Renal peripelvic lymphangiectasia after bilateral renal vein thrombosis

Abstract

Renal peripelvic lymphangiectasia (RPL) is one of the rare conditions that mimic renal cysts. Its physiopathology remains unknown, but an association with renal vein thrombosis has been reported. We share the case of a male patient in his 20s suffering from antiphosphlipid syndrome. The patient was hospitalised for thrombosis of the inferior vena cava (IVC) extending from the iliac veins to the level of renal veins. Consecutive CT and clinical follow-up over the course of 14 years showed the development of numerous retroperitoneal venous collaterals and the apparition of several bilateral peripelvic cystic lesions after extensive thrombosis of the IVC and both renal veins. The renal function remained normal throughout the follow-up. We suggest that the development of RPL is secondary to bilateral renal vein thrombosis. The presumed mechanism would be an increased hydrostatic pressure in the kidney capillaries leading to a more important interstitial fluid drainage by the lymphatic system. To our knowledge, this is the first well-documented case of renal vein thrombosis followed by RPL, contrasting with the previous hypothesis that compression by the lymphangiectasia could cause the thrombosis.

Development of unilateral renal peripelvic lymphangiectasia after renal vein thrombosis

“…Although no consensus exists on the underlaying mechanism leading to lymphatic ectasia, it is presumed that in adults an acquired etiology is more prevalent. Various reports have associated renal vein thrombosis with the development of RLT [4] , [5] , [6] . One report documented the case of a young male that developed bilateral renal vein thrombosis secondary to antiphospholipid syndrome [4] .…”

“…Various reports have associated renal vein thrombosis with the development of RLT [4] , [5] , [6] . One report documented the case of a young male that developed bilateral renal vein thrombosis secondary to antiphospholipid syndrome [4] . Subsequent longitudinal monitoring spanning 14 years revealed the progressive development of RLT in this patient.…”

“…Another case reported a middle-aged male with a history of chronic renal vein thrombosis with multiple perirenal collaterals that was incidentally noted to have RLT after an evaluation for abdominal pain [7] . Some authors have proposed that an elevation in starling hydrostatic pressure within the renal capillaries owing to thromboembolic events in the renal vessels augments trans-endothelial filtration, resulting in increased lymphatic drainage and the formation of ecstatic channels [4] .…”

“…Several reports have documented instances of complicated RLT encompassing refractory HTN, pleural effusion, ascites, rupture after abdominal blunt trauma, and renal insufficiency and anemia [ 8 , [12] , [13] , [14] ]. It is noteworthy that certain reports identify renal vein thrombosis as a complication and a potential etiology [4] , [5] , [6] .…”

“…MN, the leading cause of nephrotic syndrome in adults, is strongly linked to SLE and renal vein thrombosis [ 21 ]. While the literature on RLT is scant, a few case reports have described an association with renal vein thrombosis [4] , [5] , [6] . However, given the intricate profile of our patient, it remains uncertain whether an autoimmune factor played a role in the accelerated development of RLT.…”