Teaching Neuro Images: Intracranial hypotension in a patient with Marfan syndrome

Abstract

An 18-year-old man with Marfan syndrome and migraine headaches presented with acute worsening of headaches with postural changes following spinal fusion surgery for scoliosis. Lumbar spine MRI done before surgery showed diffuse dural ectasia (figure 1). Brain MRI after surgery showed distended transverse and sagittal dural venous sinuses1 and an enlarged pituitary gland suggesting intracranial hypotension (figure 2). He was treated conservatively with rest, fluids, and caffeine. Patients with Marfan syndrome frequently have dural ectasia2 and are at risk of CSF leaks after spinal surgery leading to intracranial hypotension. Our case highlights subtle changes on brain MRI suggesting intracranial hypotension.