Nutcracker syndrome - a mini review on current knowledge

Nutcracker syndrome (NCS) seems to be an under-diagnosed condition defined as external compression of the left renal vein and consequent blood outflow impairment. The majority of cases involve the left renal vein entrapment between abdominal aorta and the superior mesenteric artery. The exact epidemiology is unknown due to lack of consensus on diagnostic criteria and often asymptomatic courses. NCS may occur at any age with peak presentation in the second and third decade of life. There is a slight prevalence in females. The most frequent presentations include micro and macroscopic hematuria, orthostatic proteinuria, orthostatic hypotension, flank pain. Patients may develop pelvic congestion syndrome comprising dyspareunia, dysmenorrhea, abdominal pain, pelvic, gluteal, vulvar varicose veins and varicocele in men. Clinical suspicion of NCS based on signs and symptoms, requires imaging confirmation utilising modalities such as doppler ultrasonography, computed tomography and magnetic resonance angiography, intravascular ultrasound and phlebography. Treatment options of NCS range from conservative surveillance to nephrectomy, thus appropriate approach should be based on clinical manifestation and severity of symptoms. Patients presenting with mild to moderate haematuria, and acceptable symptoms should be treated conservatively. In cases of severe symptoms or when conservative management fails, invasive treatment should be considered. Recommended open surgical procedures include left renal vein distal transposition and renal autotransplantation. Endovascular stenting approach seems to be an encouraging solution. Further long term follow-up is required to create objective treatment guidelines.

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Our Clinical Experiences in Patients with Nutcracker Syndrome

Journal of Dr Behcet Uz Children s Hospital ◽

10.5222/buchd.2021.54366 ◽

2021 ◽

Author(s):

Hatice Sonay Yalçın Cömert ◽

Elif Bahat Özdoğan ◽

Sema Tural Bozoğlu ◽

Gül Şalcı ◽

Haluk Sarıhan ◽

...

Keyword(s):

Superior Mesenteric Artery ◽

Renal Vein ◽

Mesenteric Artery ◽

Left Renal Vein ◽

Rare Condition ◽

Nutcracker Syndrome ◽

Clinical Experiences ◽

Orthostatic Proteinuria ◽

The Mean ◽

Age Range

INTRODUCTION: Nutcracker syndrome (NCS) is a rare condition caused by the compression of the left renal vein between the abdominal aorta and superior mesenteric artery. The purpose of the study was to evaluate our management of NCS. METHODS: Patients were retrospectively reviewed and sex, age, main symptoms at application, physical examination, radiological findings, laboratory examinations, and treatment were recorded. RESULTS: 23 patients (16 girls, 7 boys; age range, 5 to 16) diagnosed with NCS. Microhematuria was detected with 14 (60.8%) patients before diagnosis. And four of 9 (39.1%) were determined after diagnosis. Microhematuria was not detected in 5 (21.7%) of the patients. All patients (100%) had orthostatic proteinuria. 17 (73.9%) patients had mild, 3 (13.0%) patients had moderate and 3 (13.0%) had severe proteinuria. The mean diameter of the left renal vein (LRV) at the aortomesenteric (AM) portions and the hilar were 1.69mm±0.70 and 8.01mm±2.27. The mean angle between the superior mesenteric artery and LRV was 25.26º±7.98. DISCUSSION AND CONCLUSION: In the case of especially colic flank pain, hematuria, and proteinuria NCS should keep in mind in the differential diagnosis. Surgical management may supply more enough clinic improvement when the patient has a response to medical therapy.

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A therapeutic option in nutcracker syndrome and ovarian vein insufficiency

Phlebology The Journal of Venous Disease ◽

10.1177/0268355512474253 ◽

2013 ◽

Vol 29 (3) ◽

pp. 144-149 ◽

Cited By ~ 3

Author(s):

V Bekou ◽

C Zollikofer ◽

N Nieuwkamp ◽

A Von Weymarn ◽

S Duewell ◽

...

Keyword(s):

Renal Vein ◽

Varicose Veins ◽

Therapeutic Option ◽

Left Renal Vein ◽

Balloon Dilation ◽

Nutcracker Syndrome ◽

Ovarian Vein ◽

Pelvic Vein ◽

Collateral Vein ◽

Vein Insufficiency

Objectives: The nutcracker syndrome (NS) may lead to insufficient perirenal collaterals as well as incompetence of the left ovarian vein with consecutive ovarian vein insufficiency. Methods: A female patient with NS and severe genital varicosis was treated with dilation of the renal vein and coiling of a left perirenal collateral vein feeding an insufficient left ovarian vein (LOV) with pelvic vein varicosity. Results: In re-evaluation 18 month later with left renal vein (LRV) and LOV phlebography, a widely patent LRV was found. The embolized LRV to LOV collateral was occluded. However, left hypogastric phlebography showed incompetent branches of the left hypogastric vein feeding the genital varicose veins. These were successfully embolized with coils and the genital varicosity decreased on follow-up. Conclusions: In our patient a combined therapeutic approach with balloon dilation of the NS and embolization of the genital varicose veins by left hypogastric vein coil was performed.

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Nutcracker Syndrome in Children: Role of Doppler Ultrasonographic Indices in Detecting the Pattern of Symptoms

Journal of Clinical Medicine ◽

10.3390/jcm7080214 ◽

2018 ◽

Vol 7 (8) ◽

pp. 214 ◽

Cited By ~ 3

Author(s):

Hulya Nalcacioglu ◽

Meltem Ceyhan Bilgici ◽

Demet Tekcan ◽

Gurkan Genc ◽

Yakup Bostanci ◽

...

Keyword(s):

Renal Vein ◽

Pediatric Patients ◽

Left Renal Vein ◽

Flank Pain ◽

Nutcracker Syndrome ◽

Macroscopic Hematuria ◽

Presenting Symptoms ◽

Left Flank ◽

The Mean ◽

Left Flank Pain

The purpose of this study was to evaluate the clinical characteristics of 44 pediatric patients who were diagnosed as having nutcracker syndrome (NCS). We also investigated the left renal vein Doppler ultrasonography (DUS) results, to determine whether or not there was an association between clinical symptoms and DUS findings among these patients. The clinical data from 44 pediatric patients who were diagnosed as having NCS from January 2008 to December 2015 were retrospectively reviewed. We grouped the patients according to the presenting symptoms as symptomatic (loin pain; macroscopic hematuria or both) and non-symptomatic (microscopic hematuria and proteinuria were detected incidentally) and evaluated the left renal vein DUS indices in these two groups separately. Asymptomatic NCS was found in 27 (61.4%) patients; 21 (47.7%) of whom were admitted for the evaluation of proteinuria. The most frequent presenting symptoms were left flank pain (20.5%) and macroscopic hematuria (13.6%); and 2 (4.5%) patients presented with a combination of left flank pain and macroscopic hematuria. The mean ratio of the diameter of the hilar portion of the left renal vein (LRV) to that of the aortomesenteric portion was 4.36 ± 1.55. The mean ratio of the peak velocity (PV) between the two sites of the LRV was 7.32 ± 2.68 (3.1–15.6). The differences in the ratio of the diameters were statistically significant between the two groups and significantly higher in children with asymptomatic NCS (p = 0.025). The PV ratios of the LRV (p = 0.035) were significantly higher in asymptomatic children with NCS than in the symptomatic group. Our study identifies that increased compression ratio of the LRV entrapment is most observed in orthostatic proteinuria and microscopic hematuria.

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V484: Laparoscopic Spleno-Renal Venous Bypass for Left Renal Vein Nutcracker Syndrome

The Journal of Urology ◽

10.1016/s0022-5347(18)32151-7 ◽

2007 ◽

Vol 177 (4S) ◽

pp. 161-162

Author(s):

Benjamin I. Chung ◽

Monish Aron ◽

Nicholas J. Hegarty ◽

Inderbir S. Gill

Keyword(s):

Renal Vein ◽

Left Renal Vein ◽

Nutcracker Syndrome ◽

Venous Bypass

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The desperate plea of women with the nutcracker syndrome

Phlebologie ◽

10.1055/s-0037-1622296 ◽

2010 ◽

Vol 39 (02) ◽

pp. 104-111

Author(s):

J. L. Villavicencio

Keyword(s):

Renal Vein ◽

Imaging Techniques ◽

Left Renal Vein ◽

Diagnostic Tools ◽

Nutcracker Syndrome ◽

Treatment Techniques ◽

Children And Young Adults ◽

Good Number ◽

Pelvic Congestion

Summary Objective: To increase awareness on the severe impact of the nutcracker syndrome in women with undiagnosed disease. Patients and methods: We reviewed the medical literature and analyzed six representative series with 73 patients with nutcracker syndrome. Women with left flank pain, dyspareunia, dysuria, dysmenorrhea, micro- or macrohaematuria and pelvic congestion symptoms, should be carefully investigated for evidence of meso aortic left renal vein compression. A good number of our colleagues do not believe in the existence of the nutcracker syndrome and send these patients in a long pilgrimage in search of someone who can help them to get relief to their pain. New and improved imaging techniques can assist in the diagnosis but retrograde reno-gonadal phlebography and renocaval gradient are the most reliable diagnostic tools. Results: Among an assortment of treatment techniques, renal vein transposition and endovenous stenting were the two most commonly used procedures. There are no long term studies on renal vein stenting in children and young adults. Its use in these cases should be carefully considered. The nutcracker syndrome may present with pelvic congestion symptoms and its diagnosis missed. The patient's age, severity of symptoms and haemo dynamic renal studies should guide the treatment. Conclusion: An increased awareness of the existence of the nutcracker syndrome may prevent many unfortunate undiagnosed women from spending many months and often years of suffering.

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Transposition of the Left Renal Vein in Nutcracker Syndrome

European Journal of Vascular and Endovascular Surgery ◽

10.1016/j.ejvs.2005.08.012 ◽

2006 ◽

Vol 31 (1) ◽

pp. 80-82 ◽

Cited By ~ 23

Author(s):

J.Y. Kim ◽

J.H. Joh ◽

H.Y. Choi ◽

Y.S. Do ◽

S.W. Shin ◽

...

Keyword(s):

Renal Vein ◽

Left Renal Vein ◽

Nutcracker Syndrome

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Pelvic Venous Congestion Secondary to a Circumaortic Renal Collar in an Adolescent Female: Report of a Case

European Journal of Pediatric Surgery Reports ◽

10.1055/s-0041-1730998 ◽

2021 ◽

Vol 09 (01) ◽

pp. e56-e60

Author(s):

Verónica Alonso-Arroyo ◽

Jose Javier Velasco ◽

Sonia Pérez-Bertólez ◽

Maria Elena Molina ◽

Jose Manuel Marugan-de-Miguelsanz ◽

...

Keyword(s):

Renal Vein ◽

Varicose Veins ◽

Left Renal Vein ◽

Recurrent Abdominal Pain ◽

Massive Hemorrhage ◽

Resonance Imaging ◽

X Ray ◽

Abdominal Palpation ◽

Internal Iliac ◽

Pelvic Congestion

AbstractWe report a 13-year-old girl who presented with a recurrent abdominal pain that started after her menarche. The abdominal palpation revealed tenderness over the left ovarian point. The laboratory study, ultrasonography, and abdominal X-ray were normal. The computed tomography and magnetic resonance imaging showed a double left renal vein with a retroaortic component, an increased left parauterine circulation, and ipsilateral ovarian vein engorgement. A diagnostic and therapeutic phlebography allowed a selective catheterization of a group of pelvic varicose veins draining to the left ovarian and to the internal iliac veins. There were no complications during the procedure and the symptoms disappeared 2 days later. Circumaortic left renal vein may cause hematuria, proteinuria, pelvic congestion syndrome, and massive hemorrhage during surgery. A conservative treatment is recommended for patients without gynecourological/renal symptoms or with mild hematuria. The endovascular treatment by gonadal venous embolization is safe and effective.

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