|Year : 2020 | Volume
| Issue : 3 | Page : 59-63
Nutcracker syndrome at a Glance
Dimitrios C Karathanasis1, Androula C Karaolia2, Christos-Rafail D Karathanasis3
1 Department of Nephrology, 404 General Military Hospital, Larissa, Greece
2 Department of Neurosurgery, University of Thessaly, Larissa, Greece
3 School of Medicine, European University Cyprus, Nicosia, Cyprus
|Date of Submission||03-Dec-2020|
|Date of Acceptance||30-Dec-2020|
|Date of Web Publication||13-Apr-2021|
Dr. Dimitrios C Karathanasis
Department of Nephrology, 404 General Military Hospital, Larissa
Source of Support: None, Conflict of Interest: None
The term of nutcracker phenomenon describes the entrapment of the left renal vein (LRV) between the superior mesenteric artery and the aorta without accompanying symptoms. The clinical term of nutcracker syndrome corresponds to the additional presence of symptoms. It is a rare anatomical and functional disorder that appears mainly with hematuria and left kidney pain and may be accompanied by albuminuria and pelvic congestion syndrome with varicose veins. It is diagnosed primarily by Doppler ultrasound where the main parameters are the anteroposterior diameter and the flow velocity in the dilated LRV and secondarily by computed tomography where the characteristic finding is the “beak sign” at the site of the stenosis. Therapeutically, surveillance or conservative management is initially chosen due to the high probability of spontaneous remission, while in a persistent and intense clinical picture, the main treatment options are the placement of an endovascular stent and the transposition of the LRV.
Keywords: Hematuria, left renal vein entrapment, nutcracker
|How to cite this article:|
Karathanasis DC, Karaolia AC, Karathanasis CRD. Nutcracker syndrome at a Glance. J Renal Nutr Metab 2020;6:59-63
|How to cite this URL:|
Karathanasis DC, Karaolia AC, Karathanasis CRD. Nutcracker syndrome at a Glance. J Renal Nutr Metab [serial online] 2020 [cited 2021 Jun 13];6:59-63. Available from: http://www.jrnm.in/text.asp?2020/6/3/59/313632
| Introduction|| |
The term of nutcracker syndrome (NS) was proposed by de Schepper in 1972 to describe trapping of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta. It was first described in 1950 by El-Sadr and Mina, while in 1971, Chait et al. were the first who likened the aorta and upper mesenteric artery to the arms of a nutcracker. Later, NS was associated with orthostatic albuminuria in children., While the nutcracker phenomenon (NP) is just an anatomical term that describes the entrapment of the LRV between the SMA and the aorta, NS is the corresponding clinical term which denotes the presence of symptoms.
There are three types of NS depending on the location of the entrapment:
Anterior nutcracker syndrome
It is the classic and most common entrapment of the LRV between the SMA and the aorta.
- Normally, trapping of the LRV is avoided because the SMA protrudes from the aorta at an angle of 90° and after following an anterior course for 4–5 mm then goes downhill. In NS, the protrusion angle of the SMA is acute,
- Posterior renal ptosis with subsequent pressure of the LRV over the aorta
- A high course of the LRV
- Low or lateral origin of the SMA
- Fibrous tissue at the origin of the SMA
- Pancreatic neoplasms, para-aortic lymphadenopathy, and significant lordosis of the lumbar spine
- NP on the right side has been observed in a gravid uterus.
Posterior nutcracker syndrome
Entrapment of the LRV in a retroaortic position between the aorta and the vertebral column.
Combination of anterior and posterior nutcracker syndrome
The LRV divides into an anterior branch trapped between the SMA and the aorta and a posterior arm trapped between the aorta and the vertebrae (renal collar).
| Incidence|| |
The exact incidence of the syndrome has not been determined. Asymptomatic dilatation of the LRV has been found at a rate of 72%., A study of 7 million school-age children in Korea found 65 cases (9.3/million). There has been an increase in the incidence in adolescence and in the third to fourth or second to third decade of life. It has been observed that a low body mass index predisposes to the onset of the syndrome. There seems to be no consensus on gender as some studies have found a slightly increased incidence in women compared to men,, while newer research does not find a difference in gender.
| Clinical Presentation|| |
It should be clear that the term NP corresponds to asymptomatic patients while symptomatic ones are considered to have NS. The absence of symptoms is common, especially in children. Although the main symptoms are hematuria and left flank pain, a significant number of other findings can be identified.
It manifests as microhematuria which is four times more frequent than macrohematuria. Increased pressure in the LRV and consequently in the entire venous system of the left kidney leads to rupture of the thin wall of the small veins at points of close contact with the urine collecting system. Cystoscopy shows that the hematuria comes from the left ureteric orifice. Rarely hematuria can lead to anemia.
Left flank pain
Seldom, it is accompanied by abdominal pain and has been attributed to the formation and removal of blood clots.
It was observed and early correlated with NS in children. It is usually moderate.,, The frequency of symptoms of hematuria, albuminuria, and left kidney pain is proportional to the degree of narrowing of the LRV.
It has been linked to chronic fatigue in children.
The increase in pressure in the LRV and its tributaries leads to the development of varicose veins and collateral veins. The main veins that are distended are the left gonadal vein, the left ureteral vein, the capsular veins, the left lumbar ascending vein which ends up in the LRV with an ascending course, and the suprarenal vein which has a descending course. Left ovarian vein dilatation can lead to vulvar varices, left limb varicosities, and pelvic congestion syndrome, manifested by dysmenorrhea.,, Left testicular vein dilatation may result in left-sided varicocele and left limb varicose veins.,,
Thrombosis of the left renal vein
It has been described as a rare complication.
| Diagnosis|| |
The diagnosis is based on imaging findings. It is notable that in the upright position, the weight of the intestine makes the angle of the SMA with the aorta more acute. Imaging tests performed in a supine or decubitus position may not show the entrapment.
Renal Doppler ultrasound
It has a sensitivity of 69%–90% and a specificity of 89%–100% and is considered a first-choice test., Typical findings are first the dilatation of the peripheral part of the LRV and second the increased pressure in the LRV concerning the pressure in the inferior vena cava. For the evaluation of these findings, the anteroposterior diameter of the LRV (DLRV) and the flow velocity in the LRV (VLRV) are measured. While normally the gradient of pressure between the peripheral part of LRV and the inferior vena cava is up to 1 mmHg, in NS is >3 mmHg.
For adults, it has been suggested that if the DLRV ratio (DLRV at the level of the renal hilum to DLRV at the level of stenosis) is >5, then the diagnosis of NS is safe with a sensitivity of 69% and a specificity of 89%. Furthermore, if the VLRV ratio (VLRV at the site of stenosis to VLRV at the level of the renal hilum) is >5, then the diagnosis of NS has a sensitivity of 80% and a specificity of 94%. Furthermore, the combination of the above two criteria sets the diagnosis with 90% sensitivity and 100% specificity.,,,
For children, it has been suggested that the diagnosis of NS is set if the DLRV ratio is >4.2 and if the VLRV ratio is >4. Recent research has disclosed that if the DLRV ratio is >2.25, then the diagnosis of NS has 91% sensitivity and specificity.
The most typical finding at the point of stenosis is the “beak sign” formed by the protrusion of SMA with an acute angle from the aorta. According to Kim et al., if the DLRV ratio is >4.9, then the diagnosis of NS has a sensitivity of 66.7% and a specificity of 100%. The main disadvantage of computed tomography (CT) is radiation.
Magnetic resonance imaging
It provides a detailed display of the point of entrapment without the disadvantage of radiation.
Three-dimensional computed tomography
It provides the advantage of a detailed display without being un invasive method.
Digital subtraction angiography
It provides the most complete illustration, but it is an invasive method that is applied not exclusively for diagnostic purposes but mainly for the application of therapeutic options.
| Management|| |
In general, treatment is divided into conservative, stenting, and open surgery. As a rule, children are treated conservatively as spontaneous remission has been observed over time.
Conservative treatment is reserved for patients with mild symptoms and mild hematuria and is recommended for up to 2 years., Depending on the case, it includes:
- Treatment of orthostatic albuminuria with angiotensin inhibitors,
- Treatment of pelvic congestion by applying elastic compression stockings.
Endovascular methods are a top priority as they have fewer complications than open methods. The initial attempt of balloon angioplasty was quickly sidelined by the implementation of stents.
The placement of an endovascular expandable stent to treat the NS was first described by Neste et al. in 1996. The growing experience confirmed the safety and efficacy of the option. There was a reduction of the blood pressure in the LRV and an alleviation of symptoms within 6 months. Possible complications include stent migration, thrombosis, and restenosis. Stent migration appears a frequency of 7.3%, and it occurs due to displacement, early mobilization of the patient, constant pressure from the pulsating aorta, or finally due to large dilatation of the distal part of the LRV causing insufficient adherence of the stent to the venous wall.,
| Surgery|| |
Surgical treatment is reserved only for patients with severe clinical manifestations such as persistent pain or severe macroscopic hematuria.
The surgical options are:
- Removal of the fibrous tissue that surrounds and strangles the LRV at the point of stenosis
- Transposition of the LRV (renocaval venous reimplantation). It is considered as the surgical method of choice. Beyond the open surgery,, it has been successfully performed laparoscopically., Recently, robotic-assisted laparoscopic transposition of the LRV has been proposed initially by Wang et al. with metal clamps and later by Chau et al. with vessel loops. The most common complications of LRV transposition, especially in open surgery, include hemorrhage, retroperitoneal hematoma, and LRV thrombosis. The combination of LRV reimplantation with stent placement has also been tested
- Bypass of the LRV. A portion of the saphenous vein is used to create a bypass between the LRV and the inferior vena cava at a level below the LRV
- Transposition of the SMA below the LRV. It appears an increased risk of bleeding and thrombosis resulting in intestinal ischemia
- Left kidney nephropexy or even auto-transplantation
- Installation of external stents. It was originally proposed by Barnes et al. in 1988. Lately, three-dimensional printing extravascular titanium stents have been tested successfully
- Decongestion of the LRV. It has three options: (a) shunting between the LRV and the splenic vein,, (b) shunting between the left gonadal vein and the inferior mesenteric vein, and (c) transposition of the left gonadal vein to the inferior vena cava.
| Posterior Nutcracker Syndrome|| |
It is a developmental anomaly of the embryonic circumaortic venous ring. It appears an incidence of 1%–2.4%.,Unlike anterior NS, it concerns mainly adults and especially men. The main clinical manifestation is hematuria, which in contrast to anterior NS presents either as microhematuria or macrohematuria by the same frequency. According to management, transposition of the LRV is recommended as the method of choice.
| Conclusions|| |
NS should not be confused with NP. Underdiagnosis due to its rarity could be avoided if clinicians bear in mind NS in cases of hematuria. Diagnosis can be easily made by either renal Doppler ultrasound or CT. The main CT elements of the extended LRV are the anteroposterior diameter and the flow rate. Although conservative treatment is the usual approach, in cases with severe clinical manifestations, endovascular stent or even transposition of the LRV is performed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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