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      What Each Clinical Anatomist Has to Know about Left Renal Vein Entrapment Syndrome (Nutcracker Syndrome): A Review of the Most Important Findings

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          Abstract

          Nutcracker syndrome (NCS) is the most common term for compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. The development of NCS is associated with the formation of the left renal vein (LRV) from the aortic collar during the sixth to eighth week of gestation and abnormal angulation of the superior mesenteric artery from the aorta. Collateralization of venous circulation is the most significant effect of NCS. It includes mainly the left gonadal vein and the communicating lumbar vein. Undiagnosed NCS may affect retroperitoneal surgery and other radiological and vascular procedures. The clinical symptoms of NCS may generally be described as renal presentation when symptoms like haematuria, left flank pain, and proteinuria occur, but urologic presentation is also possible. Radiological methods of confirming NCS include Doppler ultrasonography as a primary test, retrograde venography, which can measure the renocaval pressure gradient, computed tomography angiography, which is faster and less traumatic, intravascular ultrasound, and magnetic resonance angiography. Treatment can be conservative or surgical, depending on the severity of symptoms and degree of LRV occlusion. Nutcracker syndrome is worth considering especially in differential diagnosis of haematuria of unknown origin.

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          Most cited references53

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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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            Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.

            Nutcracker syndrome (NCS) describes left renal vein compression between the superior mesenteric artery and the aorta. Although uncommon, it is an important diagnosis due to the important morbidity associated with it, including the risk of chronic kidney disease from long-term left renal vein (LRV) hypertension and the risk of LRV thrombosis.
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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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                Author and article information

                Contributors
                Journal
                Biomed Res Int
                Biomed Res Int
                BMRI
                BioMed Research International
                Hindawi
                2314-6133
                2314-6141
                2017
                11 December 2017
                : 2017
                : 1746570
                Affiliations
                1Department of Angiology, Medical University of Łódź, Narutowicza 60, 90-136 Łódź, Poland
                2Department of Normal and Clinical Anatomy, Medical University of Łódź, Narutowicza 60, 90-136 Łódź, Poland
                3Department of Radiological and Isotopic Diagnosis and Therapy, Medical University of Łódź, ul. Żeromskiego 113, 90-549 Łódź, Poland
                4Department of Radiology, Medical University of Łódź, Kopcińskiego 22, 90-153 Łódź, Poland
                Author notes

                Academic Editor: Hossein Tezval

                Author information
                http://orcid.org/0000-0003-4146-4998
                Article
                10.1155/2017/1746570
                5742442
                8bc76a0b-5691-48da-8199-db43c594c98b
                Copyright © 2017 Krzysztof Orczyk et al.

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

                History
                : 14 April 2017
                : 12 October 2017
                Categories
                Review Article

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