Loeys–Dietz syndrome (LDS) is an
autosomal dominant genetic
connective tissue disorder. The disorder is marked by
aneurysms in the
aorta, often in children. The aorta may also undergo sudden
dissection in the weakened layers of the wall of aorta. Aneurysms and dissections also can occur in
arteries other than the aorta. Because aneurysms in children tend to rupture early, children are at greater risk for dying if the syndrome is not identified.
Surgery to repair aortic aneurysms is essential for treatment.