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Because their central nervous system abnormalities include type I lissencephaly and marked dilatation of the ventricles, we believe that some children with MDS may have increased intracranial pressure.
In advanced cases there can be marked dilatation of both the pancreatic duct and CBD simulating the 'double duct' sign seen with carcinoma of the head of pancreas.
Coronal MIP image shows a dilatation of cisterna chyli (arrow) and marked dilatation of both right and left retroperitoneal trunks (arrowheads) Open image in new window Fig. 4 A 47-year-old woman presenting with non-specific abdominal discomfort.
An abdominal radiograph showed marked dilatation of the small intestine.
Brain CT (Fig. 1b, c) showed marked dilatation of the lateral ventricles with moderate dilatation of the third ventricle, there was a striking decrease in attenuation surrounding the dilated ventricles, the inferior cerebellar vermis was absent, and the cerebellum was hypoplastic.
Marked dilatation of the right atrium and ventricle can be seen.
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Contrast enema studies show marked segmental dilatation of small bowel loop with normal colon findings.
Also, congested dilated central veins and marked sinusoidal dilatation with hupffer cell hyperplasia were also evident.
Worrisome imaging findings include progression of strictures on serial cholangiograms, marked biliary dilatation above a dominant stricture, and a polypoid ductal mass ≥1 cm in diameter[ 28 30 ].
A liver biopsy showed engrossment of centrilobular veins walls and fibrosis, with centrilobular hemorrhage and marked sinusoidal dilatation in acinar zone 3, suggesting increased intrahepatic venous pressure.
Intense polymorph nuclear infiltrates, marked sinusoidal dilatation and thick-walled arteries are seen on histopathology.
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