Duraektasie

Imaging of

Marfans syndrome. MRI spine showing dural ectasia of lumbo-sacral spine.

Further

delineation of Loeys-Dietz syndrome type 4 in a family with mild vascular involvement and a TGFB2 splicing mutation. Clinical and radiological features of the proband and her daughter. (A) Clinical phenotype of the proband including microretrognathia, and elongated philtrum (a, b), mild generalized skin hyperextensibility (c), joint hypermobility (d, e). (B) MRI in the proposita showed dural ectasia of the lumbar tract (a), MRA disclosed tortuosity and ectasia of carotid, vertebral, and cerebral arteries (b, c) and marked tortuosity of both segmental pulmonary arteries (d). (C) Clinical presentation of the 34 years-old proband’s daughter: hypotelorism, elongated philtrum and malar hypoplasia (a, b), mild skin hyperextensibility over the neck (c), atrophic post-surgical scar and ecchymosis (d), arachnodactyly with positive wrist and thumb signs (e, f).

bmcmedgenet.biomedcentral.com • CC-by-4.0

Dural ectasia

• Ankylosing spondylitis - Ganzer Fall bei Radiopaedia

Radswiki, T. Ankylosing spondylitis. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-11188 • CC by-nc-sa 3.0 (modified)

Dural ectasia

• Dural ectasia in Marfan syndrome - Ganzer Fall bei Radiopaedia

Ruales, F. Dural ectasia in Marfan syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-16114 • CC by-nc-sa 3.0 (modified)

Dural ectasia

• Dural ectasia: with neurofibromatosis type 1 - Ganzer Fall bei Radiopaedia

Daire, A. Dural ectasia: with neurofibromatosis type 1. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-31032 • CC by-nc-sa 3.0 (modified)

Dural ectasia

• Dural ectasia: Marfan syndrome - Ganzer Fall bei Radiopaedia

Gaillard, F. Dural ectasia: Marfan syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-35210 • CC by-nc-sa 3.0 (modified)

Dural ectasia

• Dural ectasia in Marfan syndrome - Ganzer Fall bei Radiopaedia

Keshavamurthy, J. Dural ectasia in Marfan syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-40020 • CC by-nc-sa 3.0 (modified)

Dural ectasia

• Dural ectasia with dural diverticula in ankylosing spondylitis - Ganzer Fall bei Radiopaedia

Hidayatov, A. Dural ectasia with dural diverticula in ankylosing spondylitis. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-55919 • CC by-nc-sa 3.0 (modified)

Dural ectasia

• Dural ectasia in Marfan syndrome - Ganzer Fall bei Radiopaedia

Hekmatnia, A. Dural ectasia in Marfan syndrome. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-73742 • CC by-nc-sa 3.0 (modified)

Dural ectasia

• Dural ectasia - neurofibromatosis - Ganzer Fall bei Radiopaedia

Bickle, I. Dural ectasia - neurofibromatosis. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-78817 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

Dural ectasia refers to ballooning or widening of the dural sac which can result in posterior vertebral scalloping and is associated with herniation of nerve root sleeves.

Clinical presentation

Patients with dural ectasia may present with low back pain or radicular pain in the buttocks or legs. Pain may be accompanied by leg weakness or urinary incontinence. Other associations include spondylolisthesis, scoliosis, vertebral erosions, and vertebral fractures. There is also an increased incidence of anterior sacral meningoceles which may present as an abdominal mass.

Associations

Marfan syndrome: dural ectasia has been observed in 60-90% of patients; in these patients, the dilatation of the dural sac is almost always in the lumbar region
Ehlers-Danlos syndrome
Loeys-Dietz syndrome
neurofibromatosis type 1
ankylosing spondylitis
osteogenesis imperfecta
acromegaly
trauma
post surgery
tumors
scoliosis

Radiographic features

Dural ectasia describes widening of the dural sac or spinal nerve root sleeves, usually associated with bony erosions of the posterior vertebral body . One study suggests that the anteroposterior diameter of the thecal sac at the S1 level should be greater than that of the thecal sac at the L4 level in order to describe dural ectasia .

Plain radiograph

Posterior vertebral scalloping on a lateral radiograph may be an indirect indicator . However, this is not specific, as it is seen in a significant percentage of the normal population and is also associated with several other conditions.