Retroperitoneoskopische Nephrektomie bei einem Neugeborenen wegen Faktor-V-Leiden-Mutation und in utero vena cava inferior und Nierenvenenthrombose: erster Fallbericht

Abstract

BACKGROUND: Renal vein thrombosis is a well-recognized but now rare clinical entity in children. METHODS (CASE REPORT): We report successful retroperitoneoscopic nephrectomy for a non functioning kidney secondary to in-utero inferior vena cava and renal vein thrombosis due to heterozygosity for factor V Leiden mutation leading to resistance to activated protein C, and discuss the associated prothrombotic genetic risk factors, safety and feasibility of the procedure in the presence of abnormal retroperitoneal venous collaterals and describe the difficulties faced by the laparoscopic surgeon when entering into a retroperitoneal space with collateral vessels brought on by an old standing blockage of a major vein. RESULTS: To our knowledge, retroperitoneoscopic nephrectomy has never been reported in children with thrombophilic mutation and IVC thrombosis. CONCLUSIONS: The retroperitoneal approach can be performed safely in the presence of renal vein and IVC thrombosis. The relatively long operating time is necessary for hemostasis in the presence of abnormal retroperitoneal venous collaterals.