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Spontaneous Intracranial Hypotension and Dural Ectasia in Marfan Syndrome: An Illustrative Case Successfully Treated with Steroid Therapy and Literature Review.
Brain Sci
November 2024
Institute of Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Catholic University, 00168 Roma, Italy.
Article Synopsis
- Spontaneous intracranial hypotension (SIH) is a rare condition often misdiagnosed, particularly in patients with connective tissue disorders like Marfan Syndrome, which can lead to structural weaknesses in the spinal dural membrane and increased risk of CSF leaks.
- A 52-year-old woman with genetically confirmed Marfan Syndrome presented with severe headaches and diplopia; imaging revealed complications such as dural ectasia and subdural hematoma, which were effectively managed with bed rest and corticosteroids.
- A review of 25 studies on SIH treatment in patients with Marfan Syndrome indicated high success rates for symptoms improvement, particularly with epidural blood patches and conservative treatments.
Ruptured venous aneurysm associated with a dural arteriovenous fistula: Two case reports.
World J Clin Cases
October 2024
Department of Neurosurgery, Chonnam National University Hospital and Medical School, Gwangju 61469, South Korea.
Article Synopsis
- Venous aneurysms associated with dural arteriovenous fistula (dAVF) typically arise from long-standing venous hypertension and present as an enlargement of the draining vein, but some saccular aneurysms can occur without clear evidence of hypertension, making them quite rare.
- This report highlights two cases of ruptured saccular venous aneurysms linked to dAVF, both lacking signs of venous hypertension and exhibiting significant curvature or narrowing in the draining veins, which were successfully treated using transarterial embolization.
- The findings suggest that while hemodynamic stress is important in the formation of venous aneurysms, prior venous hypertension is not essential for their development, emphasizing the
A successful combined spinal-epidural anesthesia for cesarean section in a patient with neurofibromatosis type 1-associated dural ectasia.
JA Clin Rep
October 2024
Department of Anesthesiology and Intensive Care Medicine, Nagoya City University Graduate School of Medical Sciences, 1, Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi, 467-8601, Japan.
Article Synopsis
- Dural ectasia, a condition linked to neurofibromatosis type 1, can complicate spinal anesthesia procedures, as seen in a case of a parturient who previously experienced a failed spinal block during a cesarean section.
- For her repeat cesarean, preoperative imaging confirmed dural ectasia, leading to the decision to use combined spinal-epidural anesthesia to address potential complications.
- Ultimately, while the spinal block alone was insufficient, the additional epidural anesthesia effectively provided the necessary pain relief for the surgery.
Cluster analysis of 100 Marfan patients based on aortic 4D flow MRI and Z-score: insights into disease heterogeneity and stratification of subgroups.
Eur Radiol
September 2024
Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Objectives: 4D flow MRI-derived variables from Marfan patients are highly heterogeneous. Our aim was to identify distinct Marfan patient subgroups based on aortic 4D flow MRI and Z-score for stratification of distinct hemodynamic profiles and clinical features by means of hierarchical cluster analysis.
Materials And Methods: One hundred Marfan patients underwent baseline aortic 4D flow MRI at 3 T.
Spinal Dural Ectasia Spectrum: Management of Two Cases and Systematic Review of the Therapeutic Options.
Clin Spine Surg
September 2024
Department of Neurosurgery, IRCCS Istituto "G. Gaslini," Genoa, Italy.
Article Synopsis
- - The text discusses two cases of lumbosacral dura mater expansion that compressed nerves, treated with lumboperitoneal shunts (LPS) after reviewing cases from existing literature on symptomatic dural ectasia (DE).
- - A comprehensive search evaluated surgical management approaches for DE, including treatments for both children and adults, while excluding cases with cerebrospinal fluid leaks and considering severe forms like meningoceles.
- - The conclusion highlights that DE is rare and mainly linked to connective tissue disorders, suggesting treatment strategies should be tailored based on the specific symptoms and etiologies, as the condition can lead to ongoing complications.
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