High flow vascular malformation and hemangioma in the setting of PIK3CA hemi-hypertrophy syndrome

2019-10-21 13:03:00

Category: Musculoskeletal Imaging, Region: Other, Plane: Other

Sagittal contrast enhanced MRI of the lumbar spine at 8 years old demonstrates normal bone marrow signal (Figure 1A). Follow up MRI 3 years latter shows interval development of an enhancing L3 vertebral body mass expanding into the ventral epidural space (arrow) resulting in compression of the cauda equina (left image, Figure 1B). Axial (right image) T1 gadolinium enhanced MRI of the L3 vertebral body demonstrates an enhancing mass with marked vascularity (arrow heads). Digital subtraction angiography of the second lumbar artery shows markedly increased vascularity involving the enhancing mass at the L3 level (left) (Figure 1C). The mass had blood supply arising from the L2-L4 levels (not shown). The blood supply is significantly decreased following multilevel coil embolization (right). Post-operative sagittal contrast enhanced MRI demonstrates decreased size of the mass and resolution of canal stenosis (Figure 1D).