Arterial dissections in autosomal dominant polycystic kidney disease – chance association or part of the disease spectrum?

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) has been associated with a number of vascular abnormalities of which the most widely known is intracranial aneurysm (Berry aneurysm). Arterial dissections involving both the thoracic and abdominal aorta, coronary, cervical and vertebral arteries have been anecdotally reported usually within the context of a medical emergency. As hypertension is a frequent early accompaniment of ADPKD (50 - 70% of nonazotemic patients) the occurrence of these dissections has commonly been attributed to hypertension. However, the question arises whether ADPKD patients have an inherited predisposition to the development of arterial dissections or are the latter alternatively part of the spectrum of extrarenal manifestations of ADPKD? We report the case of a 61-year-old woman with ADPKD on maintenance hemodialysis in whom asymptomatic dissection of the abdominal aorta was first suspected on a routine abdominal ultrasound performed as part of her pretransplantation checkup. A computed tomographic angiogram (CTA) showed the presence of polycystic kidney and liver disease and confirmed the existence of synchronous dissection of the left subclavian artery and the abdominal aorta beginning below the renal arteries and extending to the bifurcation and left common iliac artery. Although the patient was hypertensive, her blood pressure was well controlled on relatively mild antihypertensive medication. This case highlights the association of arterial dissection in ADPKD and provides the stage for discussing the question posed above.