Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome: Case Report and Review of the Literature
Autor: | David Zagzag, Daniel B. Rifkin, Girish M. Fatterpekar, Luigi Bassani, Navid Behrooz, Vineet Tyagi, Christopher S. Graffeo, Mary Helen Barcellos-Hoff, Dimitris G. Placantonakis, Taylor A. Wilson, Saul Penaranda |
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Jazyk: | angličtina |
Rok vydání: | 2014 |
Předmět: |
Marfan syndrome
musculoskeletal diseases Pediatrics medicine.medical_specialty medicine.diagnostic_test business.industry Dural ectasia Mechanical Aortic Valve Magnetic resonance imaging Case Report Disease Magnetic resonance myelography medicine.disease Cerebrospinal fluid Lumbar medicine Surgery Neurology (clinical) Radiology spontaneous intracranial hypotension business Myelography |
Zdroj: | Surgical Neurology International |
ISSN: | 2152-7806 2229-5097 |
Popis: | Background Spontaneous intracranial hypotension is an uncommon clinical entity. Heritable connective tissue disorders (HCTD), such as Marfan syndrome, are frequently implicated as an underlying cause, due to dural structural weaknesses that predispose patients to spontaneous cerebrospinal fluid (CSF) leak. Due to the high prevalence of multi-system disease in HCTD, diagnosis and treatment are often complicated. Case description We present a 58-year-old female with Marfan syndrome on anticoagulation for a mechanical aortic valve replacement who came to medical attention with severe, acute-onset headache following a straining episode. Noninvasive magnetic resonance (MR) myelography confirmed thoracic CSF extravasations and multiple lumbar diverticula. The patient was treated conservatively and her symptoms resolved. Conclusion We discuss the common presentation, diagnostic tools, and treatment options for spontaneous CSF leaks in patients with Marfan syndrome or related HCTD with an emphasis on noninvasive modalities and a review of the major radiographic criteria used to diagnose dural abnormalities, such as dural ectasia. |
Databáze: | OpenAIRE |
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