BACKGROUND AND PURPOSE Dural ectasia is well recognized in Marfan syndrome (MFS) as one of the major diagnostic criteria, but the exact prevalence of dural ectasia is still unknown in Loeys-Dietz syndrome (LDS), which is a recently discovered connective tissue disease. In this study, we evaluated the prevalence of dural ectasia in LDS according by using qualitative and quantitative methods and ...

purpose : to report two patients with spontaneous scleral perforation and bleb formation associated with marfan syndrome (mfs) which one of them also showed bilateral optic nerve dural ectasia. case reports : an 11-year-old girl was referred with progressive myopia. past medical history was negative. slit-lamp examination revealed bilateral scleral thinning and perforation with bleb formation, ...

Cauda equina syndrome (CES) associated with dural ectasia is a rare neurologic complication in patients with longstanding ankylosing spondylitis (AS). We report a 68-year-old male with a 30-year history of AS who presented a typical symptom and signs of progressive CES, urinary incontinence and neuropathic pain of the lumbosacral radiculopathy. Computed tomography (CT) and magnetic resonance im...

An 18-year-old man with Marfan syndrome and migraine headaches presented with acute worsening of headaches with postural changes following spinal fusion surgery for scoliosis. Lumbar spine MRI done before surgery showed diffuse dural ectasia (figure 1). Brain MRI after surgery showed distended transverse and sagittal dural venous sinuses and an enlarged pituitary gland suggesting intracranial h...

Marfan syndrome (MFS) is a genetic disorder of connective tissue, characterized by variable clinical features and multisystem complications. The anesthetic management during delivery is debated. Regional anesthesia has been used with success during cesarean delivery, but in some MFS patients there is a probability of erratic and inadequate spread of intrathecal local anesthetics as a result of ...

Purpose: To report two patients with spontaneous scleral perforation and bleb formation associated with Marfan syndrome (MFS); which one of them also showed bilateral optic nerve dural ectasia. Case reports: An 11-year-old girl was referred with progressive myopia. Past medical history was negative. Slit-lamp examination revealed bilateral scleral thinning and perforation with bleb formation, s...

Abstracts: Dural ectasia is circumferential expansion or dilatation of the dural sac, and has been frequently reported in association with type 1 neurofibromatosis (NF1). It should be differentiated from posterior mediastinal tumors such as neurofibroma, neuroblastoma, and ganglioneuroma because NF-1 has a high risk of tumor formation. In the spinal deformities of NF-1 patients, despite the sco...

Optic nerve dural ectasia is a rare cause of optic nerve sheath enlargement due to the accumulation of CSF around the optic nerve with no associated pathology. It diagnosed by MRI studies and can follow benign or sometimes an unfavorable course. We describe the case of a 24-day-old female referred for a visual blurring, which we diagnosed as a dural ectasia of the optic nerve sheath by MRI and ...

Pregnancy is considered a period of high risk for cardiovascular complications in patients with Marfan syndrome. Therefore the choice of anesthetic technique for delivery should be focused on minimizing hemodynamic fluctuations, and preferably provide adequate post-operative pain control. For this purpose, neuraxial blocks, such as spinal or epidural anesthesia, may be deemed a safe option. How...