Ten-year clinical and imaging follow-up of dural ectasia in adults with Marfan syndrome
Department of Orthopaedic Surgery, The Johns Hopkins University, 601 N. Caroline St, Baltimore, MD 21287, USA.The spine journal: official journal of the North American Spine Society (Impact Factor: 2.43). 12/2012; 13(1). DOI: 10.1016/j.spinee.2012.10.021
BACKGROUND CONTEXT: Dural ectasia in the lumbosacral spine is a common feature of Marfan syndrome and is associated with low back pain and surgical complications, but its natural history is unknown. PURPOSE: To evaluate the natural history of dural ectasia in adults with Marfan syndrome by determining if, over time, symptoms associated with dural ectasia worsen, dural ectasia imaging findings worsen, or spondylolisthesis/spondylolysis develops or worsens. STUDY DESIGN: Prospective cohort study. PATIENT SAMPLE: For our prospective follow-up study, we enrolled 20 patients with Marfan syndrome and dural ectasia who, from 1998 through 1999, had undergone magnetic resonance imaging (MRI) and computed tomography (CT) of the lumbosacral spine and had completed the Oswestry Disability Index (ODI) questionnaire. Of the 20, five did not meet the inclusion criterion of a completed 2009 ODI questionnaire and were excluded. The remaining 15 patients (mean age, 49.6 years; mean follow-up, 10.5 years) formed our study group. OUTCOME MEASURES: The ODI, MRI-based qualitative and quantitative measurements, CT-based quantitative measurements. METHODS: We performed matched-pair analyses via Student t test and Wilcoxon signed-rank test of the ODI scores (15 pairs), dural volume of L5-S2 (eight pairs), dural sac ratio (DSR) of L4-S2 (nine pairs), development/progression of spondolysthesis/spondylolysis (11 pairs), and Fattori qualitative grading of dural ectasia size (10 pairs). Significance was set at p<.05. RESULTS: We found no statistical differences in the 1998/1999 and 2009 ODI scores (25.8 vs. 22.2 points), dural volume (70.4 vs. 73.9 cm(3)), or DSR (0.68, 0.78, 2.04, and 58.1 vs. 0.69, 0.83, 2.30, and 70.20). There was also no development or progression of spondylolisthesis/spondylolysis and no increase in dural ectasia size. CONCLUSIONS: During this 10-year period, the natural history of dural ectasia in adults with Marfan syndrome was not associated with a significant increase in ODI scores, dural ectasia size, or with the development/progression of spondylolisthesis or spondylolysis.
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ABSTRACT: Neuraxial anaesthetic techniques are considered useful to minimise haemodynamic stress during labour. In Marfan's syndrome, connective tissue abnormalities not only affect the thoracic aorta but also predispose to dural ectasia. A dural ectatic sac may cause difficulties with neuraxial analgesia and anaesthesia. We discuss magnetic resonance imaging appearances and anaesthetic experience of three deliveries in a parturient with stable echocardiographic findings. We consider that epidural analgesia and anaesthesia are a safe and pragmatic solution for labour and delivery. Lumbo-sacral magnetic resonance imaging at presentation will define dural ectasia and assist in management.
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ABSTRACT: To perform a comprehensive study of dural ectasia related to FBN1 mutations. Database analysis of two German metropolitan regions of 150 patients (68 men, 82 women; mean age 35 ± 16 years). All patients had a FBN1 mutation and underwent dural magnetic resonance imaging. Age was <16 years in 20, 16-25 in 27, 26-35 in 67, and >35 in 36 patients. Prevalence of dural ectasia was 89% with criteria of Oosterhof and Habermann, 83% with Fattori, 78% with Lundby, and 59% with Ahn. Dural ectasia was less frequent in patients <16 years with Ahn and Fattori. Dural ectasia related to skeletal manifestations with all criteria, to aortic Z-scores and mitral valve prolapse with criteria of Habermann and Lundby, and to age with criteria of Fattori. The Fattori-grade of dural ectasia increased with age, aortic Z-scores, and skeletal score points. There was no consistent relationship of dural ectasia with any type of FBN1 mutation. Dural ectasia is frequent in patients with FBN1 mutations irrespective of age and its severity increases during life. Criteria of Oosterhof and Habermann yielded most consistent diagnostic results. Dural ectasia relates to skeletal involvement, aortic Z-scores, and mitral valve prolapse.
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ABSTRACT: Purpose: Marfan syndrome (MFS) is a genetic disorder of the connective tissue. Aortic root dilation is a main criterion of the Ghent Nosology. Dural ectasia and the presence of mitral valve prolapse (MVP) contribute to its systemic score. The purpose of this study was to investigate the frequency of dural ectasia and its correlation with cardiovascular manifestations in a pediatric study population. Patients and methods: 119 pediatric patients with confirmed or suspected MFS were examined in the local Marfan Clinic. 31 children with MFS who underwent magnetic resonance imaging (MRI) were included. Each patient was evaluated according to the Ghent nosology. Echocardiography was used to measure the aortic root diameter and assess the presence of MVP and mitral regurgitation. Z-scores were calculated for the evaluation of the aortic root diameters. MRI was performed to determine the dural sac ratio (DSR). Results: The prevalence of dural ectasia was 90.3 %, of aortic root dilation 32.2 %, of MVP 64.5 % and of mitral regurgitation 51.6 %. DSR at L5 correlated with the intraindividual z-scores (slope, 3.62 ± 1.5 [0.56; 6.68]; r = 0.17; p = 0.02; F = 5.84). Z-scores ≥ 2 were accompanied by dural ectasia in 100 %, MVP in 95 % and mitral regurgitation in 100 % of cases. MVP was accompanied by mitral regurgitation in 70 % of cases. Conclusion: As the examined cardiac manifestations show a coincidence with dural ectasia in 95 - 100 % of cases, MRI for diagnostic dural sac imaging should be reserved for MFS suspicions with the absence of those manifestations in order to establish the diagnosis according to the Ghent criteria. Thus, the present study supports the recent downgrading of dural ectasia to a contributor to the systemic score.
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