Contrast-enhanced ultrasonography in patients with glycogen storage disease type Ia and adenomas.
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.
Nguyen
Anh T
AT
Department of Pediatric Radiology, Children's Hospital, Institut National de la Santé et de la Recherche Médicale, Vandoeuvre, France.
Bressenot
Aude
A
Manolé
Simona
S
Galloy
Marie A
MA
Bronowicki
Jean P
JP
Vidailhet
Michel
M
Feillet
François
F
Claudon
Michel
M