Catalog

Aortic aneurysm syndrome, Loeys-Dietz type

No MMS code

Link to WHO Definition

Aortic aneurysm syndrome, Loeys-Dietz type – http://id.who.int/icd/entity/999490064

Loeys-Dietz syndrome type 1 is a Marfan-related disorder characterised by the association of aortic aneurysms, hypertelorism (widely spaced eyes), cleft palate and/or bifid uvula and generalised arterial tortuosity. Other findings include craniosynostosis, exotropy, micrognathia and retrognathia, structural brain abnormalities, intellectual deficit, congenital heart disease, translucent skin, joint hyperlaxity and aneurysm with dissection throughout the arterial tree.