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Congenital Dural Ectasia

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I want to finish up the congenital section

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with demonstration of dural ectasia.

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Dural ectasias can occur de novo, or they can be

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associated with a variety of different syndromes.

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By dural ectasia, we're effectively saying that there is

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expansion of the subarachnoid space and expansion

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of the intraspinal dural spaces.

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And this generally leads to posterior

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scalloping of vertebrae.

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So you see that these vertebrae are indented by what

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appears to be the subarachnoid space and the dura.

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This is what was referred to as dural ectasia.

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If we think about a lateral thoracic meningocele, we

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could say that that also is a variety of dural ectasias.

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And dural ectasias are associated with

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neurofibromatosis type 1.

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So in our differential diagnosis of dural ectasia, we have

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neurofibromatosis type 1, which can account for the

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posterior scalloping that you see in this lumbar region,

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and it may also be associated with a lateral thoracic

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meningocele. The other entities are Marfan syndrome.

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The Marfan syndrome, again, is a connective tissue disorder

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which may be associated with a similar variety

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of posterior scalping and dural ectasia.

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We then generally include the mucopolysaccharidoses.

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And the mucopolysaccharidoses may also be associated

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with thickening of the dura at the C1-C2 junction in general

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leading to potentially narrowing also at the foramen magnum.

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So the various types of mucopolysaccharidoses

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are also in the differential diagnoses.

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Patients who have achondroplasia

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also may have dural ectasias.

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And if the patient has a cause of an obstructive

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hydrocephalus intracranially,

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it also may lead to a dural ectasia.

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So there's a variety in the differential diagnoses.

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These are some of the most common and it requires you to

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be looking at other aspects of the imaging in order

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to diagnose these variety of congenital disorders.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Spine

Pediatrics

Neuroradiology

Musculoskeletal (MSK)

MRI

Congenital

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