, Volume 19, Issue 6, pp 553-556
Date: 20 Apr 2012

Aortic dissection in osteogenesis imperfecta: case report and review of the literature

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Introduction

Osteogenesis imperfecta (OI) is a group of hereditary connective tissue disorders affecting the biosynthesis of type I collagen [1, 2]. Although there is considerable phenotypic heterogeneity, the clinical hallmark of OI is osseous fragility with predisposition for fracture. Its extraskeletal manifestations often involve other collagen-rich structures, such as the skin, sclerae, dentition, middle and inner ear, tendons, and ligaments [2, 3]. Less commonly, the cardiovascular system may be affected [47].

The association between OI and valvular heart disease and aortic root dilatation has been well established. However, reports of aortic dissection in patients with OI remain rare; the first description of a Stanford A aortic dissection in the setting of OI was reported in 1993 by Ashraf et al. [8]; since that time, only three more proximal aortic dissections and one distal dissection [9] have been reported. We present a sixth case of aortic dissection in a patient with OI (th