Update on the Diagnosis and Management of Colon Ischemia

Abstract

Marfan syndrome is a common, heritable disorder of connective tissuetransmitted as an autosomal dominant trait with variable penetrance. Themolecular defect is related to abnormalities of fibrillin, an integralconnective tissue protein. Clinical manifestations includemusculoskeletal, ophthalmologic, and cardiovascular involvement.Cardiovascular involvement generally determines survival and includessevere mitral valve prolapse, mitral insufficiency, and aortic dilation.Affected individuals generally manifest aortic root involvement in theirteens and twenties; the lesion exhibits an unpredictable growth rate andresults in aortic aneurysm formation. The mean age of death is 32 years.Sudden death in Marfan syndrome is related in over 70% of cases toaortic rupture or dissection. Therefore, healthcare professionals mustfollow these individuals closely, with aggressive blood pressure andheart rate control as well as serial echocardiography with attention toaortic root size. Prophylactic aortic root replacement should beperformed when the aortic root is found to be greater than 55 mm indiameter. The patient should be referred to a specialized center with acardiovascular surgeon who has experience in aortic root replacement.