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Pediatr Radiol. 2011 Dec;41(12):1495-504; quiz 1616. doi: 10.1007/s00247-011-2195-z. Epub 2011 Jul 23.

Loeys-Dietz syndrome: cardiovascular, neuroradiological and musculoskeletal imaging findings.

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  • 1Department of Diagnostic Radiology, Yale-New Haven Medical Center, Box 208042, Tompkins East 2, 333 Cedar St., New Haven, CT 06520-8042, USA.

Abstract

Loeys-Dietz syndrome (LDS) is an increasingly recognized autosomal-dominant connective tissue disorder with distinctive radiological manifestations, including arterial tortuosity/aneurysms, craniofacial malformations and skeletal abnormalities. LDS exhibits a more aggressive course than similar disorders, such as Marfan or the vascular subtype of Ehlers-Danlos syndrome, with morbidity and mortality typically resulting from complications of aortic/arterial dissections. Early diagnosis, short-interval follow-up imaging and prophylactic surgical intervention are essential in preventing catastrophic cardiovascular complications. This review focuses on the cardiovascular, neuroradiological and musculoskeletal imaging findings in this disorder and recommendations for follow-up imaging.

PMID:
21785848
[PubMed - indexed for MEDLINE]
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