Aneurysms in EDS

[Allyson]

Aneurysms are know to be more common in those with EDS - and not just in the vascular type

CONTINUUM: Lifelong Learning in Neurology:
April 2014 - Volume 20 - Issue 2, Cerebrovascular Disease - p 387-398
doi: 10.1212/01.CON.0000446108.12915.65
Review Articles
Unruptured Intracranial Aneurysms: Screening and Management

Kelly, Adam G. MD





Abstract

Purpose of Review:

Unruptured intracranial aneurysms are found commonly in the general public, and more frequently in certain populations. This article focuses on the epidemiology, screening strategies, and management options for patients with unruptured aneurysms.

Recent Findings:

Recent epidemiologic studies show the overall prevalence of intracranial aneurysms to be approximately 3%, with higher rates seen in familial aneurysm syndromes and in certain medical conditions, such as autosomal dominant polycystic kidney syndrome and Ehlers-Danlos Syndrome. Aneurysm treatment may include surgical or endovascular techniques, with increasing utilization of endovascular strategies over time. Increased aneurysm diameter, certain locations, and other anatomical considerations may be associated with higher risks of aneurysm rupture.

Summary:

Given the high morbidity and mortality associated with aneurysm rupture, screening for unruptured aneurysms is generally recommended for high-risk patients (patients who have at least two first-degree relatives with aneurysms, and patients with autosomal dominant polycystic kidney disease). Screening may be considered for other patients (eg, one first-degree relative with aneurysm) after discussion of the risks and benefits of imaging. Following identification of an aneurysm, decisions regarding observation or treatment should be based on patient characteristics, features of the aneurysm, and provider expertise.



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[Allyson]

and in pregnancy EDS ers need to take special care - again all types not just vascular

http://dx.doi.org/10.1016/j.bpobgyn.2014.03.007
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In addition to the haemodynamic changes in pregnancy, hormones also induce changes in the aortic wall. Women with diseases like Marfan syndrome, Ehlers–Danlo syndrome, or other aortic abnormalities, have an increased risk of complications during pregnancy. Counselling and risk assessment before pregnancy is mandatory for all women with known aortic disease. Proper information should be provided about the risks of morbidity and mortality during pregnancy and information on the risks for the fetus, including the potential recurrence of disease in the offspring. Evaluation of past medical and family history, the aortic size before conception, and any increase in size before and during pregnancy, is essential to try and estimate the risk of aortic dissection. If the aorta is dilated, prophylactic repair before pregnancy may be indicated. In some cases, elective surgery during pregnancy may be warranted. In women with a severely dilated ascending aorta, caesarean section is, at present, the advised mode of delivery.


Keywords

• pregnancy;
• aortic aneurysm;
• dissection;
• Marfan syndrome;
• Ehlers–Danlos syndrome;
• Turner syndrome;
• Loeys–Dietz syndrome;
• Smad3 protein

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