In 14 children with Sturge-Weber syndrome, cortical calcifications on CT scan was present in 12, localized brain atrophy in 10, enlargement of the choroid plexus in 7, and abnormal veins in 7. Cortical enhancement was present on 12 CTs performed shortly after an episode of severe seizures or hemiplegia but was absent or considerably less marked at a distance from the acute episodes. We suggest that cortical enhancement is related to seizure activity and/or blood-brain disturbances rather than to the extension of pial angioma.

Studies of pancreatic function should be performed routinely in cystic fibrosis, especially in pancreatic sufficiency or in patients with normal pancreas images. Acute pancreatitis should be diagnosed and properly identified to be differentiated from other acute abdominal syndromes occurring in cystic fibrosis.

We report a 29-week male fetus with healthy consanguineous parents. He showed a severe sclerosing bone disorder affecting all skeletal elements, resulting in insufficient modeling, generalized densification, and fragility of the skeleton. This skeletal dysplasia was associated with an abnormal craniofacial development (hypertelorism, severe microretrognathia, cleft palate, absent epiglottis, reduced number, and mineralization of teeth buds) and abnormal terminal phalanges. Neuropathologic examination showed bilateral fronto-parietal cerebral polymicrogyria. This syndrome appears to represent a new variant of congenital sclerotic bone disorder of unknown origin. Autosomal recessive inheritance is possible.