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© 2009 by the American Institute of Ultrasound in Medicine
J Ultrasound Med 28:497-505 • 0278-4297

Case Series

Contrast-Enhanced Ultrasonography in Patients With Glycogen Storage Disease Type Ia and Adenomas

Anh T. Nguyen, MD, Aude Bressenot, MD, Simona Manolé, MD, Marie A. Galloy, MD, Jean P. Bronowicki, MD, Michel Vidailhet, MD, François Feillet, MD and Michel Claudon, MD

Department of Radiology, Children’s Hospital, and Institut National de la Santé et de la Recherche Médicale, Vandoeuvre, France (A.T.N., S.M., M.A.G., M.C.); Departments of Pathology (A.B.) and Pediatrics (M.V., F.F.), Centre de Reference des Maladies Héréditaires du Métabolisme, Institut National de la Santé et de la Recherche Médicale, Vandoeuvre, France; and Department of Hepatogastro-enterology, Centre Hospitalier Universitaire de Nancy Brabois, Vandoeuvre, France (J.P.B.).

Address correspondence to Michel Claudon, MD, Department of Pediatric Radiology, Children’s Hospital, Centre Hospitalier Universitaire de Nancy Brabois, Rue du Morvan, 54511 Vandoeuvre, France E-mail: m.claudon{at}chu-nancy.fr

Objective. The purpose of this series was to evaluate the value of contrast-enhanced ultrasonography (CEUS) in the characterization of focal liver lesions (FLLs) in patients with glycogen storage diseases (GSDs). Methods. Contrast-enhanced ultrasonographic data obtained for characterization of 8 FLLs (size, 0.9–10.2 cm) in 2 patients with GSD type Ia (GSD-Ia) and lesion growth or recurrent abdominal pain were reviewed and compared with computed tomographic (CT) and magnetic resonance imaging (MRI) data. After total and left hepatectomy, pathologic examination confirmed benign adenomas in 6 of the evaluated lesions. Follow-up confirmed benignity in the 2 remaining lesions. Results. In all FLLs, CEUS showed marked hypervascularity in the early arterial phase. Centripetal filling was shown in only 1 lesion, and diffuse enhancement without any clear direction was shown in all other lesions. During the portal and late phases, 6 of the 8 lesions showed sustained enhancement, including 2 lesions that appeared heterogeneous during all phases of CT and MRI. In an aspect of 1 of these 6 large adenomas, late wash-out could be explained by sinusoid compression. The other 2 adenomas showed moderate wash-out but remained homogeneous. Conclusions. Focal liver lesions found in patients with GSD-Ia have similar patterns on CEUS compared with incidental adenomas. Global or partial hypoenhancement observed in the late phase did not indicate a transition to hepatocellular carcinoma but may have been related to ischemia.

Key Words: glycogen storage disease • hepatocellular adenomas • imaging • liver • ultrasonographic contrast agents

Abbreviations: CEUS, contrast-enhanced ultrasonography • CT, computed tomographic • FLL, focal liver lesion • GSD, glycogen storage disease • HCA, hepatocellular adenoma • HCC, hepatocellular carcinoma • MRI, magnetic resonance imaging • OMIM, Online Mendelian Inheritance in Man

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