This usually occurs in the lumbosacral region, as this is where the cerebrospinal fluid pressure is greatest,[1] but the spinal canal can be affected in any plane.

[11] It may also occur in Ehlers-Danlos Syndrome, neurofibromatosis type I,[12] ankylosing spondylitis,[1] and is associated with spondylolisthesis, vertebral fractures,[13] scoliosis, tumors or trauma.

[14] In neurofibromatosis type I, it has been theorized that local infiltration of the dura by plexiform neurofibromas leads to a weakening of the dural allowing the outpouching.

[17] Associated signs include a lack of epidural fat at the posterior wall of the vertebral body, the presence of radicular cysts,[18] anterior meningoceles, nerve root sleeve herniation and gradual erosion of the vertebral bodies (scalloping).

[5] It has been reported that acetazolamide can be used to treat dural ectasia in individuals with Marfan syndrome, however, the only supporting evidence for this assertion is a small study of 14 patients which was not peer-reviewed or submitted for publication.