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In conclusion, about one-quarter to one-third of Norwegian hospital doctors seem to disagree with current guidelines that all doctors should address their patients' smoking habits.

The age‐dependent penetrance of organ manifestations in Marfan syndrome (MFS) is not known. The aims of this follow‐up study were to explore how clinical features change over a 10‐year period in the same Norwegian MFS cohort. In 2003–2004, we investigated 105 adults for all manifestations in the 1996 Ghent nosology. Ten years later, we performed follow‐up investigations of the survivors (n = 48) who consented. Forty‐six fulfilled the revised Ghent criteria. Median age: females 51 years, range 32–80 years; males 45 years, range 30–67 years. New aortic root dilatation was detected in patients up to 70 years. Ascending aortic pathology was diagnosed in 93 versus 72% at baseline. Sixty‐five percent had undergone aortic surgery compared to 39% at baseline. Pulmonary trunk mean diameter had increased significantly compared to baseline. From inclusion to follow‐up, two patients (three eyes) developed ectopia lentis, four developed dural ectasia, four developed scoliosis, three developed incisional or recurrent herniae, and 14 developed hindfoot deformity. No changes were found regarding protrusio acetabuli, spontaneous pneumothorax, or striae atrophicae. The study confirms that knowledge of incidence and progression of organ manifestations throughout life is important for diagnosis, treatment, and follow‐up of patients with verified or suspected MFS.

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.

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