Her diagnosis was delayed but was eventually made on CT angiography after several months and after numerous consultations with several different specialties. 7. Pournasiri Z. In this article, we re-fer to this heterogeneous group of disorders as “vascular compression syndromes,” since they all involve either the compression of vascular of nutcracker syndrome is difficult and requires a high index of suspicion. “Dilatation” of the left renal vein on computed tomography in children: a normal variant. Nutcracker syndrome (NCS) is a rare entity defined by the symptomatic hypertension of the left renal vein (LRV) compressed in the majority of cases between the superior mesenteric artery and the aorta. NCS is a term that should be used only when symptoms are present in addition to the NCP (10, 18). 1). Author information: (1)Department of Radiology, Zhejiang Provincial People's Hospital, Hangzhou, Zhejiang, P. R. China. Most cited articles. Symptoms can be due to venous hypertension within the left kidney: left-flank pain that can radiate to the buttock and hematuria (from microscopic to gross hematuria). Dig Surg 2007;24:149–156. To obtain an optimal Doppler angle at the AM portion of the LRV, we should adjust the position and the direction of the transducer by slightly moving it to the left and rotating it counterclockwise, until the AM portion of the LRV is located in the left corner of the US image (Fig. Superior mesenteric artery syndrome accompanying with nutcracker syndrome: a case report. persistent or recurrent hematuria causing anemia, and blood clots J Ultrasound Med 2004;23:595–601. Patients may present with hematuria, proteinuria, collateral vessel formation and pain. Therefore, we suggested that beak sign can be considered as a practically useful feature in CT diagnosis of NCS (Fig. TRUE Nutcracker Syndrome is when the left renal vein is compressed, regardless of position. syndrome is difficult in patients with borderline left renal vein Fong JKK, Poh ACC, Tan AGS, Taneja R. Imaging findings and clinical features of abdominal vascular compression syndromes. Received March 22, 2019; Accepted July 17, 2019. An axial Nutcracker syndrome (NCS) is a syndrome caused by compression of the left renal vein (LRV), between the abdominal aorta and the superior mesenteric artery, resulting in hypertension of the LRV and hematuria. Axial contrast-enhanced CT of the abdomen demonstrated compression of 3). vessels. For the diagnosis of NCS, Doppler US can be helpful. Nutcracker syndrome can also cause mild to moderate proteinuria. the urinary tract should raise suspicion for Nutcracker syndrome.2 Elevated pressure in the left renal vein in patients with varicocele: preliminary observations. By Peter H Takeyama, MD, Shweta Bhatt, MD, and Vikram S. Dogra, MD, University of Rochester School of Medicine, Rochester NY, Cardiac MRI perfusion and viability imaging: Clinical value in cardiac care. Powerpoint slides. Barnes RW, Fleisher HL 3rd, Redman JF, Smith JW, Harshfield DL, Ferris EJ. 7D). A narrow the aorta as the left renal vein crosses midline (Figure 1). PDF | Nutcracker syndrome (NCS) is a syndrome caused by compression of the left renal vein (LRV), between the abdominal aorta and the superior... | Find, … hypertension.2, Controversy also exists regarding Hohenfellner M, Steinbach F, Schultz-Lampel D, Schantzen W, Walter K, Cramer BM, et al. 59-year-old female with concurrent NCS and pelvic congestion syndrome. Computerized tomography (CT) was used to … Other Barsoum MK, Shepherd RFJ, Welch TJ. Javaid MM, Ong CC, Subramanian S. Blood in urine: a hard nut to crack. The purpose of this article is to review the usefulness of Doppler US, with its technical considerations, and CECT findings of the LRV in patients with NCS. A cystoscopic finding of left-sided hematuria is a supporting evidence for NCS. J Vasc Surg 2009;49:386–393. Diagnostic value of computed tomographic findings of nutcracker syndrome: correlation with renal venography and renocaval pressure gradients. Therefore, reassurance and general supportive care are treatment options in most cases with NCS (12, 28, 46). Jpn J Vasc Surg 2001;10:503–507. 22.4. Sebro K, Goetz L, Persaud S. Nutcracker syndrome: a rare and potentially under diagnosed cause of haematuria. However, several technical issues, such as Doppler angle and sample volume, need to be … This leads to LRV varices, left gonadal vein varices and therefore, the pelvic congestion syndrome. Even though the pressure gradient between the two vessels needs to be determined by invasive catheterization, we may estimate the pressure gradient theoretically from the flow velocity obtained through Doppler US. Am J Med 2017;130:e89–e91. Surgical options include nephrectomy, Although these conditions can occur with or without NCP, the conditions are more severe and prone to recur after treatment if NCP is associated with varicocele or PCS, due to the pressure gradient caused by NCP (39, 40, 41, 42). Radisic MV, Feldman D, Diaz C, Froment RO. alleviate symptoms.6 Indications for surgery include severe Radiology 1996;198:93–97. diagnosed.1, Doppler ultrasound measurements of the Although the diameter ratio was significantly different in three of the four aforementioned studies (7, 8, 11, 32), we should keep in mind that there are situations in which applying a diameter ratio criterion in clinical practice is difficult, especially when diameters are measured using US. vein.2 A hypothesis for the narrowing of the aortomesenteric Varicocele and pelvic congestion syndrome (PCS) occur when there is reflux of blood flow into the gonadal veins due to incompetence of venous valve function in men and women, respectively. In some cases, nutcracker syndrome results in no symptoms, Boodman reports. To study the pathophysiology of the nutcracker syndrome and to describe a new method of treatment. Shokeir AA, El-Diasty TA, Ghoneim MA. CECT images in early cortical phase show retroarotic LRV (arrows). CT and MRI are used to follow up afterwards, and if further conrfirmation is necessary, venography is used to confirm. Conflicts of Interest:The author has no potential conflicts of interest to disclose. Reed NR, Kalra M, Bower TC, Vrtiska TJ, Ricotta JJ 2nd, Gloviczki P. Left renal vein transposition for nutcracker syndrome. Nutcracker syndrome in children with gross haematuria: Doppler sonographic evaluation of the left renal vein. The normally wide aortomesenteric angle is maintained by US National Guidelines Clearinghouse. Semin Vasc Surg. For this reason, coexistence of NCS and pelvic congestion syndrome … Nutcracker syndrome associated with severe anemia and mild proteinuria. 2) (23). American Roentgen Ray Society Images of Nutcracker syndrome CT All Images X-rays Echo & Ultrasound CT Images MRI; Ongoing Trials at Clinical Trials.gov. Nephrol Dial Transplant 2005;20:2009. color Doppler sonography: Correlation with flow patterns on retrograde aortomesenteric angle causes compression or entrapment of the left renal In the normal population, the pressure gradient between the LRV and IVC is less than 1 mm Hg. There have been two reports regarding NCP (19, 20), which showed that asymptomatic compression of the AM portion of the LRV is a very common phenomenon. The nutcracker syndrome as a rare cause of chronic abdominal pain: a case report. system or between dilated venous sinuses and adjacent renal calyces.2 Vasc Endovascular Surg 2011;45:749–755. NCS refers to a syndrome caused by significant compression of LRV between the abdominal aorta and SMA, resulting in hypertension of the LRV and hematuria due to rupture of the thin-walled veins close to the calyceal fornices (10, 11). a Axial contrast-enhanced CT image on the portal venous phase shows … NCS typically causes intermittent gross hematuria but may be accompanied by microscopic hematuria, proteinuria, or left flank pain, without gross hematuria (12, 13, 14, 15, 16, 17). J Ultrasound Med 2002;21:39–45. Therefore, if the PV of the LRV at the AM portion is approximately 5 times higher than that at the hilar portion in both adults and children, we may suspect NCS. Nutcracker syndrome (renal vein entrapment). NCS may be suspected noninvasively on the basis of a causative anatomical change, i.e., compression of the LRV between the aorta and SMA. Mesoaortic compression of the left renal vein (the so-called nutcracker syndrome): repair by a new stenting procedure. Xu D, Liu Y, Gao Y, Zhang L, Wang J, Che J, et al. NCS should be considered as a causative condition in patients with unexplained hematuria, either gross or microscopic. hypertension. The nutcracker syndrome: new methods of diagnosis and treatment. renal vein and the IVC is the gold standard for demonstrating renal vein treatment of nutcracker syndrome. Nutcracker syndrome complicated by left renal vein thrombosis and resolution with anticoagulation: A case report and review of the literature ... LRV with no clinical findings suggestive of post-thrombotic syndrome. Unexplained hematuria during pregnancy: right-sided nutcracker phenomenon. The incidence of NCS is unknown. The transabdominal ultrasound with the Holdstock-White Protocol (tipping the patient backward as in the photo above) often shows an opening of the left renal vein, which is … To date, surgery remains the gold standard in its treatment. The observation of a jetting flow on CDUS may also be helpful in determining the appropriate sample volume during spectral Doppler US. Sugimoto I, Ohta T, Ishibashi H, Takeuchi N, Nagata Y, Honda Y. Eur J Vasc Endovasc Surg 2017;53:886–894. J Urol 1980;123:761–763. However, in a later study that recruiting pediatric subjects, the mean ratio was not significantly different between the two groups: 5.3 ± 1.4 in the NCS group and 4.7 ± 1.9 in the control group (8). Demographics and Clinical Features … Renal nutcracker syndrome (NCS) is a condition that occurs when the left renal vein (the vein that carries blood purified by the left kidney) becomes compressed. CT angiography are other noninvasive modalities that can demonstrate For Doppler US, LRV should be carefully examined to measure the PV of the LRV at the AM portion. J Urol 1982;127:1070–1071. Because Doppler US may be the easiest way to measure blood flow velocity in the body, it can be used for the diagnosis of NCS. that are a normal variant and those that indicate early nutcracker After the abdominal aorta and superior mesenteric artery (SMA) were described as two arms of a nutcracker, the condition was named nutcracker syndrome (NCS) by de Schepper in 1972 (2, 3). Nutcracker syndrome is a variation of nutcracker phenomenon or renal vein entrapment syndrome, in which the arteries near the kidney compress the left renal (kidney) vein. Nutcracker syndrome is diagnosed through imaging such as doppler ultrasound (DUS), computed tomography (CT), magnetic resonance imaging (MRI), and venography. causing abdominal or flank pain. Radiology 1982;145:647–650. 2019 Dec;20(12):1627-1637. Because 1 mm Hg is 1333 g/cm/s2 and the density of blood (ρ) is approximately 1.06 g/cm3, we may calculate a theoretical pressure gradient in mm Hg unit using two PV values at the hilar and AM portions of the LRV. hypertension is renal venography combined with measurement of renocaval anterior-posterior (A-P) diameter and peak velocities of the left renal However, if hematuria is severe and persistent, surgical or other interventional management may be considered (5, 28, 35, 38, 47, 48, 49, 50, 51, 52) although the effectiveness of these surgical or interventional treatments is not yet fully established. In this context, suggesting NCS as a likely cause of unexplained hematuria is important in the management of such patients, because it allows a more relaxed work-up of hematuria. urinalysis is proposed for mild hematuria, since the development of 4). Hematuria, pelvic or back pain and left varicocele are the most commonly symptoms. Kim KW, Cho JY, Kim SH, Yoon JH, Kim DS, Chung JW, et al. compression of the left renal vein in the aortomesenteric angle and In the normal control group, however, it was 18.6 ± 3.7 cm/s at the renal hilar portion and 50.9 ± 27.9 cm/s at the AM portion in adults (7), and 21.2 ± 5.2 cm/s at the renal hilar portion and 72.4 ± 24.4 cm/s at the AM portion in children (8).
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