Renal lymphangiectasia is a rare disorder characterized by ectasia of perirenal, peripelvic and intrarenal lymphatic vessels. A disturbance in the drainage of renal sinus lymphatics results in ectasia of peripelvic, intrarenal and perirenal lymphatics, and leads to the formation of renal lymphangiectasia. Multiple cysts with various sizes are seen in the perinephric space, renal parenchyma and in the renal sinus. The presence of fluid, density lesions in the retroperitoneum and around the great vessels, crossing the midline at the level of the origin of renal vessels are the diagnostic criteria. We report a case of a 27-year old female with multiple tiny cysts in the perinephric space and renal cortex, and a large cyst in the fat of the renal sinus, discovered with sonography, confirmed with CT Urography and MRI.
Keywords: renal lymphangiectasia, peripelvic cyst, renal lymphangioma, intrarenal and perinephric cyst.