Loeys-Dietz Syndrome (cont.)Medical Author:
Melissa Conrad Stöppler, MD
Melissa Conrad Stöppler, MDMelissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology. Medical Editor:
Jay W. Marks, MD
Jay W. Marks, MDJay W. Marks, MD, is a board-certified internist and gastroenterologist. He graduated from Yale University School of Medicine and trained in internal medicine and gastroenterology at UCLA/Cedars-Sinai Medical Center in Los Angeles. In this Article
How is Loeys-Dietz syndrome treated?
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The only treatment for Loeys-Dietz syndrome to prolong life expectancy is surgical repair of the aortic aneurysm. Surgical repair of the aneurysms is generally successful. Since the aneurysms tend to rupture early, early and accurate diagnosis is critical to ensure that affected individuals receive prompt surgical treatment. The genetic test can be of value in identifying which individuals with aortic aneurysms have Loeys-Dietz syndrome, and therefore, should have immediate surgery. In contrast to Loeys-Dietz syndrome, in other inherited syndromes associated with aortic aneurysms, surgery carries a poorer prognosis, and the aneurysms can be managed with medications for a longer period of time before surgery becomes necessary. Studies are ongoing to determine whether drug treatment of the Loeys-Dietz syndrome also may be of value. At the time of diagnosis, imaging studies of the aorta are recommended and should be repeated after 6 months to determine if aortic enlargement is occurring. If the diameter of the aorta is not enlarging, yearly magnetic resonance imaging (MRI) scans from the of the circulation from the barin to the pelvis are recommended, since affected persons commonly develop aneurysms that are treatable surgically. Loeys-Dietz Syndrome At A Glance
References: Last Editorial Review: 9/3/2010 Patient CommentsViewers share their comments
Loeys-Dietz Syndrome - Signs and Symptoms
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Loeys-Dietz Syndrome - Treatment
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