Combined nutcracker and Ehlers-Danlos syndromes: A case report

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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 EhlerseDanlos syndrome made, possibly explaining the mobility of viscera and unusual compression of the left renal vein by the portal vein. Conclusion: EhlerseDanlos syndrome can cause nutcracker syndrome and may give rise to visceral pain of mixed origin. Ó 2020 The Author(s). Published by Elsevier Ltd on behalf of European Society for Vascular Surgery. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Article history: Received 23 October 2019, Revised 7 January 2020, Accepted 17 February 2020, Keywords: Nutcracker syndrome, Low back pain, Pelvic pain, Diagnosis, Treatment

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