Combined Nutcracker and Ehlers-Danlos Syndromes: A Case Report

• Published in: EJVES vascular forum Vol. 47; pp. 12 - 17
• Main Authors: Amato, Alexandre Campos Moraes, da Silva, Arthur Ethan Cardoso, Bernal, Isabela Moraes, de Oliveira, Julia Cuginotti, Di Paschoal Almeida Ribeiro, Maísa, Schinzari, Priscila Souza, Dos Santos, Ricardo Virgínio
• Format: Report
• Language: English
• Published: 01-01-2020
• ISSN: 2666-688X
• DOI: 10.1016/j.ejvsvf.2020.02.005

INTRODUCTIONNutcracker syndrome refers to the clinical manifestations of left renal vein compression between the superior mesenteric artery and the abdominal aorta, causing urinary changes and low back pain. REPORTA 44 year old woman presented with low back and pelvic pain. Following the diagnosis of nutcracker syndrome, she underwent endovascular treatment with renal vein stent placement; however, the patient continued to complain of pain. Further examinations revealed left renal vein compression by the portal vein. The patient underwent a second procedure; however, improvement was temporary and her pain returned. Further investigation revealed previously undetected nephroptosis and hyperelasticity. A diagnosis of Ehlers-Danlos syndrome made, possibly explaining the mobility of viscera and unusual compression of the left renal vein by the portal vein. CONCLUSIONEhlers-Danlos syndrome can cause nutcracker syndrome and may give rise to visceral pain of mixed origin.