Tuberous Sclerosis

  • Etiology:
  • Imaging:
    — Brain – subcortical tubers with abnormal signal extending to ventricles along radial fibers, subependymal tubers, subependymal giant cell astrocytoma (SEGA) near Foramen of Monroe
    — Skin – angiofibroma
    — Cardiac – rhabdomyoma
    — Lungs – lymphangioleomyomatosis
    — GI – cysts in liver + pancreas
    — Renal – cysts, angiomyolipomas in 50%
    — Bone – bone islands

Cases of Tuberous Sclerosis

Axial CT without contrast of the abdomen shows the right kidney to be normal in size and to contain multiple small low density lesions that when measured demonstrate negative Hounsfield units. The left kidney, which is massively enlarged, also contains multiple small low density lesions as well as one extremely large low density lesion in its anterior aspect.