BACKGROUND: Alagille's syndrome,
also called arteriohepatic dysplasia, is a congenital anomaly consisting
of hepatic, ocular, skeletal, and cardiac anomalies. The abdominal imaging
findings were reviewed in eight patients with biopsy-proven Alagille's
syndrome. One patient also had coexistent hepatocellular carcinoma. METHODS:
Seven right upper quadrant sonograms, six hepatic CT studies, five hepatobiliary
imaging studies, two hepatic MRI examinations, and two sulphur colloid
liver spleen radionuclide studies were reviewed. RESULTS: The most striking
abnormality was gross distortion of hepatic architecture. Five patients
(63%) had marked external hepatic contour abnormalities, usually with either
the entire liver or lobe having a predominately spherical shape. The portal
vein was displaced by the spherical parenchymal component in four cases.
Three other patients demonstrated marked hepatomegaly with no external
contour abnormality. Hepatobiliary imaging studies demonstrated markedly
prolonged excretion of the radiopharmaceutical in three of four patients
examined. CONCLUSIONS: A diagnosis of Alagile's syndrome is suggested when
a large, deformed and somewhat spherical liver is encountered, especially
when hepatobiliary imaging studies demonstrate delayed excretion of radiopharmaceutical.
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