Tordis Böker1, Thy Thy Vanem2, Are Hugo Pripp3, Svend Rand-Hendriksen4, Benedicte Paus5, Hans-Jørgen Smith6, Rigmor Lundby7. 1. Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway; Department of Radiology and Nuclear Medicine, Oslo University Hospital, Ullevål, PO BOX 4956 Nydalen, Oslo 0424, Norway. Electronic address: tordis.boker@medisin.uio.no. 2. Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway; Department of Cardiothoracic Surgery, Oslo University Hospital, Oslo, Norway. 3. Oslo Centre for Biostatistics and Epidemiology, Oslo University Hospital, Oslo, Norway. 4. TRS, National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, Nesoddtangen, Norway. 5. Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway; Department of Medical Genetics, Oslo University Hospital, Oslo, Norway. 6. Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway. 7. Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway; Department of Radiology and Nuclear Medicine, Oslo University Hospital, Ullevål, PO BOX 4956 Nydalen, Oslo 0424, Norway.
Abstract
BACKGROUND CONTEXT: Dural ectasia is widening of the dural sac often seen in patients with Marfan syndrome and other hereditary connective tissue disorders. Dural ectasia can cause specific symptoms and is associated with surgical complications. The knowledge on how and at which age dural ectasia develops is incomplete. There is no established gold standard for diagnosing dural ectasia, making it difficult to compare results from different studies. PURPOSE: Our primary aim was to explore whether the radiological findings of dural ectasia changed after 10 years in an adult cohort with suspected Marfan syndrome. Our secondary aim was to re-evaluate the radiological criteria of dural ectasia. STUDY DESIGN: Prospective cohort study. PATIENT SAMPLE: Sixty-two persons from a cross-sectional study of 105 persons with suspected Marfan syndrome were included in a 10-year follow-up of dural ectasia. Forty-six were diagnosed with Marfan syndrome, 7 with Loeys-Dietz syndrome, and 5 with other hereditary connective tissue disorders. For comparison 64 matched hospital controls were evaluated. OUTCOME MEASURES: Previously used radiological criteria for dural ectasia based on quantitative measurements of the lumbosacral spine. METHODS: MRI of the lumbosacral spine was performed if not contraindicated, and if so then CT was performed. Differences in the study group between baseline and follow-up were assessed with paired Student t test, Wilcoxon rank signed test, and McNemar test. Receiver operating characteristic curves were constructed to assess the ability of radiological measurement to differentiate between the study and control group. RESULTS: Fifty-two of 58 patients with hereditary connective tissue disorders and 11 controls had dural ectasia at follow-up. Forty-five Marfan patients had dural ectasia at follow-up vs. 41 at baseline. Five Loeys-Dietz patients had dural ectasia at follow-up vs. four at baseline. Twenty-four Marfan and 2 Loeys-Dietz patients had anterior sacral meningocele at follow-up, compared with 21 and 1, respectively, at baseline. Three Marfan patients developed herniation of a nerve root sleeve during follow-up. This was not seen in other individuals. The dural sac ended significantly lower at follow-up, and the dural sac ratio at level L5 was significantly increased from baseline in the Marfan patients. CONCLUSIONS: In Marfan and Loeys-Dietz syndrome, dural ectasia may present or worsen during adulthood. The cut-off value of dural sac ratio at level S1 is suggested elevated to 0.64. The results from the present study may help as guidance for appropriate follow-up of patients with dural ectasia.
BACKGROUND CONTEXT: Dural ectasia is widening of the dural sac often seen in patients with Marfan syndrome and other hereditary connective tissue disorders. Dural ectasia can cause specific symptoms and is associated with surgical complications. The knowledge on how and at which age dural ectasia develops is incomplete. There is no established gold standard for diagnosing dural ectasia, making it difficult to compare results from different studies. PURPOSE: Our primary aim was to explore whether the radiological findings of dural ectasia changed after 10 years in an adult cohort with suspected Marfan syndrome. Our secondary aim was to re-evaluate the radiological criteria of dural ectasia. STUDY DESIGN: Prospective cohort study. PATIENT SAMPLE: Sixty-two persons from a cross-sectional study of 105 persons with suspected Marfan syndrome were included in a 10-year follow-up of dural ectasia. Forty-six were diagnosed with Marfan syndrome, 7 with Loeys-Dietz syndrome, and 5 with other hereditary connective tissue disorders. For comparison 64 matched hospital controls were evaluated. OUTCOME MEASURES: Previously used radiological criteria for dural ectasia based on quantitative measurements of the lumbosacral spine. METHODS: MRI of the lumbosacral spine was performed if not contraindicated, and if so then CT was performed. Differences in the study group between baseline and follow-up were assessed with paired Student t test, Wilcoxon rank signed test, and McNemar test. Receiver operating characteristic curves were constructed to assess the ability of radiological measurement to differentiate between the study and control group. RESULTS: Fifty-two of 58 patients with hereditary connective tissue disorders and 11 controls had dural ectasia at follow-up. Forty-five Marfan patients had dural ectasia at follow-up vs. 41 at baseline. Five Loeys-Dietz patients had dural ectasia at follow-up vs. four at baseline. Twenty-four Marfan and 2 Loeys-Dietz patients had anterior sacral meningocele at follow-up, compared with 21 and 1, respectively, at baseline. Three Marfan patients developed herniation of a nerve root sleeve during follow-up. This was not seen in other individuals. The dural sac ended significantly lower at follow-up, and the dural sac ratio at level L5 was significantly increased from baseline in the Marfan patients. CONCLUSIONS: In Marfan and Loeys-Dietz syndrome, dural ectasia may present or worsen during adulthood. The cut-off value of dural sac ratio at level S1 is suggested elevated to 0.64. The results from the present study may help as guidance for appropriate follow-up of patients with dural ectasia.
Authors: Lily Pollock; Ashley Ridout; James Teh; Colin Nnadi; Dionisios Stavroulias; Alex Pitcher; Edward Blair; Paul Wordsworth; Tonia L Vincent Journal: Curr Rheumatol Rep Date: 2021-11-26 Impact factor: 4.592