Multinodular liver involvement in Abernethy syndrome

A 15-year-old male child with the radiological finding of multiple hepatic nodules associated to a slight elevation of AST, ALT and GGT was referred to our institution. Tumor serum markers were normal.

A MRI revealed multiple bilobar liver nodules, ranging from 1.5 to 8 cm. The dynamic study showed a gross vascular structure (the fistula, asterix) at the hylum in relation with ICV and the common portal trunk. Intra-hepatic portal branches were not recognisable (Panel A). All the findings were suggestive of Regenerative Nodular Hyperplasia in a Porto-Cava shunt setting, but the radiological behaviour of the biggest nodule [wide necrotic-looking fluid content in T1 and haemorrhagic signal in T2 (Panels B,C) and hypointensity in the hepatospecific phase (Panel D)] was suggestive for degeneration, so we performed a left lobectomy.

At the microscopic examination, a complex Regenerative Multinodular Hyperplasia was found without any dysplasia, compatible with those associated with vascular malformations.

Acknowledgements

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Footnote

Conflicts of Interest: The authors have no conflicts of interest to declare.

Informed Consent: Written informed consent was obtained from the patient for publication of this article and any accompanying images. A copy of the written consent is available for review by the editor-in-chief of this journal.