Background: Marfan’s Syndrome is an autosomal dominant disorder of elastic connective tissue resulting from mutations in the FBN1 gene which encodes the glycoprotein fibrillin-1. Neurological complications of Marfan’s Syndrome may arise from dissection of arterial supply to the brain or spinal cord resulting in stroke, or pain syndromes resulting from musculoskeletal abnormality.
Methods: The case report of a 36 yr old woman with Marfan’s syndrome with severe acute lower back pain radiating to the posterior thigh region bilaterally is presented. Walking was limited by severity of pain which improved in the recumbent position. There was a history of recent aortic dissection. Imaging (to be shown) investigated the differential diagnosis of extension of aortic dissection or a neurological cause for back pain.
Results: MRI of the lumbosacral spine demonstrated dramatic dural ectasia, with erosive scalloping of the lumbo-sacral vertebrae (images shown). The patient responded to focussed physiotherapy and analgesia, with care taken to avoid valsalva manoeuvres.
Conclusions: Dural ectasia is one of the major Ghent criteria for the diagnosis of Marfan’s Syndrome. Case series reports have identified a degree of dural ectasia in up to 90% of patients with Marfan’s syndrome. The diagnosis of dural ectasia and its grading have received attention in recent years in the radiology community without a single grading system being accepted. Back pain in Marfan’s Syndrome can be a cause of significant morbidity. Features suggestive of dural ectasia may include improvement with recumbence. Aortic dissection is an important differential diagnosis to evaluate.