Multinodular liver involvement in Abernethy syndrome
Images in Clinical Medicine

Multinodular liver involvement in Abernethy syndrome

Caterina Cusumano, Luciano De Carlis

Milan Surgery and Abdominal Transplantation Division, Niguarda Ca' Granda Hospital, Italy

Correspondence to: Caterina Cusumano, MD. Niguarda Ca Granda Hospital, Surgery and Abdominal Transplantation Division, Piazza Maggiore 3, 20162 Milano, Italy. Email:

Submitted Feb 21, 2017. Accepted for publication Mar 06, 2017.

doi: 10.21037/hbsn.2017.03.08

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.




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.

Cite this article as: Cusumano C, De Carlis L. Multinodular liver involvement in Abernethy syndrome. Hepatobiliary Surg Nutr 2017;6(3):210-211. doi: 10.21037/hbsn.2017.03.08