Combined Nutcracker and Ehlers-Danlos Syndromes: A Case Report

INTRODUCTION: Nutcracker 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. REPORT: A 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. CONCLUSION: Ehlers–Danlos syndrome can cause nutcracker syndrome and may give rise to visceral pain of mixed origin.