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Saudi Journal of Kidney Diseases and Transplantation
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ORIGINAL ARTICLE Table of Contents   
Year : 2010  |  Volume : 21  |  Issue : 2  |  Page : 237-241
The role of renal autotransplantation in treatment of nutcracker syndrome


1 Department of Urology, Shiraz University of Medical Sciences, Shiraz, Iran
2 Department of Radiology, Shiraz University of Medical Sciences, Shiraz, Iran

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Date of Web Publication9-Mar-2010
 

   Abstract 

To report our experience with renal autotransplantation in treatment of gross hema­turia caused by nutcracker Syndrome (NCS). Between September 2005 and January 2008, four pa­tients of mean age 25.5 years (range: 23-28) with gross hematuria were diagnosed to have NCS. Investigations revealed isolated hematuria on urinalysis, a bloody efflux from left ureteral orifice by urethrocystoscopy, dilatation of left renal vein (LRV) with significant difference in peak sys­tolic velocity in Colour Doppler UltraSonography (CDUS) and dilatation and compression of LRV between aorta and superior mesenteric artery in MRA. After operation, hematuria dis­appeared in all patients. No vascular or urological complication was seen. Follow up ranged from 4 to 24 months. In conclusion, autotransplatation of left kidney is very effective for the treatment of symptomatic NCS.

How to cite this article:
Salehipour M, Rasekhi A, Shirazi M, Haghpanah A, Jahanbini S, Eslahi SA. The role of renal autotransplantation in treatment of nutcracker syndrome. Saudi J Kidney Dis Transpl 2010;21:237-41

How to cite this URL:
Salehipour M, Rasekhi A, Shirazi M, Haghpanah A, Jahanbini S, Eslahi SA. The role of renal autotransplantation in treatment of nutcracker syndrome. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2020 Oct 26];21:237-41. Available from: https://www.sjkdt.org/text.asp?2010/21/2/237/60059

   Introduction Top


The nutcracker syndrome (NCS) refers to the compression of the left renal vein (LRV) bet­ween the aorta and the superior mesenteric ar­tery (SMA) that results in LRV compression and development of collateral veins. Abnormal congested venous plexuses around the renal pelvis and calyceal fornices cause microhema­turia or painless gross hematuria. [1],[2],[3] Other po­ssible symptoms include left flank pain, [4] left­ sided varicocele [5],[6] pelvic congestion syndrome, [7] chronic fatigue syndrome in children, [8] or ortho­static proteinuria, [9] gastrointestinal symptoms [10] and arterial hypertension. [11]

The aim of the present work is to evaluate efficacy of renal autotransplantation in treat­ment of symptomatic NCS.


   Patients and Methods Top


Between September 2005 and January 2008 four patients, (three men and one woman) of mean age 25.5 years (range=23-28) were diag­nosed to have NCS. All patients complained of intermittent gross hematuria.

After history taking and physical examination (PE), urinalysis (U/A), urine culture (U/C), re­nal function tests(RFT) complete blood count (CBC), urine cytology, tests for coagulation dis­orders, TB and schistosomiasis were performed. Ultrasound of the urinary tract, IVP, urethro­cystoscopy, ureteropyeloscopy, colour Doppler ultra sonography of renal vessels (CDUS), scout spiral CT scan of kidneys and pelvic cavity and magnetic resonance angiography (MRA) were performed as diagnostic investigations.

In CDUS, antero-posterior (AP) diameter of LRV and peak systolic velocity (PSV) at hilum and aorto-mesenteric portion of LRV were mea­sured. Renal biopsy was done for all patients.

After documentation of NCS, all patients un­derwent renal autotransplantation. Through a midline abdominal incision, initially, right ex­ternal and internal iliac vein and artery were prepared for anastomosis. Then left nephrec­tomy was done as the same as donor neph­rectomy in allograft renal transplantation, and left renal artery and vein were anastomosed to internal iliac and external iliac veins respec­tively. The ureter was reimplanted into urinary bladder with modified lich-Gregoir technique. After placement of a hemovac drainage in right iliac fossa abdominal incision was closed as routinely. DTPA diuretic renal scan was done on the first day after surgery and one month later.


   Results Top


Two patients had a past history of surgery (Transposition of LRV) for their gross hema­turia. physical examination was unremarkable. U/A revealed many RBCs without proteinuria or pyuria in all patients. RFT and coagulation profiles were normal. Hemoglobin was above 12g per dL in 3 patients and below 10 g per dL in one patient. U/C, urine cytology and tests for TB and schistosomiasis were negative.

Ultrasonography and IVP demonstrated no abnormality as cause of hematuria. Urethro­cystoscopy revealed a bloody efflux from left ureteral orifice in all patients. Left ureteropy­loscopy was normal and selective urine cytology from left renal pelvis was negative. Scout spi­ral CT scan of kidneys and pelvic cavity were normal too. Renal biopsy in all patients sho­wed no abnormality by light or immunofluo­rescent microscopy. CDUS revealed LRV dila­tion (the mean ± SD AP drimeter of LRV was 12.5 ± 2.1 mm) with significant difference in PSV of LRV at hilum and aortomesenteric por­tion. The mean ± SD of PSV at hilum was 16.75 ± 4.1 cm/sec and in aortomesenteric por­tion was 140.75 ± 31.1 cm/sec. MRA demons­trated dilatation and compression of LRV bet­ween aorta and SMA.

After diagnosis of NCS, left renal autotrans­plantation was done for all patients. Post ope­rative hospital course was uneventful and he­maturia disappeared in all patients. No vascu­lar or urological complication was seen. Renal scan showed good perfusion and function of both kidneys without ureteral obstruction in all patients on one day and one month post ope­ration. No patients have had further hematuria during the follow up period of 4-24 months.


   Discussion Top


The term nutcracker syndrome dates back to the anatomical description by Grant who stated that "the left renal vein as it lies between the aorta and the superior mesenteric artery resem­bles a nut between the jaws of a nutcracker". [12] In the NCS, LRV is compressed between the aorta and the SMA. This phenomenon results in left renal venous hypertension.

The underlying pathophysiology of the NCS is not fully understood. Some experts reported that decreased amounts of perirenal fat may be a factor. [4],[8] Wendel et al, hypothesized that pos­terior renal ptosis with stretching of the LRV over the aorta may contribute to NCS. [13]

Recently, Hohenfellner et al demonstrated that abnormal branching of the SMA from the aorta was the underlying cause of the NCS. [14] Sagit­tal images on MRA reveal that SMA originates from the aorta in almost a rectangular configu­ration so that SMA has a 4-5 mm course in the ventral direction before beginning a caudal des­cent thus resulting in an inverted J configuration. This configuration normally prevents compre­ssion of the LRV by the SMA. In NCS cases the SMA has been noted to branch from the aorta at an acute angle with initial sleep cau­dal descent causing compression of the LRV. ,[14],[15]

Intermittent hematuria (macroscopic or mic­roscopic) is the most common presenting symp­tom of NCS. De Schepper was the first to show the relationship between NCS and gross hematuria in a 16-year-old boy. [16] It has been postulated that rupture of thin walled septum between hypertensive small veins and collec­ting system in the calyceal fornices or com­munication between dilated venous sinuses and adjacent renal calices caused hematuria in NCS. [17]

In a study Nishimura et al showed that 88% of the patients with left renal bleeding in the absence of any other detectable pathology had LRV hyper tension. [18]

Another clinical manifestation of NCS is left­sided varicocele. Salehipour et al demonstrated that 33% patients with primary high grade vari­cocele and 50% patients with recurrent high grade varicocele had NCS. [6]

The NCS is difficult to diagnose. There are no specific clinical presentations, and clinical and laboratory evaluation is non diagnostic as well as the radiologic investigations are not helpful.

Ali-El-Dein et al proposed a step wise diag­nostic evaluation for the NCS. These include phase contrast microscopy, abdominal ultraso­nography and IVP, coagulation profiles. Tests for urinary TB and bilharziasis, cystoscopic lo­calization of the hematuria, urine cytology, left flexible ureterorenoscopy, renal biopsy, CT or MR angiography, LRV and inferior vena cava (IVC) phlebography and venous pressure ma­nometry. [19]

LRV and IVC phlebogrophy with measure­ment of the pressure gradient between the IVC and LRV has been used to confirm the NCS.

However, such examinations are very inva­sive, time-consuming and have serious compli­cations and therefore not indicated for all pa­tients. On the other hand, the pressure gradient depends on several other factors such as inten­sity of development of the retroperitoneal col­lateral venous circulation. In addition a clear cut-off between normal and pathological pres­sure gradient values does not exist yet. [20] Ne­vertheless pressure gradient of 3 mm or more is significant.

Color Doppler ultrasonography (CDUS) may be employed as the diagnostic method of choice in the screening of the NCS. Kim et al first demonstrated that measurement of AP diame­ter and PSV in the hilar portion of the LRV and in aortomesenteric portion could be used for diagnosis of the NCS.

They reported that a ratio of AP diameter and PSV more than 5 should be the cut off the syndrome. [21] Park et al and shin et al proposed that cut-off greater than 4.2 for the ratio of AP diameter and 4 for the ratio of the PSV should be used as sonographic criteria in diagnosing childhood NCS. [9],[22]

The most reliable non-invasive method for diagnosis of the NCS is CT angiography or MRA.

Kaneko et al showed ability of three dimen­sional helical computed tomography on the de­tection of the NCS. [23] Three dimensional CT angiography (3D-CTA), with its unique capa­bility of imaging in three planes, may be extremely useful in identifying a pathological abnormality of the SMA in the diagnosis the NCS. [24] The 3D-CTA was superior to invasive venography and ultrasonography in revealing LRV entrapment and compression, and the de­velopment of the collateral vein network. [24],[25]

Although 3D-CTA has become the investi­gation of choice in many centers but it has the disadvantage of radiation exposure.

MRA is safe and also reliable for diagnosing the NCS. MRA is non-invasive, radiation free tool and offers the advantage of multi-planar imaging of the body, thus allowing an excel­lent anatomical definition. MRA manifestions of the NCS include: dorso-lateral torsion of the left kidney, abnormally high course and dila­tation of the LRV and abnormal configuration of SMA. [15]

Management of the NCS depends on the se­verity of its symptoms. Patients with occasio­nal microhematuria, and mild flank pain and no anemia require only close follow up. How­ever, severe persistent or recurrent gross he­maturia that has caused anemia, sever flank pain due to ureteral passage of blood clots are indication for intervention. [19],[26]

Several surgical procedures for treatment of the NCS have been described: nephropexy, intra-or extravasular stents, LRV transposition, trans­position of SMA, gonado-caval bypass and renal autotransplantation.

Nephropexy with excision of varicosities is no more recommended because it fails to cor­rect underlying pathology. [15]

Experience with intra-or extravascular stents are limited to a number of case reports. On the other hand stents in the venous system have serious complications such as fibromuscular hyperplasia, venous occlusion, proximal mig­ration and embolization. So the role of stenting in the management of the NCS remains to be established. [15],[27]

Gil-Vernet first described transposition of re­nal vein for the treatment of extensive damage to the right lumbar ureter. [28] Stewart and Rei­man used this technique for the treatment of the NCS. [29] Hohenfellner et al reported their experience with transposition of the LRV in treatment of the NCS. They successfully trea­ted seven out of their eight patients with NCS associated with recurrent gross hematuria and flank pain by transposition of the LNV. [30] Ho­henfellner's series is the largest series reported to date. We have successfully used transposi­tion of the LRV to treat a patient with recur­rent high grade varicocele due to the NCS. [31]

In the recent study, two of our patients pre­viously underwent transposition of the LRV. But unfortunately, their gross hematuria did not subside. So decision was made for renal auto­transplantation in these patients.

Renal autotransplantation was first described by Hardy in 1963 for treatment of extensive ureteral injury. [32] Shokeir et al used renal au­totransplantation in management of sympto­matic NCS. They achieved satisfying results and hematuria caused in all the pariets. [26] Ali­El-Dein et al Recommended renal autotrans­plantation for anterior NCS and transposition of LRV for posterior (retroaortic left renal vein) NCS. [19]

Although renal autotransplantation is a more invasive procedure than transposition of the LRV but it may result in normalization of re­nal venous circulation and symptomatic im­provement. [30]

In conclusion, the low morbidity and excellent results of renal vein-related procedures make these operation the treatment of choice for the NCS. We believe that autotransplantation pro­vides better efficacy in eradication of symp­toms of NCS.

 
   References Top

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6.Salehipour M, khezri AA, Rasekhi AR, et al. The nutcracker syndrome: An under diagnosed cause for high grade varicocele. Iran J Med Sci 2007;32(3):1-4.  Back to cited text no. 6      
7.Scultetus AH, Villavicencio JH, Gillespie DL. The nutcracker syndrome: Its role in the pelvic venous disorders. J Vasc Surg 2001;34:812-9.  Back to cited text no. 7      
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9.Park SJ, Lim JW, Cho BS, et al. Nutcracker Syndrome in children with orthostatic protei­nuria: Diagnosis on the basis of Doppler sono­graphy. J Ultrasound Med 2002;21:39-45.  Back to cited text no. 9  [PUBMED]    
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14.Hohenfellner M, Steinbach F, Schultz-Lample D, et al. The nutcracker syndrome: New aspect of pathophysiology, diagnosis and treatment. J Urol 1991;146:685-8.  Back to cited text no. 14      
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16.De Schepper A. Nutcracker phenomenon of the renal vein causing left renal vein pathology. J Belge Rad 1972;55:507-11.  Back to cited text no. 16      
17.Beinard C, Sniderman KW, Saddekni, et al. Left renal vein hypertension: A cause of occult haematuria. Radiology 1982;145:647-50.  Back to cited text no. 17      
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19.Ali-EL-Dein B, Osman Y, Shehab EL-Din AB, et al. Anterior and posterior nutcracker syndrome. A report on 11 cases. Transplant Proc 2003;35: 851-3.  Back to cited text no. 19      
20.Lidove O, Orozco R, Gucry B, et al. A young woman with intermittent macroscopic haema­turia. Nephrol Dial Transplant 2001;16:853-5.  Back to cited text no. 20  [PUBMED]    
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22.Shin J 2nd, Park JM, Lee JS, et al. Effect of renal Doppler ultrasound on the detection of nutcracker syndrome in children with hematuria. Eur J Pediatr 2007;166:399-404.  Back to cited text no. 22      
23.Kaneko K, Kiya K, Nishimura K, et al. Nut­cracker phenomenon demonstrated by three dimensional computed tomography. Pediatr Nephrol 2001;16:745-7.  Back to cited text no. 23  [PUBMED]    
24.Fu WJ, Hong BF, Gao JP, et al. Nutcracker phenomenon: A new diagnostic method of multi­slice computed tomography angiography. Int J Urol 2006;13:870-3.  Back to cited text no. 24  [PUBMED]    
25.Kawamoto S, Horton KM, Fishman EK. Nut­cracker phenomenon demonstrated by multi­detector computed tomography with three di­mensional imaging. Urology 2008;71(2):228­-9.  Back to cited text no. 25      
26.Shokeir AA, EL-Diasty TA, Ghoneim MA. The nutcracker syndrome: New methods of diag­nosis and treatment. Br J Urol 1994;74:139-43.  Back to cited text no. 26  [PUBMED]    
27.Park YB, Lim SH, Ahn JH, et al. Nutcracker syndrome: Intravascular stenting approach. Nephrol Dial Transplant 2000;15:99-101.  Back to cited text no. 27  [PUBMED]    
28.Gilvernet JM. Descent of the right renal vein. J Urol 1978;120:668-70.  Back to cited text no. 28      
29.Stewart BH, Reiman G. left renal venous hyper­tension "nutcracker" syndrome: Managed by direct renocaval reimplantation. Urology 1982; 20:365-9.  Back to cited text no. 29  [PUBMED]    
30.Hohenfellner MD, Elia G, Hample C, et al. Transposition of the left renal vein for treat­ment of the nutcracker phenomenon: Long­term follow up. Urology 2002;59:354-7.  Back to cited text no. 30      
31.Salehipour M, Khezri AA, Rasekhi A, et al. Left renal vein transposition for treatment of the nutcracker syndrome. Arch Iran Med 2006; 9:161-2.  Back to cited text no. 31      
32.Hardy JD. High ureteral injury: management by auto-transplantation of the kidney. J Am Med Assoc 1963;184:97-101.  Back to cited text no. 32      

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Correspondence Address:
Mehdi Salehipour
Department of Urology, Faghihi Hospital, Shiraz
Iran
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    Abstract
    Introduction
    Patients and Methods
    Results
    Discussion
    References
 

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