Radiology Cases Neurofibromatosis LSBU

Description: Neurofibromatosis, Von Recklinghausen's disease, is an autosomal dominant inherited disorder of mesenchymal tissue, where fibrous skin tumours, neurilemmomas and areas of skin pigmentation ( cafe-au-lait spots ) are typical features. Neurolemmomas can affect cranial or peripheral nerves.
Bone radiographs may show disturbance of bone architecture which can immitate polyostotic fibrous dysplasia, metabolic bone disease ( vitamin D resistant rickets ) and pressure from neighbouring fibromas. Scoliosis is common. The condition is associated with an increased incidence of meningiomas, phaeochromocytomas, and renal artery stenosis. In the skull there is enlargement of the middle cranial fossa and deficient ossification of the greater wing of sphenoid.
Pseudarthrosis may occur, typically in the tibia and presumably the result of abnor