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Loeys-Dietz syndrome: MDCT angiography findings Johnson PT; Chen JK; Loeys BL; Dietz HC; Fishman EKAJR Am J Roentgenol 2007[Jul]; 189 (1): W29-35OBJECTIVE: Loeys-Dietz syndrome is a newly described phenotype caused by heterozygous mutations in the genes encoding type I or II transforming growth factor-beta (TGF-beta) receptor. Characterized by a unique constellation of clinical and pathologic findings, Loeys-Dietz syndrome manifests with aggressive vascular pathology. Aneurysms may form at a young age and have a propensity for arterial dissection. In addition, aneurysms rupture at diameters smaller than those used to dictate surgical intervention for other syndromes and disorders. This article presents the spectrum of arterial pathology that may be identified on MDCT angiography in patients with Loeys-Dietz syndrome. CONCLUSION: For patients with Loeys-Dietz syndrome, early diagnosis and rapid intervention are instrumental in averting catastrophic events. Serial imaging assessment by radiologists is an important component in the management of these patients.|Abnormalities, Multiple/*diagnostic imaging[MESH]|Adolescent[MESH]|Adult[MESH]|Angiography/instrumentation/*methods[MESH]|Aortic Aneurysm, Thoracic/*diagnostic imaging[MESH]|Aortic Dissection/*diagnostic imaging[MESH]|Child[MESH]|Child, Preschool[MESH]|Female[MESH]|Humans[MESH]|Image Enhancement/instrumentation/*methods[MESH]|Infant[MESH]|Male[MESH]|Middle Aged[MESH]|Syndrome[MESH]|Tomography, X-Ray Computed/instrumentation/*methods[MESH] |
