Nutcracker syndrome
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Nutcracker syndrome is a vascular compression disorder that refers to the compression of the left renal vein most commonly between the superior mesenteric artery (SMA) and aorta, although other variations can exist 1. This can lead to renal venous hypertension, resulting in rupture of thin-walled veins into the collecting system with resultant haematuria.
Terminology
In certain situations, the syndrome can result from a retroaortic or circumaortic left renal vein.
Nutcracker syndrome should not be confused with superior mesenteric artery syndrome (Wilkie syndrome) also a superior mesenteric artery compression disorder, where the SMA compresses the third part of the duodenum (the two conditions, however, may be associated).
Epidemiology
There is a slightly greater female predilection.
Clinical presentation
The most common clinical manifestations of nutcracker syndrome are left flank pain, pelvic pain, hematuria and gonadal varices. Orthostatic proteinuria has also been reported. Hematuria can be microscopic or macroscopic. Haematuria should be from the left ureteric orifice only 4. In the absence of clinical symptoms, renal vein compression is referred to as nutcrackerNutcracker phenomenon.
Pathology
Associations
- may occur simultaneously with SMA syndrome
- an association with a thin or asthenic body habitus has long been noted.
Radiographic features
Radiographic features are similar on ultrasound, Doppler ultrasound, CT, MRI, and conventional angiography:
- reduced aortic-SMA angle
(the: normal angle between aorta and SMA isapproximately 45~45°(38(range 38-65°)) - left renal vein stenosis
- collateral pathways: main collateral pathway is the left gonadal vein which will display early enhancement during portal venous phase
. - pressure gradient >3 mm Hg on renal venography in early stage nutcracker syndrome. Well developed collateral veins dissipate the high
pressure-pressure gradient. - compression ratio (CR) given by the equation CR=P-C/C
-
i.e. diameter of pre-compressed vein (P) minus diameter of compressed vein (C) divided by the diameter of the compressed vein (C).
A -
a compression ratioabove 2.25 is highly sensitive and specific for Nutcracker syndrome 10
.
-
i.e. diameter of pre-compressed vein (P) minus diameter of compressed vein (C) divided by the diameter of the compressed vein (C).
Complications
Persistent haematuria can precipitate renal vein thrombosis 4.
Treatment and prognosis
Treatment should be started strictly when it is causing symptoms (haematuria and left flank pain). Surgical treatment modalities have their inherent complications and should be contemplated only when strongly indicated. A few of the reported surgical choices are:
- left renal vein transposition 5
- kidney autotransplant
- superior mesenteric artery transposition
- gonadal vein transposition
- nephrectomy
- extravascular stent placement
- endovascular stent-graft placement 6
History and etymology
The first clinical report of this syndrome was made by El-Sadr and Mina in 1950 while the term "nutcracker syndrome" is thought to have been first used by de Schepper in 1972 7.
See also
-<p><strong>Nutcracker syndrome</strong> is a <a href="/articles/vascular-compression-disorders">vascular compression disorder</a> that refers to the compression of the left renal vein most commonly between the <a href="/articles/superior-mesenteric-artery">superior mesenteric artery</a> (SMA) and <a href="/articles/aorta">aorta</a>, although other variations can exist <sup>1</sup>. This can lead to renal venous hypertension, resulting in rupture of thin-walled veins into the collecting system with resultant <a href="/articles/haematuria-adult">haematuria</a>.</p><h4>Terminology</h4><p>In certain situations, the syndrome can result from a <a href="/articles/retroaortic-left-renal-vein-1">retroaortic</a> or <a href="/articles/circumaortic-left-renal-vein">circumaortic left renal vein</a>.</p><p>Nutcracker syndrome should not be confused with <a href="/articles/superior-mesenteric-artery-syndrome">superior mesenteric artery syndrome</a> (Wilkie syndrome) also a <a href="/articles/superior-mesenteric-artery-compression-disorders">superior mesenteric artery compression disorder</a>, where the SMA compresses the third part of the duodenum (the two conditions however may be associated).</p><h4>Epidemiology</h4><p>There is a slightly greater female predilection.</p><h4>Clinical presentation</h4><p>The most common clinical manifestations of nutcracker syndrome are left flank pain, pelvic pain, hematuria and gonadal varices. Orthostatic proteinuria has also been reported. Hematuria can be microscopic or macroscopic. Haematuria should be from the left ureteric orifice only <sup>4</sup>. In the absence of clinical symptoms, renal vein compression is referred to as nutcracker phenomenon.</p><h4>Pathology</h4><h5>Associations</h5><ul>- +<p><strong>Nutcracker syndrome</strong> is a <a href="/articles/vascular-compression-disorders">vascular compression disorder</a> that refers to the compression of the left renal vein most commonly between the <a href="/articles/superior-mesenteric-artery">superior mesenteric artery</a> (SMA) and <a href="/articles/aorta">aorta</a>, although other variations can exist <sup>1</sup>. This can lead to renal venous hypertension, resulting in rupture of thin-walled veins into the collecting system with resultant <a href="/articles/haematuria-adult">haematuria</a>.</p><h4>Terminology</h4><p>In certain situations, the syndrome can result from a <a href="/articles/retroaortic-left-renal-vein-1">retroaortic</a> or <a href="/articles/circumaortic-left-renal-vein">circumaortic left renal vein</a>.</p><p>Nutcracker syndrome should not be confused with <a href="/articles/superior-mesenteric-artery-syndrome">superior mesenteric artery syndrome</a> (Wilkie syndrome) also a <a href="/articles/superior-mesenteric-artery-compression-disorders">superior mesenteric artery compression disorder</a>, where the SMA compresses the third part of the duodenum (the two conditions, however, may be associated).</p><h4>Epidemiology</h4><p>There is a slightly greater female predilection.</p><h4>Clinical presentation</h4><p>The most common clinical manifestations of nutcracker syndrome are left flank pain, pelvic pain, hematuria and gonadal varices. Orthostatic proteinuria has also been reported. Hematuria can be microscopic or macroscopic. Haematuria should be from the left ureteric orifice only <sup>4</sup>. In the absence of clinical symptoms, renal vein compression is referred to as <strong>Nutcracker phenomenon.</strong></p><h4>Pathology</h4><h5>Associations</h5><ul>
-<li>reduced aortic-SMA angle (the normal angle between aorta and SMA is approximately 45° (38-65°))</li>- +<li>reduced aortic-SMA angle: normal angle between aorta and SMA is ~45° (range 38-65°)</li>
-<li>collateral pathways: main collateral pathway is the left gonadal vein which will display early enhancement during portal venous phase.</li>-<li>pressure gradient >3 mm Hg on renal venography in early stage nutcracker syndrome. Well developed collateral veins dissipate the high pressure gradient.</li>-<li>compression ratio (<strong>CR</strong>) given by the equation <strong>CR=P-C/C </strong>i.e. diameter of pre-compressed vein (<strong>P</strong>) minus diameter of compressed vein (<strong>C</strong>) divided by the diameter of the compressed vein (<strong>C</strong>). A compression ratio<strong> </strong>above 2.25 is highly sensitive and specific for Nutcracker syndrome <sup>10</sup>.</li>- +<li>collateral pathways: main collateral pathway is the left gonadal vein which will display early enhancement during portal venous phase</li>
- +<li>pressure gradient >3 mm Hg on renal venography in early stage nutcracker syndrome. Well developed collateral veins dissipate the high-pressure gradient</li>
- +<li>compression ratio (<strong>CR</strong>) given by the equation <strong>CR=P-C/C </strong><ul>
- +<li>
- +<strong></strong>i.e. diameter of pre-compressed vein (<strong>P</strong>) minus diameter of compressed vein (<strong>C</strong>) divided by the diameter of the compressed vein (<strong>C</strong>).</li>
- +<li>a compression ratio<strong> </strong>above 2.25 is highly sensitive and specific for Nutcracker syndrome <sup>10</sup>
- +</li>
- +</ul>
- +</li>
References changed:
- 1. Kurklinsky A & Rooke T. Nutcracker Phenomenon and Nutcracker Syndrome. Mayo Clin Proc. 2010;85(6):552-9. <a href="https://doi.org/10.4065/mcp.2009.0586">doi:10.4065/mcp.2009.0586</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/20511485">Pubmed</a>
- 11. Little A & Lavoipierre A. Unusual Clinical Manifestations of the Nutcracker Syndrome. Australas Radiol. 2002;46(2):197-200. <a href="https://doi.org/10.1046/j.1440-1673.2001.01037.x">doi:10.1046/j.1440-1673.2001.01037.x</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/12060163">Pubmed</a>
- 1. Kurklinsky AK, Rooke TW (June 2010). "Nutcracker phenomenon and nutcracker syndrome". Mayo Clinic Proceedings. 85 (6): 552–9. doi:10.4065/mcp.2009.0586. PMC 2878259. PMID 20511485.
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