liver lesions, to compare sedation requirements, and to assess imaging features correlated with malignancy. MATERIALS AND METHODS: This was a retrospective review of patients with molecular BWS and liver lesions who underwent MRI and CEUS within 6 months. Demographics, sedation requirement, and final diagnosis (pathology or follow-up imaging) were recorded. Two blinded radiologists per category classified lesions (benign/malignant/indeterminate) and detailed imaging features. Test performance and inter-rater reliability (Cohen's κ) were calculated. RESULTS: 12 patients (7 females, mean age 3 months) were included. A final diagnosis of 3 hepatoblastomas and 9 benign lesions was derived from pathology in 4 cases and from imaging follow-up in 8. Ten MRI examinations (83%) and no CEUS examinations required sedation. On consensus read, CEUS had a sensitivity of 67% and a specificity of 89%; κ = 0.70. MRI had a sensitivity of 75% and a specificity of 100%; κ = 0.25. Iso-enhancement and washout on CEUS and cystic change, necrosis, and diffusion restriction on MRI correlated with malignancy. CONCLUSION: Our findings suggest that CEUS has good diagnostic accuracy and high inter-rater reliability and allows sedation avoidance, suggesting that its use could allow MRI to be reserved for indeterminate or malignant lesions.