Aortic dissection associated with segmental arterial mediolysis in polycystic kidney disease

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is characterised by progressive cyst formation in the kidney leading to end-stage renal disease (ESRD) in approximately 50% of affected individuals. Although spontaneous artery dissection has been described in patients with ADPKD, it is not a well-recognized feature of the disease. Because it occurs often in ADPKD patients with uncontrolled hypertension, aorta dissection is regarded more as a complication than as a consequence of the genetic disorder itself. Nevertheless, the frequent presence of vascular abnormalities and sparse reports of familial clustering of arterial dissection in ADPKD patients suggests that ADPKD, as a multi-system connective tissue disorder, also directly involves the arterial wall [ 1 Bobrie G. Brunet-Bourgin F. Alamowitch S. et al. Spontaneous artery dissection: is it part of the spectrum of autosomal dominant polycystic kidney disease?. Nephrol Dial Transplant. 1998; 13: 2138-2141 Crossref PubMed Scopus (33) Google Scholar , 2 Somlo S. Rutecki G. Giuffra L.A. et al. A kindred exhibiting cosegregation of an overlap connective tissue disorder and the chromosome 16-linked form of autosomal dominant polycystic kidney disease. J Am Soc Nephrology. 1993; 4: 1371-1378 PubMed Google Scholar , 3 Biagini A. Maffei S. Baroni M. et al. Familial clustering of aortic dissection in polycystic kidney disease. Am J cardiol. 1993; 72: 741-742 Abstract Full Text PDF PubMed Scopus (36) Google Scholar ]. In this report, we present a case of thoracic aortic dissection associated with segmental lysis of the media in a middle-aged non-hypertensive woman with ADPKD.