Dural Ectasia as an Additional Prognostic Marker of Marfan's Syndrom in Children

Objectives: In children, Marfan's syndrome (MFS) is often diagnosed long before dilatation of the aorta occurs. It is difficult to predict which patient will have a relevant dilatation of the aorta and thus require drug therapy. In this study, we investigate whether dural ectasia as a typical symptom of MFS can be used as an additional prognostic parameter.

Methods: In our pediatric Marfan's clinic, we diagnosed MFS in 202 patients (9.3 ± 6 year). We retrospectively analyzed data and the presence of dural ectasia via MRI in 103 patients with a genetic and/or clinical diagnosis of MFS according to the Ghent criteria and analyzed the need for medical prophylaxis (Beta-blocker, sartan, or both) of aortic dilatation during follow-up.

Result: In our cohort of patients with MFS 63 had dural ectasia (61%; 13.6 ± 4.4 years) and 40 (13.49 ± 4.0 years) did not. Of those having dural ectasia 45 (71%) needed medical treatment because of dilatation of the aorta versus 13 (32%) of those without dural ectasia (p < 0.0002; odds ratio = 5.1 (2.11–11.78; Table 1).

Conclusion: In this single-center, pediatric population of patients with MFS, the presence of dural ectasia is significantly associated with the need for prophylactic drug therapy in the course of further care. Despite the fact, that dural ectasia is not a main criteria in the revised Ghent Nosology anymore, this symptom must be used as a prognostic marker. As soon as children are able to receive an MRI examination without sedation, it should be performed. If a dura ectasia is present, prophylactic drug therapy should be started generously.

Table 1 Number of patients with MFS and dural ectasia and need for medical treatment during follow-up

	Med positive (n)	Med negative (n)	Total	p-Value
Dura positive (n)	45	18	63
Dura negative (n)	13	27	40
Total	58	45	103	<0.0002

Publication History

Article published online:
21 February 2021