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      Nutcracker Syndrome Accompanying Pelvic Congestion Syndrome; Color Doppler Sonography and Multislice CT Findings: A Case Report

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          Abstract

          Nutcracker syndrome (NCS) is a rare pathology, caused by compression of the left renal vein (LRV) between the abdominal aorta (AA) and the superior mesenteric artery (SMA), due to reduction of the angle between AA and SMA. This leads to LRV varices, left gonadal vein varices and therefore, the pelvic congestion syndrome. For this reason, coexistence of NCS and pelvic congestion syndrome has been described. It manifests by hematuria, proteinuria, and nonspecific pelvic pain secondary to pelvic congestion, dyspareunia and persistent genital arousal. We report a 27-year-old woman who experienced hematuria and left flank pain. The diagnosis of NCS accompanied by pelvic congestion syndrome was missed initially, but later on the diagnosis was made by color Doppler ultrasound, abdominal computed tomography (CT) and CT angiography that were later performed. She refused interventional and surgical treatments, and was lost to follow up.

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          Nutcracker phenomenon and nutcracker syndrome.

          Nutcracker phenomenon refers to compression of the left renal vein, most commonly between the aorta and the superior mesenteric artery, with impaired blood outflow often accompanied by distention of the distal portion of the vein. The nutcracker syndrome (NCS) is the clinical equivalent of nutcracker phenomenon characterized by a complex of symptoms with substantial variations. Depending on specific manifestations, NCS may be encountered by different medical specialists. Although it may be associated with substantial morbidity, the diagnosis of NCS is often difficult and is commonly delayed. Diagnostic and treatment criteria are not well established, and the natural history of NCS is not well understood. We performed an initial review of the literature through MEDLINE, searching from 1950 to date and using the keywords nutcracker syndrome, nutcracker phenomenon, and renal vein entrapment. We performed additional reviews based on the literature citations of the identified articles. We attempted to elucidate clinical relevance of these conditions and their prominent features and to summarize professional experience.
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            Current trends in the diagnosis and management of renal nutcracker syndrome: a review.

            Nutcracker syndrome is caused by compression of the left renal vein between the aorta and the superior mesenteric artery where it passes in the fork formed at the bifurcation of these arteries. The phenomenon results in left renal venous hypertension. The syndrome is manifested by left flank and abdominal pain, with or without unilateral haematuria. Other common presentation is as "pelvic congestion syndrome" characterized by symptoms of dysmenorrhea, dyspareunia, post-coital ache, lower abdominal pain, dysuria, pelvic, vulvar, gluteal or thigh varices and emotional disturbances. Likewise compression of the left renal vein can cause left renal-to-gonadal vein reflux resulting in lower limb varices and varicoceles in males. Its diagnosis is based on history and physical examination, basic lab tests to exclude other causes of haematuria, cystoscopy and ureteroscopy to confirm unilateral haematuria and exclude other causes of this sinister symptom. Sequence of imaging has more or less been rationalised to USS with Doppler studies, CT or MR angiography and finally phlebography with renal vein and IVC manometery to confirm the diagnosis.
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              Diagnostic value of computed tomographic findings of nutcracker syndrome: correlation with renal venography and renocaval pressure gradients.

              To evaluate the diagnostic values of CT findings of nutcracker syndrome (NCS). Twenty seven subjects that underwent CT and renal venography, were divided into three groups based on the venographic renocaval pressure gradient (PG) and collateral veins of the left renal vein (LRV): non-compensated NCS patients with PG≥3 mm Hg (group 1, n=12), partially compensated NCS patients with borderline PG (1<3 mm Hg) and collateral veins (group 2, n=6), and control group with low PG (0-1 mm Hg) without collateral veins (group 3, n=9). The CT findings were analyzed with regard to abrupt narrowing of the LRV with an acute angle (beak sign), aortomesenteric angle between the superior mesenteric artery and aorta on sagittal images, and LRV diameter ratio (hilar-aortomesenteric). Beak sign of the LRV was found in 91.7% (11/12) of group 1, 50% (3/6) of group 2, and in 11.1% (1/9) of group 3 with the significant difference between groups 1 and 3 (P<.05, χ2 test). Mean values of all quantitative CT parameters differed significantly only between groups 1 and 3 (P<.05, one-way ANOVA test). For differentiating the non-compensated NCS from the control group, the beak sign showed 91.7% sensitivity and 88.9% specificity. Of the various CT parameters, the beak sign and LRV diameter ratio of ≥4.9 showed the greatest diagnostic accuracy (AUC 0.903, ROC analysis). Beak sign of the LRV and CT findings can be useful in diagnosing the non-compensated NCS. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.
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                Author and article information

                Journal
                Iran J Radiol
                Iran J Radiol
                10.5812/iranjradiol
                Kowsar
                Iranian Journal of Radiology
                Kowsar
                1735-1065
                2008-2711
                15 May 2014
                May 2014
                : 11
                : 2
                : e11075
                Affiliations
                [1 ]Department of Radiology, Kirikkale University, Yahsihan, Kirikkale, Turkey
                Author notes
                [* ]Corresponding author: Mikail Inal, Department of Radiology, Kirikkale University, Yahsihan, Kirikkale, Turkey, Tel: +90 0318 2252485, Fax: +90 0318 2252484, E-mail: inal_m@ 123456hotmail.com
                Article
                10.5812/iranjradiol.11075
                4090635
                25035694
                aac9a712-0a6a-4964-88d6-41fabefaeb54
                Copyright © 2014, Tehran University of Medical Sciences and Iranian Society of Radiology; Published by Kowsar Corp.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 05 March 2013
                : 04 April 2013
                : 08 May 2013
                Categories
                Abdominal Imaging

                Radiology & Imaging
                hematuria,renal nutcracker syndrome,flank pain
                Radiology & Imaging
                hematuria, renal nutcracker syndrome, flank pain

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