Dysgenesie des Vermis

Masses of

developmental and genetic origin affecting the paediatric craniofacial skeleton. Typical manifestations of nevoid basal cell carcinoma syndrome (NBCCS) in a 16-year-old boy. a Orthopantomography (OPT) shows cystic lesions of the mandible and maxilla (arrows), with unilocular and multilocular pattern and smooth or scalloped borders associated with displaced and unerupted permanent teeth. b Coronal CT scan (bone window) shows ectopic calcifications of the falx cerebri and tentorium cerebelli (arrows) and spotted meningeal calcifications (arrowheads). Brain MRI reveals a cavum veli interpositi on axial T2 (asterisk in c) and coronal contrast-enhanced T1 (asterisk in d) and also vermian dysgenesis (arrowheads in d)