Renal Artery Stenosis Complicating Polycystic Kidney Disease

Abstract

Polycystic kidney disease (PCKD) commonly causes hypertension and leads to chronic renal insufficiency. Correctable renal artery stensosis can coexist with PCKD and contribute to both hypertension and progressive decline in renal function but is often overlooked. To illustrate the diagnostic and therapeutic implications of this unusual combination of pathology, the authors present the case of a 59-year-old woman with severe PCKD who presented with recurrent bouts of malignant hypertension, flash pulmonary edema, and accelerated decline of renal function over a 3-month period. In the course of normal findings from cardiac catheterization, aortography demonstrated severe right renal artery stenosis. Captopril renal scintigraphy confirmed the viability of both kidneys and the physiologic significance of the right renal artery stenosis even in the setting of chronic renal insufficiency. Right renal artery revascularization was achieved by using a hepatorenal bypass with reversed greater saphenous vein. The patient had an immediate improvement in renal function and blood pressure control as evidenced by a decline in creatinine and a reduction of medication requirements, respectively. In 15 months' follow-up, this therapeutic result has been maintained and she has not experienced any further bouts of pulmonary edema. This unusual case emphasizes the need to pursue correctable pathology of the renal arteries despite the presence of chronic renal insufficiency unrelated to vascular occlusive disease in order to preserve renal function and stabilize hypertension. Patients with multiple etiologies of renal dysfunction represent diagnostic and therapeutic challenges that are not insurmountable but require diligence in pursuing repairable lesions when their clinical course deviates from that expected from the uncorrectable pathology.