dural ectasia


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dural ectasia

A dilation of the membranes that line the lumbosacral spine, typically seen in patients with Marfan syndrome.
See also: ectasia
References in periodicals archive ?
In this case report, we present a patient with visual disturbances related with IHH associated with dural ectasia and monocular proprosis.
Dural ectasia of the optic nerve is considered in diagnosis.
Revised 2010 Ghent nosology uses dural ectasia as one of the systemic features used in diagnostic scoring.
The differential diagnosis for an obstructing pelvic cyst includes dural ectasias extending into the pelvis as an anterior sacral meningocele, as well as other masses of genito-urinary, gynecologic and gastrointestinal origin including ovarian cysts, bladder diverticuli and congenital arachnoid cysts.[sup.4] Historically, large dural ectasias in the pelvis were treated using cyst aspiration via the rectum or vagina; however, this procedure was associated with a high morbidity and mortality from meningitis.[sup.5] Conservative management consisting of primarily observation is selected for patients with high surgical risk or in whom the dural ectasia is asymptomatic, small and unlikely to enlarge.
It has been studied that these patients do not carry a mutation in the Marfan syndrome gene FBN1 encoding fibrillin 1, but instead a defect of microfibrils which are important components of the extracellular matrix associated with fibrillin.12 This has been speculated to be associated with the dural ectasias seen in SIH patients and is thought to relate to alterations in the elastin component of the dura resulting in the dilation of the dural sac due to CSF pulsation.13 Some patients have also shown to have a personal or family history of early age spontaneous retinal detachment which suggests the presence of a connective tissue disorder affecting both, the dura and the retina.
7).Rarely, it may be caused by dural ectasia (Figs 8a-c), arachnoid cysts or lateral thoracic meningocoeles.
* Lumbosacral dural ectasia visible by computed tomography (CT) or magnetic resonance imaging (MRI).
Dural ectasia was added to the list of the Ghent criteria because this condition is mainly limited to patients with Marfan syndrome and other connective tissue disorders.
* Nervous system--If dural ectasia (swelling of the covering of the spinal cord) develops, medication may help minimize any associated pain.