European Journal of Vascular & Endovascular Surgery
Volume 31, Issue 1 , Pages 80-82, January 2006

Transposition of the Left Renal Vein in Nutcracker Syndrome

  • J.Y. Kim

      Affiliations

    • Division of Vascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
    • ,
  • J.H. Joh

      Affiliations

    • Division of Vascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
    • ,
  • H.Y. Choi

      Affiliations

    • Department of Urology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
    • ,
  • Y.S. Do

      Affiliations

    • Department of Radiol, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Division of Vascular Surgery, Samsung Medical Center, 50 Irwon-dong, Seoul, South Korea
    • ,
  • S.W. Shin

      Affiliations

    • Department of Radiol, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Division of Vascular Surgery, Samsung Medical Center, 50 Irwon-dong, Seoul, South Korea
    • ,
  • D.I. Kim

      Affiliations

    • Division of Vascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
    • Corresponding Author InformationCorresponding author. Dr Dong-Ik Kim, Division of Vascular Surgery, Samsung Medical Center, 50 Irwon-dong, Seoul, South Korea.

Accepted 3 August 2005. published online 05 October 2005.

Article Outline

Abstract 

Mesoaortic compression of left renal vein produces left renal vein hypertension resulting in left flank pain, hematuria and pelvic-ureteral varices. This is called the nutcracker syndrome. The nutcracker syndrome has been treated in various ways. We recently experienced two cases of patients with nutcracker syndrome. We treated the patients with transposition of their left renal vein.

Keywords: Nutcracker syndrome, Hematuria, Renal vein, Mesenteric artery

 

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1. Introduction 

Meso-aortic compression of the left renal vein (LRV) produces LRV hypertension, and this causes left flank pain, haematuria and pelvic-ureteral varices. This condition is called the nutcracker syndrome or pelvic congestion syndrome. Nutcracker syndrome should be considered in the differential diagnosis of haematuria.

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2. Report 

2.1. Case 1 

A previously healthy 22-year-old female was referred to our hospital with idiopathic gross haematuria, with blood clots, that had occurred intermittently for about 5 months. She had no pain in her abdomen and flank, no history of major health problems and there was no relevant family history. Physical examination did not reveal flank tenderness. No varices of the vulva were present. All blood tests, including complete blood cell count, blood chemistry and blood coagulation profile were within the normal limits. Urine analysis revealed numerous red blood cells, without dysmorphic change. We performed diagnostic studies to examine this seemingly idiopathic haematuria. Cystoscopy disclosed bloody urine draining through the opening of the left ureter. CT angiography of the abdomen showed that the LRV was compressed between the aorta and the superior mesenteric artery (SMA) (Fig. 1). Conventional angiography of the SMA and venography of the LRV and the inferior vena cava (IVC) was performed simultaneously, and showed that the LRV had an abrupt narrowing at the aorto-mesenteric angle, with stagnation of the contrast material. Also blood refluxed into the adrenal and ovarian veins, independent of respiration. The pressure gradient between the LRV and the IVC was 5cm H2O. Therefore, nutcracker syndrome was diagnosed.

  • View full-size image.
  • Fig. 1. 

    (Left) Preoperative abdominal CT scan showed the compression of the left renal vein (black arrow) between the superior mesenteric artery (white arrow) and the abdominal aorta. (Right) Left renal venography demonstrated periureteral venous collaterals (white arrow) with reflux into the gonadal and adrenal veins. The pressure gradient between the inferior vena cava and the left renal vein (black arrow) was 5cm H2O.

In the operating theatre, a trans-abdominal incision was made and the retroperitoneal space was opened through the division of Treitz's ligament in the fourth portion of the duodenum. The LRV was mobilized from the surrounding tissues. The dilated peri-renal vein, peri-ureteral vein, ovarian vein and adrenal vein on the left side were ligated and divided from the LRV. The LRV was excised from the entry site of the IVC and the IVC was closed using continuous 4–0 prolene suture. Then, the LRV was anastomosed with the IVC 3.5cm below the original anatomic site. The patient was discharged without complication and has remained in good health. The haematuria disappeared 4 months postoperatively. The duration of follow up was 42 months.

2.2. Case 2 

The patient was a 24-year-old male who was without previous illness and he complained of gross, painless haematuria, which had occurred intermittently for 1 year and was aggravated by exercise. He had no varicoceles and no tenderness in the abdomen or flank. He had no medical history of note. His family had no major health problems. Blood tests and urine analysis were normal. We started diagnostic studies for the idiopathic haematuria. The results of cystoscopy and CT angiography of the abdomen were the same as in Section 2.1. We performed arteriography of the SMA and venography of the LRV simultaneously and found that the LRV was indented by meso-aortic compression. The pressure gradient between the LRV and the IVC was 16cm H2O. Again, nutcracker syndrome was diagnosed.

The patient underwent transposition of the LRV 3.5cm below the original anatomic site via transabdominal exposure of the retroperitoneum (Fig. 2). The peritoneum lateral to the duodenum was incised and Kocher's manoeuvre was performed. The duodenum was reflected medial, and the LRV and IVC were mobilized. The patient recovered and was discharged without complication and has remained in good health since then. The haematuria disappeared in 1 month. The duration of the follow up was 41 months.

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3. Discussion 

The clinical syndrome caused by compression of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the abdominal aorta has been termed nutcracker syndrome and was first described by the anatomist Grant.1

The clinical manifestations of nutcracker syndrome are gonadal varices (varicocele or ovarian vein syndrome), flank pain and/or hematuria. The association between nutcracker syndrome and haematuria was explained by the careful pathologic studies of Macmahon and Latorraca.2 They documented communications between the dilated venous sinuses and the adjacent renal calyces as an explanation for the haematuria.

The diagnosis of nutcracker syndrome cannot be established using routine diagnostic methods. The compression of the LRV between the SMA and the abdominal aorta can be confirmed by ultrasonography, selective renal venography or CT. Measurement of the pressure gradient between the inferior vena cava (IVC) and the LRV is important; however, there is no specific cut-off value for the diagnosis of nutcracker syndrome. Haematuria from the left ureter orifice, confirmed by cystoscopy, in the absence of any other detectable pathology is one of the indicators of nutcracker syndrome. Zerhouni et al.3 have made an important contribution to understanding and diagnosing nutcracker syndrome. They observed elevated LRV pressures in three patients who were suffering from left sided varicoceles and pelvic pain, in the presence of normal right sides. Since that report, measurement of the pressure gradient between the LRV and the IVC at venography and detection of the reflux from the LRV to the collateral braches has been an important diagnostic method.

The treatment of nutcracker syndrome is controversial. Conservative therapy using haemostatic agents has been proposed for cases with mild hematuria.4 Surgical therapy such as medial nephropexy,5 renal vein bypass,6, 7 transposition of the LRV8 and auto-transplantation of the left kidney9 have been reported. An endovascular approach with stent implantation has been reported recently.10

We report two cases of transposition of the LRV below its original anatomic site. The mobilization of the IVC through Kocher's manoeuvre gave us a better anatomical view than did the approach through the division of the Treitz's ligament in the fourth portion of the duodenum. The mobilization of the LRV from the peri-renal vein, the peri-ureteral vein, the gonadal vein and the adrenal vein of the left side is important to for transposition of the LRV.

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References 

  1. Grant HCB. Method of anatomy. Baltimore: Williams & Wilkins; 1937;p. 158
  2. Macmahon HE, Latorraca R. Essential renal hematuria. J Urol. 1954;71:667–676
  3. Zerhouni EA, Siegelman SS, Walsh PC, White RI. Elevated pressure in the left renal vein in patients with varicocele: preliminary observations. J Urol. 1980;123:512–513
  4. Dever DP, Ginsburg ME, Millet DJ, Feinstein MJ, Cockett ATK. Nutcracker phenomenon. Urology. 1986;27:540–542
  5. Wendel RG, Crawford ED, Hehman KN. The nutcracker phenomenon; an unusual cause for renal varicosities with hematuria. J Urol. 1980;123:761–763
  6. Coolsaet BLRA. Ureteric pathology in relation to right and left gonadal veins. Urology. 1978;12:40–49
  7. Shaper KR, Jackson JE, Williams G. The nutcracker syndrome: an uncommon cause of hematuria. Br J Urol. 1994;74:144–146
  8. Stewart BH, Reiman G. Left renal venous hypertension ‘nutcracker’ syndrome. Managed by direct renocaval reimplantation. Urology. 1982;20:365–369
  9. Chuang CK, Chu SH, Lai PC. The nutcracker syndrome managed by autotransplantation. J Urol. 1997;157:1833–1834
  10. Chiesa R, Anzuini A, Marone EM, Briguori C, Moura MRL, Melissano G, et al. Endovascular stenting for the nutcracker phenomenon. J Endovasc Ther. 2001;8:652–655

PII: S1078-5884(05)00517-4

doi:10.1016/j.ejvs.2005.08.012

European Journal of Vascular & Endovascular Surgery
Volume 31, Issue 1 , Pages 80-82, January 2006

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