|Year : 2010 | Volume
| Issue : 2 | Page : 237-241
|The role of renal autotransplantation in treatment of nutcracker syndrome
Mehdi Salehipour1, Alireza Rasekhi2, Mehdi Shirazi1, Abdolreza Haghpanah1, Shahrokh Jahanbini1, Seyed Ali Eslahi1
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 Publication||9-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 Jul 12];21:237-41. Available from: http://www.sjkdt.org/text.asp?2010/21/2/237/60059
| Introduction|| |
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. ,, Other possible symptoms include left flank pain,  left sided varicocele , pelvic congestion syndrome,  chronic fatigue syndrome in children,  or orthostatic proteinuria,  gastrointestinal symptoms  and arterial hypertension. 
The aim of the present work is to evaluate efficacy of renal autotransplantation in treatment of symptomatic NCS.
| Patients and Methods|| |
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|| |
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|| |
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".  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. , Wendel et al, hypothesized that posterior renal ptosis with stretching of the LRV over the aorta may contribute to NCS. 
Recently, Hohenfellner et al demonstrated that abnormal branching of the SMA from the aorta was the underlying cause of the NCS.  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. ,,
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.  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. 
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. 
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. 
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. 
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.  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.  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. ,
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.  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.  The 3D-CTA was superior to invasive venography and ultrasonography in revealing LRV entrapment and compression, and the development of the collateral vein network. ,
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. 
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. ,
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. 
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. ,
Gil-Vernet first described transposition of renal vein for the treatment of extensive damage to the right lumbar ureter.  Stewart and Reiman used this technique for the treatment of the NCS.  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.  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. 
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.  Shokeir et al used renal autotransplantation in management of symptomatic NCS. They achieved satisfying results and hematuria caused in all the pariets.  AliEl-Dein et al Recommended renal autotransplantation for anterior NCS and transposition of LRV for posterior (retroaortic left renal vein) NCS. 
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. 
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.
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Department of Urology, Faghihi Hospital, Shiraz
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