Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 2  |  Issue : 4  |  Page : 160-162

Nutcracker Syndrome in an 8-year-old Boy: A Rare Encounter


Department of CVTS, Lokmanya Tilak Municipal Medical College and Sion Hospital, Mumbai, Maharashtra, India

Date of Web Publication13-Apr-2016

Correspondence Address:
Chandan Kumar Ray Mohapatra
Department of CVTS, Lokmanya Tilak Municipal Medical College and Sion Hospital, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-0820.180125

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  Abstract 

The nutcracker syndrome is the clinical equivalent of nutcracker phenomenon characterized by compression of the left renal vein (LRV), 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. It is manifested by pain, hematuria, varicocele or lower or pelvic urinary symptoms. We present a case of an 8-year-old boy who presented with painless hematuria for 2 months. Multislice computed tomography angiography revealed obstruction, ectasia, and increased blood flow at the LRV level. After undergoing surgical treatment (LRV transposition), the patient is asymptomatic with complete resolution of his symptom.

Keywords: Hematuria, left renal vein, nutcracker syndrome, venous obstruction


How to cite this article:
Baviskar AA, Raut C, Mohapatra CR, Mishra P. Nutcracker Syndrome in an 8-year-old Boy: A Rare Encounter. Indian J Vasc Endovasc Surg 2015;2:160-2

How to cite this URL:
Baviskar AA, Raut C, Mohapatra CR, Mishra P. Nutcracker Syndrome in an 8-year-old Boy: A Rare Encounter. Indian J Vasc Endovasc Surg [serial online] 2015 [cited 2019 May 24];2:160-2. Available from: http://www.indjvascsurg.org/text.asp?2015/2/4/160/180125


  Introduction Top


Nutcracker phenomenon refers to the compression of the left renal vein (LRV), most commonly between the aorta and the superior mesenteric artery (SMA), 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. [1],[2],[3] The first reference to the condition dates from 1950 in a paper by El Sadr and Mine. Nevertheless, it was de Schepper, who named it the NCS (renal vein entrapment syndrome). [4] Most typical nutcracker morphologic features imply compression of the LRV between the aorta and the SMA, known as an anterior nutcracker. The retroaortic or circumaortic renal vein may be compressed between the aorta and the vertebral body, which is called posterior nutcracker. [5] It presents with hematuria, left lumbar or abdominal pain, and less frequently with varicocele, chronic pelvic pain, or dysuria.

Herein, we present a case report of an NCS (anterior) observed in our institution in an 8-year-old boy.


  Case Report Top


An 8-year-old boy was admitted in the Pediatric Department of Lokmanya Tilak Municipal Medical College, Sion Hospital, with intermittent painless gross hematuria in a period of 2 months. The patient did not have the history of renal trauma, urinary tuberculosis, urinary bilharziasis, renal calculi, or other diseases. On physical examination, the patient was pale, his vital signs were within normal limits. His hemoglobin on presentation was 9 g/dL, the urine analysis confirmed gross hematuria. Casts were absent, and the urine cytology result was negative for malignancy. Abdominal ultrasonography did not found the causes for his hematuria. The abdominal computed tomography angiography (CTA) revealed compression of the LRV between SMA and the abdominal aorta with dilation of the distal part of LRV [Figure 1]. Neither varicocele nor collateral circulation was detected. With the consent of his parents, surgery was performed through midline laparotomy [Figure 2]. The LRV was cut and transposed approximately 2 cm below to the left lateral side of inferior vena cava [Figure 3]. The surgery and the immediate postoperative course were uneventful. In 1 week after the surgery, the hematuria disappeared.
Figure 1: Abdominal computed tomography angiography image showing compression of left renal vein between the abdominal aorta and superior mesenteric artery with dilated distal left renal vein on axial section

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Figure 2: Intraoperative image: The dilated left renal vein

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Figure 3: Intraoperative image: The left renal vein transposed to left lateral side of inferior vena cava

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  Discussion Top


The first clinical report of this phenomenon was by El-Sadr and Mina in 1950. The term nutcracker is usually credited to de Schepper [5] (1972), although it was first used by Chait et al. (1971); the earliest pathologic description belongs to the anatomist Grant (1937). [4] Most typical nutcracker morphologic features imply compression of the LRV between the aorta and the SMA, known as an anterior nutcracker. Less often, the third portion of the duodenum courses in front of the LRV between the aorta and the SMA. Therefore, the anterior nutcracker is analogous to and may co-occur with compression of the duodenum by the SMA, known as the SMA syndrome (Wilkie syndrome). The retroaortic or circumaortic renal vein may be compressed between the aorta and the vertebral body, which is called posterior nutcracker. [5]

Epidemiologically, this entity occurs more frequently in women and is diagnosed within the second and third decade of life, [1],[2],[3] our case, however, was in an 8-year-old male child. It is difficult to define the exact incidence and prevalence of NCS since the majority of patients are asymptomatic and are diagnosed incidentally on imaging ordered for other reasons. The frequency and severity of the syndrome vary from asymptomatic microhematuria to severe pelvic congestion. Although some patients have severe and persistent symptoms, many especially children are asymptomatic. Symptoms are often aggravated by physical activity and commonly include hematuria, pain or gonadal vein syndrome, varicocele, orthostatic proteinuria, and orthostatic intolerance.

Often the diagnosis is reached by exclusion. As with any other condition, assessment begins with the history and exploration. The ultrasonography is a good first radiographic study as it is safe, cost effective, and screens possible renal and bladder abnormalities and identifies the turbulent vessel renal flow. [6],[7],[8] On the other, computed tomography scan is the most appropriate and single imaging modality. With regard to more invasive investigations, retrograde venography and video angiography to determine the renocaval pressure gradient will give a precise diagnosis of the aortomesenteric clamp. [6],[7] In our case, the CTA led to this diagnosis by showing a dilated LRV being compressed between aorta and SMA.

The management of NCS evolved in the last four decades depends upon the clinical presentation and the severity of the LRV hypertension, including close expectant surveillance, endoscopic proceedings such as external or internal stenting and more complex open surgical procedures LRV transposition, and renal autotransplantation of the left kidney. [6],[7],[8] Among conventional open surgery, autotransplantation, transposition of the renal vein, renal vein bypass or renal vein, and fixation and medialization through nephropexy could be considered. [8] In our case, we did transposition of LRV.


  Conclusion Top


Though NCS is a rare entity, insufficient knowledge of the natural history of these conditions results in uncertainty in treatment selection and diagnostic criteria. Many young patients are asymptomatic or have a benign clinical course and may outgrow their symptoms. CTA is the optimum choice of imaging modality to diagnose NCS as well as to find out associated other abnormalities. In asymptomatic patients, especially if they are young, expectant treatment is the best option. Those with serious impairment or severe symptoms may benefit from a surgical or intravascular intervention.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Cope C, Isard HJ. Left renal vein entrapment. A new diagnostic finding in retroperitoneal disease. Radiology 1969;92:867-72.  Back to cited text no. 1
[PUBMED]    
2.
Barnes RW, Fleisher HL 3 rd , Redman JF, Smith JW, Harshfield DL, Ferris EJ. Mesoaortic compression of the left renal vein (the so-called nutcracker syndrome): Repair by a new stenting procedure. J Vasc Surg 1988;8:415-21.  Back to cited text no. 2
    
3.
Rudloff U, Holmes RJ, Prem JT, Faust GR, Moldwin R, Siegel D. Mesoaortic compression of the left renal vein (nutcracker syndrome): Case reports and review of the literature. Ann Vasc Surg 2006;20:120-9.  Back to cited text no. 3
    
4.
Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc 2010;85:552-9.  Back to cited text no. 4
    
5.
De Schepper A. "Nutcracker" phenomenon of the renal vein and venous pathology of the left kidney. J Belge Radiol 1972;55:507-11.  Back to cited text no. 5
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6.
Shin JI, Lee JS, Kim MJ. The prevalence, physical characteristics and diagnosis of nutcracker syndrome. Eur J Vasc Endovasc Surg 2006;32:335-6.  Back to cited text no. 6
[PUBMED]    
7.
Ahmed K, Sampath R, Khan MS. Current trends in the diagnosis and management of renal nutcracker syndrome: A review. Eur J Vasc Endovasc Surg 2006;31:410-6.  Back to cited text no. 7
    
8.
Zhang H, Li M, Jin W, San P, Xu P, Pan S. The left renal entrapment syndrome: Diagnosis and treatment. Ann Vasc Surg. 2007;21:198-203.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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