Percutaneous Nitinol Stent Implantation in the Treatment of Nutcracker Syndrome in Young Adults

Antonio Basile, MD, Dimitrios Tsetis, MD, Giacomo Calcara, MD, Michele Figuera, MD, Maria Teresa Patti, MD, Giovanni Carlo Ettorre, PhD, and Antonio Granata, MD

The present report describes three young adults with nutcracker syndrome caused by left renal vein stenosis managed with nitinol stent implantation. The patients treated included a 20-year-old woman with persistent microhematuria and dyspareunia and two 18-year-old men with proteinuria, hematuria, and flank pain. All three patients were asymptomatic after a follow-up of 14 –18 months.
J Vasc Interv Radiol 2007; 18:1042–1046 Abbreviations: IVC inferior vena cava, LRV left renal vein, SMA superior mesenteric artery

THE clinical syndrome caused by impingement of the left renal vein (LRV) between the superior mesenteric artery (SMA) and abdominal aorta has been termed nutcracker syndrome (1). Although often asymptomatic, entrapment of the LRV is a rare but acknowledged cause of ovarian vein syndrome, varicocele, pelviureteral and peripelvic varices, LRV hypertension, hematuria, orthostatic proteinuria, and unexplained left flank or abdominal pain (2). Surgical approaches for the treatment of nutcracker syndrome include nephrectomy, nephropexy, renocaval reimplantation, and autotransplantation (2).

Published reports of successful treatment of LRV compression in young adults and of the use of nitinol stents have been sparse. Herein we describe successful treatment of LRV compression in three young adults, with follow-up ranging between 14 and 18 months. Institutional review board approval was not needed for this study.

CASE REPORTS
Case 1 A 20-year-old woman was admitted to the nephrology department for clinical assessment after the detection of microscopic hematuria 1 month earlier. She reported left flank pain for the previous 9 months. On questioning, she also reported symptoms of dyspareunia. Findings of physical examination were unremarkable. Urinalysis revealed microscopic hematuria and minimal proteinuria. Renal ultrasonography (US) revealed normalsized kidneys (right, 107 mm; left, 118 mm in length). Repeated US and Doppler flow analysis revealed a patent LRV that abruptly narrowed between the aorta and the SMA. The anteroposterior diameters of the LRV were 12.8 mm in the hilar area and 2.1

From the Department of Diagnostic and Interventional Radiology (A.B., G.C., M.T.P.), Ospedale Ferrarotto; Departments of Radiology (M.F.) and Nephrology (A.G.), Ospedale Vittorio Emanuele; Department of Radiology (G.C.E.), University Hospital of Catania, Catania, Italy; and Department of Radiology (D.T.), University Hospital of Heraklion, Medical School of Crete, Crete, Greece. Received December 7, 2006; final revision received May 11, 2007; accepted May 14, 2007. Address correspondence to A.B., Via Trieste 14, 95127 Catania, Italy; E-mail: antodoc@yahoo.com None of the authors have identified a conflict of interest. © SIR, 2007 DOI: 10.1016/j.jvir.2007.05.017

mm in the narrow portion, and the ratio of anteroposterior diameters was 6.10. The peak velocities in the LRV at the hilar and aortomesenteric portions were 19 cm/sec and 87 cm/sec, respectively. After informed consent was obtained, the LRV was catheterized from a right common femoral vein approach. The pressure gradient between the LRV and the inferior vena cava (IVC) at the level between the aorta and the SMA was 6 mm Hg. Venography revealed capsular venous varices joining the left ovarian vein at the lumbar region (Fig 1a), and a 14 40-mm nitinol self-expandable stent (Luminexx; C.R. Bard Angiomed, Karlsruhe, Germany) was then inserted through a 7-F sheath over a stiff wire (0.035-inch Amplatz super-stiff wire; Boston Scientific, Nanterre, France). To prevent the stent from protruding into the IVC, it was inserted under road-mapping guidance with a long sheath at the LRV ostium. Postprocedural venography demonstrated normal flow through the LRV with no pressure gradient between the LRV and the IVC (Fig 1b). To prevent thrombosis, intravenous heparin was administered for 2 days and was followed by oral aspirin therapy. The patient experienced flank pain for 3 days after the intervention,

1042

but while he was in an upright and ambulatory position in the daytime. the LRV was catheterized from a right common femoral . Doppler US peak velocity measurements in the LRV were 23 cm/sec in the hilar area and 107 cm/sec in the narrow portion. Urinalysis was normal except for 3 proteinuria and 24-hour urinary protein excretion of 0. urine specimens contained protein. Doppler study showed that the diameter of the hilar and aortomesenteric portions of the LRV were 13. At 15- month follow-up. Blood chemistry findings were normal. After informed consent was obtained.7 mm in the narrow portion and the ratio of anteroposterior diameters was 7.2 g. At 18-month followup. with disappearance of the renocaval pressure gradient.1. the patient was asymptomatic. The peak velocities in the LRV at the hilar and aortomesenteric portions were 9 cm/sec and 78 cm/ sec. After informed consent was obtained. He was normotensive and the findings of physical examination were normal. To prevent the stent from protruding into the IVC. for a ratio of 8. blood urea nitrogen. it was inserted under road-mapping guidance with a long sheath at the LRV ostium. for a ratio of 4. total protein. Repeated US and Doppler flow analysis revealed a patent LRV that abruptly narrowed between the aorta and the SMA.R.65. the patient was asymptomatic with normal Doppler US findings. a ventral ectopic origin of the right renal artery increased the stenosis just at the renocaval junction with dilated lumbar varices. US examination showed anomalous position and dilation of the LRV: anteroposterior diameters of the LRV were 13. Enhanced computed tomography (CT) imaging demonstrated stenosis of the LRV between the aorta and SMA.035-inch Amplatz super-stiff wire. Case 2 A well developed 18-year-old man was admitted to the nephrology department for clinical assessment after the detection of proteinuria with left flank pain. Enhanced CT imaging demonstrated stenosis of the LRV between the aorta and SMA (Fig 2a).Volume 18 Number 8 Basile et al • 1043 Figure 1. but there was a recurrence 1 month later. Cystoscopy showed light bleeding from the left ureter. in addition. Bard Angiomed) were inserted through a 7-F sheath over a stiff wire (0. intravenous heparin was administered for 2 days and was followed by oral aspirin therapy. Postprocedural venography demonstrated no retrograde flow toward the lumbar veins.6. There was no renal disease in the patient’s history.2 mm respectively. the LRV was catheterized. as was creatinine clearance. He had undergone percutaneous embolization for a varicocele 8 months earlier. (b) Control image after stent placement shows direct flow toward the IVC. two 14 40-mm self-expandable nitinol stents (Luminexx. capsular varices are no longer detected. The pressure gradient between the LRV and the IVC was 7 mm Hg with the presence of lumbar varices. Case 3 An 18-year-old man was admitted to the nephrology department with a 10-month history of intermittent gross hematuria. To prevent thrombosis.5 mm in the hilar area and 1. His nighttime urine was protein-free. (a) Phlebography of the LRV demonstrates the capsular varices supplying the left ovarian vein. Serum creatinine. C. A very high -globulin peak was detected by urinary protein electrophoresis. Urinalysis revealed hematuria and minimal proteinuria. and analgesic therapy was given during this period.9 –1.6 mm and 2. From a right common femoral vein approach.9. phlebography was performed to confirm and allow treatment of the stenosis. respectively. for a ratio of 6. US imaging of kidneys was normal. and albumin levels were within normal limits. Boston Scientific).

035-inch Amplatz superstiff wire. Postprocedural venography demonstrated normal flow through the renal vein with no pressure gradient between the LRV and IVC (Fig 2c). Venography revealed capsular varices (Fig 2b). which increases venous capacitance. and analgesic therapy was given during this period. which likely correspond to two different stages of the disease (2). After 14 months of follow-up. (a) Multidetector CT image reveals the LRV stenosis between the aorta and the SMA. Bard Angiomed) was inserted through a 7-F sheath over a stiff wire (0. Because of the competence of the venous valve of the ovarian or lumbar vein.R. (b) Phlebography of the LRV demonstrates capsular varices. Two peaks of incidence at young and middle age have been reported. . it was inserted under road-mapping guidance with a long sheath at the LRV ostium. (c) Control image after stent placement shows direct flow toward the IVC. To prevent thrombosis. At an early stage. LRV hypertension leads to the development of direct communication between the dilated venous sinuses and adjacent calyces with related essential hematuria and flank pain (4 – 6). A 14 40-mm self-expandable nitinol stent (Luminexx. intravenous heparin was administered for 2 days and was followed by oral aspirin therapy. To prevent the stent from protruding into IVC. the patient was asymptomatic with normal findings on Doppler US flow analysis.1044 • Nitinol Stents for Nutcracker Syndrome in Young Adults August 2007 JVIR Figure 2. Boston Scientific). leading to retrograde endovenous hypertension (3). DISCUSSION The nutcracker syndrome is caused by LRV compression between the SMA and aorta. The patient experienced flank pain for 24 hours after the intervention. no direct or low reflux is seen in these vessels at this stage. the LRV compression leads to LRV hypertension compensated by development of venous collateral vessels (usually renal capsular veins). vein approach. C. The pressure gradient between the LRV and the IVC at the level between the aorta and the SMA was 7 mm Hg.

PCS pelvic congestion syndrome. of Pts. leading to pelvic or vulvar varices with the classical symptomatology of pelvic congestion syndrome (ie. renal vein bypass (16). microscopic hematuria (n 2). transposition of the SMA (18). Diagnostic imaging may involve Doppler US.3) Chen et al (29). 2001 Wei et al (24). and autotransplantation of the left kidney (19) have been reported to alleviate LRV compression. left flank pain Flank pain Gross hematuria Long-standing incapacitating PCS (n 2). but phlebography with renal vein and IVC manometry is definitive. 2005 Hartung et al (28). after the first case of LRV stent implantation reported by Neste et al in 1996 (20). Kim et al (10) identified Doppler US criteria related to the nutcracker phenomenon by measuring LRV diameter and peak flow velocity. follow-up or conservative treatment with hemostatic agents (eg. or renal function impairment unresponsive to conservative therapy after 24-month follow-up. including severe hematuria with related anemia.—NS not specified. in addition. 2004 Kim et al (27). left limb atypical varices with venous insufficiency symptoms (n 1) Gross hematuria 3 50 (mean. more aggressive management is suggested (8). carbazochrome) have been suggested. 14. transposition of the LRV (17). or magnetic resonance (MR) imaging examinations. Recently. The authors stated that LRV hypertension provides the driving force for retrograde blood flow into the internal spermatic vein and that it is correlated with Doppler spectral analysis (11). pelvic pain. 1996 Segawa et al (21). dyspareunia. The therapy for nutcracker syndrome is still controversial. and these tools are also able to distinguish anterior nutcracker phenomena (ie. 2003 Lin et al (25). MR imaging and helical CT have been recently used in the diagnosis of this phenomenon.7). back pain. Kim and colleagues (11) recently reported the first study of the correlation between Doppler parameters of the LRV and the renocaval pressure gradient in pediatric patients with varicocele and concluded that the nutcracker phenomenon may be an important cause of pediatric varicocele. 2003 van der Laan et al (26). 16-mm/60-mm (n 2). or dysmenorrhea). 24. For more symptomatic conditions. gonadocaval bypass in patients with nutcracker syndrome associated with pelvic varices (7). dysuria.7 (mean) 4.9).Volume 18 Number 8 Basile et al • 1045 Published Cases of Endovascular Stent Implantation for Nutcracker Syndrome Study. 1999 Park et al (22). Surgical procedures including medial nephropexy (6). hematuria Stent NS 70 20-mm covered stent 18-mm nitinol stent 60-mm Palmaz stent 16 30-mm Wallstent and 20 40-mm nitinol stent Wallstent 12 40-mm Wallstent NS 10 65-mm Wallstent 20-mm/60-mm (n 1). with varying results and complication rates. 19 cases of interventional radiologic management of nutcracker syndrome have been reported with follow-up periods ranging from 3 to 54 months (7. This is mainly encountered in middle-aged women (6.5 (mean. 2001 Chiesa et al (23).2–26. particularly a renocaval pressure gradient—measured as the pressure in the LRV minus the pressure in the IVC— of more than 3 mm Hg (8. The authors suggested that distal-to-prox- imal diameter ratios and flow velocity ratios exceeding 5.0 represented cutoff levels for the diagnosis of nutcracker phenomenon.7 15 24 18. the persistence of chronic LRV hypertension causes valvular incompetence and massive reflux of blood into ovarian or lumbar veins.0) F/34. . left lumbar pain (n 2). 40-mm/ 16-mm (n 2) Follow-up (months) NS 6 4 24 12 1 3 1 1 5 M/37 M/22. CT. LRV stenosis between aortomesenteric angle) and posterior nutcracker phenomena (ie. retroaortic LRV compressed between the aorta and spine) (12–15). For asymp- tomatic hematuria and orthostatic albuminuria.0 Gross hematuria Gross hematuria. US findings suggesting nutcracker syndrome are related to proximal-to-aortomesenteric LRV diameter ratios and aortomesenteric-to-proximal LRV peak velocity ratios.20 –29) (Table). 1 1 1 1 1 Sex/Age (y) NS F/40 M/47 F/40 M/17 Symptoms NS Asymptomatic gross hematuria Hematuria PCS Flank pain.7 F/36 F/47 18. Year Neste et al (20). 2000 Scultetus et al (7). 2005 No. 2005 3 M/10 (mean) 10 40-mm selfexpandable nitinol stent 24–36 Note. At a later stage.

Magnetic resonance angiography in nutcracker phenomenon. et al. Yang JY. 54:257–262. J Vasc Interv Radiol 1996. 83:783–789. 2006. 17. et al. venography. Br J Urol 1994. et al. 16. The nutcracker syndrome: new aspects of pathophysiology. Intravenous stent placement for treatment of the nutcracker syndrome. 20:609 – 613. Zhang N. Gillespie DL. Sebastia Cerqueda C. 25. 20. Chu J. Factors affecting spontaneous resolution of hematuria in childhood nutcracker syndrome. van den Berg JC. 123:761–763. Radiology 1996. as was the case in the patients described herein. Park YB. Nutcracker or left renal vein compression phenomenon: multidetector computed tomography findings and clinical significance. Khan MS. a study reporting the treatment of three consecutive male pediatric patients with nutcracker syndrome has been published (29). Eur Radiol 2002. Recently. “Nutcracker” phenomenon of the renal vein and venous pathology of the left kidney. Chu SH. Vos JA. Hohenfellner M. 41:889 – 892. J Urol 1991. J Urol 2003. and in-stent restenosis. 22. Park JM. This article suggested that stent implantation may be indicated even in pediatric patients. 12(suppl): S133–S135. we have additional concerns regarding possible complications related to the rigidity of a stent in the setting of normal development of the body. 15:99 –101. which perfectly matches the two peaks of incidence of symptomatology previously described. Ahmed K. 14. 15:1745–1751. et al. Left renal vein hypertension in patients with left renal bleeding of unknown origin. Narasimham DL. et al. Azuma H. Chin Med J 2003. 5. 4. The nutcracker syndrome: its role in the pelvic venous disorders. Treatment of six cases of left renal nutcracker phenomenon: surgery and endografting. Han MC. 55:507–511. Re: endovascular stent placement for the treatment of nutcracker phenomenon in three pediatric patients. Radiology 1986. Neste MG. Endovascular stent placement as a treatment for renal venous hypertension. 16:1529 –1533. The nutcracker syndrome managed by autotransplantation. Domanovits H. 241: 228 –234. Renal fornical hemorrhages: their pathogenesis and treatment. Scultetus AH. 160: 663– 667. 13. Lee SM. Left renal venous hypertension ‘nutcracker’ syn- 18. Pytel A. et al. Eur J Vasc Endovasc Surg. 12. Endovascular stent placement for the treatment of nutcracker phenomenon in three pediatric patients. Fushiki M. 10. Long-term follow-up after endovascular stent placement for treatment of nutcracker syndrome. The “nutcracker” phenomenon: an unusual cause for renal varicosities with hematuria. Radiology 2006. Boye de la Presa R. For this reason. J Vasc Interv Radiol 2006.1046 • Nitinol Stents for Nutcracker Syndrome in Young Adults August 2007 JVIR Various types and sizes of stents have been used. Ahn JH. In addition. J Vasc Interv Radiol 2005. Endovascular stenting in the treatment of pelvic vein congestion caused by nutcracker syndrome: lessons learned from the first five cases. 6. SchultzLampel D. Anatomical and surgical aspects of the operative management of varicoceles. 148:433– 437. 2. Alvarez-Castells A. 9. 8:652– 655. 198:93–97. 20:120 –129. Shin JI. et al. Reiman G. 11. Nutcracker syndrome: intravascular stenting approach. 7:859 – 861. Villavicencio JL. et al. Grisoli D. 30. Nishimura Y. Kurokawa S. van der Laan L. Holmes RJ. drome managed by direct renocaval reimplantation. Aizawa S. Rudloff U. Kim WS. Shin JI. et al. Urology 1999. J Belge Radiol 1972. The centralvenous compression syndrome: rare. 21. J Vasc Interv Radiol 2005. Marone EM. Segawa N. Hemodynamic investigation of the left renal vein in pediatric varicocele: Doppler US. Endovascular stenting for the nutcracker phenomenon. Nutcracker syndrome: diagnosis with Doppler US. Hartung O. 8. Cheon JE. Mina A. Nephrol Dial Transplant 2000. 31. Kim MJ. 51:259 –260. Lim SH. Kim HD. the ages of treated patients have ranged from 17 to 47 years. Lai PC. 157: 1833–1834. Urol Cutan Rev 1950. Chung JW. Lin WQ. J Endovasc Ther 2001. Zhonghua Wai Ke Za Zhi 2003. 146:685– 688. Hehman KN. Ned Tijdschr Geneeskd 2004. 29. Latorraca R. Park JH. 20:365–369 Zhang H. Posterior “nutcracker” phenomenon in a patient with abdominal aortic aneurysm. J Urol 1980. Wei SM. Jackson JE. Mesoaortic compression of the left renal vein (nutcracker syndrome): case reports and review of the literature. Ann Vasc Surg. Siegel D. Rare reported complications of these procedures are stent fracture. 28. 116:1782– 1784. but adequately treatable with endovascular stenting. Chiesa R. et al. 15. Zhou M. migration. Kim S. 34:812– 819. et al. and pressure measurements. Kim CW. Boufi M. et al. Sampath R. Williams G. 170:1934 –1935. 3. Prem JT. Yoshida M. Stewart BH. but we and others (30) believe the preferred therapy for hematuria in childhood nutcracker syndrome is observation. Kim SJ. Kaneko K. Chen W. ranging in diameter from 10 to 20 mm. Although longer follow-up in more patients is still needed to assess the role of stent implantation in the management of nutcracker syndrome. Cuellar i Calabria H. J Vasc Surg 2005. Pediatr Nephrol 2005. diagnosis and treatment. Obinata K. thrombosis. 7. Eur Radiol 2005. 71:667– 676. MacMahon HE. 19. Moldwin R. percutaneous treatment with nitinol stents in young adults seems to be effective and safe in the short term. de Boer E. Lee JS. 16:428 – 431. Chuang CK. 17:1063. nitinol stents may be indicated in the treatment of stenosis of an LRV compressed between two large pulsatile arteries. In the literature published to date. Crawford ED. Diagnosis and therapy of the nutcracker phenomenon: long-term follow-up. Ohtomo Y. as aortomesenteric compression may resolve spontaneously as a result of physical development during childhood. 42:275–280. Wendel RG. Stuhlinger HG. Puig S. Essential renal hematuria. Li M. Kim IO. Yamashiro Y. Huang HF. De Schepper A. et al. 2006. Faust GR. and there may be possible problems related to the rigidity of the stent during normal body development (31). Clin Nephrol 1999. Moll FL. The nutcracker syndrome: an uncommon cause of haematuria. 53:631– 633. J Urol 1960. Current trends in the diagnosis and management of renal nutcracker syndrome: a review. Expandable metallic stent placement for nutcracker phenomenon. 24. Urology 1982. Their use in nutcracker syndrome could also improve the effectiveness of endovascular therapy in such cases. Chen ZD. 27. El Sadr AR. J Urol 1997. Li M. Quiroga Gomez S. Iwamoto Y. Sharper KRL. Anzuini A. 26. J Vasc Surg 2001. . Belcher KK. References 1. Steinbach F. 74:144 –146. Miranda A. we would offer endovascular stent implantation in such patients only when body development is finished. Particularly because of their flexibility and resistance to external force. J Urol 1954. Cho SW. Kim SH. 23. 31:410 – 416.

Sign up to vote on this title
UsefulNot useful