Nutcracker Syndrome

  • Ajay Savlania
  • Shivanesan Pitchai


Left renal vein (LRV) compression between the superior mesenteric artery (SMA) and abdominal aorta is referred to as the nutcracker phenomenon (NCP) or LRV entrapment and was first described by anatomist Grant in 1937 [1]. Abdominal aorta and superior mesenteric artery (SMA) act as two arms of a ‘nutcracker’ that can potentially compress the left renal vein [2]. The term nutcracker syndrome (NCS) is the clinical presentation of this phenomenon which is assigned to those patients who present with signs or symptoms of left renal venous congestion and have the anatomical LRV compression [3]. De Schepper, a Belgian physician named this phenomenon as ‘nutcracker syndrome’ [4]. Anatomically classical anterior nutcracker syndrome, in which there is compression of distal LRV between aorta and proximal SMA, is analogous to superior mesenteric artery syndrome (Wilkie’s syndrome) wherein the third part of duodenum gets compressed anteriorly by SMA and posteriorly by aorta [5–10]. The retroaortic or circumaortic renal vein may get compressed between the aorta and the vertebral body causing symptom which is called posterior nutcracker syndrome [11]. NCP on the right side, due to compression of large veins by the gravid uterus, has been also reported [12].


  1. 1.
    Grant JCB. Methods of anatomy. Baltimore: Williams & Wilkins; 1937. p. 158.Google Scholar
  2. 2.
    Shin JI, Lee JS. Nutcracker phenomenon or nutcracker syndrome? Nephrol Dial Transplant. 2005;20:2015.CrossRefGoogle Scholar
  3. 3.
    El Sadr AR, Mina E. Anatomical and surgical aspects of the operative management of varicoceles. Urol Cutan Rev. 1950;54:257–62.Google Scholar
  4. 4.
    De Schepper A. Nutcracker phenomenon of the renal vein causing left renal vein pathology. J Belg Radiol. 1972;55:507–11.PubMedGoogle Scholar
  5. 5.
    Barsoum MK, Shepherd RF, Welch TJ. Patient with both Wilkie syndrome and nutcracker syndrome. Vasc Med. 2008;13(3):247–50.CrossRefGoogle Scholar
  6. 6.
    Bedoya R, Lagman SM, Pennington GP, et al. Clinical and radiological aspects of the superior mesenteric artery syndrome. J Fla Med Assoc. 1986;73(9):686–9.PubMedGoogle Scholar
  7. 7.
    Cohen LB, Field SP, Sachar DB. The superior mesenteric artery syndrome: the disease that isn’t, or is it? J Clin Gastroenterol. 1985;7(2):113–6.CrossRefGoogle Scholar
  8. 8.
    Hearin JB. Duodenal ileus with special reference to superior mesenteric artery compression. Radiology. 1966;86(2):305–10.CrossRefGoogle Scholar
  9. 9.
    Pastershank SP. Left renal vein obstruction by a superior mesenteric artery. J Can Assoc Radiol. 1974;25(1):52–4.PubMedGoogle Scholar
  10. 10.
    Wilson-Storey D, MacKinlay GA. The superior mesenteric artery syndrome. J R Coll Surg Edinb. 1986;31(3):175–8.PubMedGoogle Scholar
  11. 11.
    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(2):851–3.CrossRefGoogle Scholar
  12. 12.
    Radisic MV, Feldman D, Diaz C, et al. Unexplained hematuria during pregnancy: right-sided nutcracker phenomenon. Int Urol Nephrol. 2007;39(3):709–11.CrossRefGoogle Scholar
  13. 13.
    Sharper KRL, Jackson JE, Williams G. The nutcracker syndrome: an uncommon cause of haematuria. Br J Urol. 1994;74:144–6.CrossRefGoogle Scholar
  14. 14.
    Satyapal KS. The renal veins: a review. Eur J Anat. 2003;7(Suppl 1):43–52.Google Scholar
  15. 15.
    Savlania A, Sashidhar K, Sidharth V, et al. Collateral venous drainage of solitary functioning left kidney with occluded renal vein following juxtarenal aortic aneurysm repair. J Vasc Surg Venous Lymphat Disord. 2015;3(3):325.CrossRefGoogle Scholar
  16. 16.
    Urban BA, Ratner LE, Fishman EK. Three-dimensional volume-rendered CT angiography of the renal arteries and veins: normal anatomy, variants, and clinical applications. Radiographics. 2001;21(2):373–86.CrossRefGoogle Scholar
  17. 17.
    Hohenfellner M, Steinbach F, Schultz-Lampel D, et al. Nutcracker syndrome: new aspects of pathophysiology, diagnosis and treatment. J Urol. 1991;146:685–8.CrossRefGoogle Scholar
  18. 18.
    Lau JLT, Lo R, Chan FL, et al. The posterior nutcracker. Haematuria secondary to retroaortic left renal vein. Urology. 1986;28:437–8.CrossRefGoogle Scholar
  19. 19.
    Wei SM, Chen ZD, Zhou M. Intravenous stent placement for treatment of nutcracker syndrome. J Urol. 2003;170:1934–5.CrossRefGoogle Scholar
  20. 20.
    Zhang HK, Shen LG, Li M, et al. Diagnosis and treatment of left renal venal entrapment syndrome. Chin J Gener Surg. 2001;16:511.Google Scholar
  21. 21.
    Beinart C, Sniderman KW, Tamura S, et al. Left renal vein to inferior vena cava pressure relationship in humans. J Urol. 1982;127:1070–1.CrossRefGoogle Scholar
  22. 22.
    Nishimura Y, Fushiki M, Yoshida M, et al. Left renal vein hypertension in patients with left renal bleeding of unknown origin. Radiology. 1986;59:663–7.CrossRefGoogle Scholar
  23. 23.
    Buschi AJ, Harrison RB, Norman A, et al. Distended left renal vein: CT/sonographic normal variant. AJR Am J Roentgenol. 1980;135:339–42.CrossRefGoogle Scholar
  24. 24.
    Beinart C, Sniderman KW, Saddekni S, et al. Left renal vein hypertension: a cause of occult hematuria. Radiology. 1982;145(3):647–50.CrossRefGoogle Scholar
  25. 25.
    Stewart BH, Reiman G. Left renal venous hypertension “nutcracker” syndrome: managed by direct renocaval reimplantation. Urology. 1982;20(4):365–9.CrossRefGoogle Scholar
  26. 26.
    Scultetus AH, Villavicencio JL, Gillespie DL. The nutcracker syndrome: its role in the pelvic venous disorders. J Vasc Surg. 2001;34(5):812–9.CrossRefGoogle Scholar
  27. 27.
    Maleux G, Stockx L, Wilms G, et al. Ovarian vein embolization for the treatment of pelvic congestion syndrome: long-term technical and clinical results. J Vasc Interv Radiol. 2000;11(7):859–64.CrossRefGoogle Scholar
  28. 28.
    Zerhouni EA, Siegelman SS, Walsh PC, et al. Elevated pressure in the left renal vein in patients with varicocele: preliminary observations. J Urol. 1980;123(4):512–3.CrossRefGoogle Scholar
  29. 29.
    Unlu M, Orguc S, Serter S, et al. Anatomic and hemodynamic evaluation of renal venous flow in varicocele formation using color Doppler sonography with emphasis on renal vein entrapment syndrome. Scand J Urol Nephrol. 2007;41(1):42–6.CrossRefGoogle Scholar
  30. 30.
    Park SJ, Lim JW, Ko YT, et al. Diagnosis of pelvic congestion syndrome using transabdominal and transvaginal sonography. AJR Am J Roentgenol. 2004;182(3):683–8.CrossRefGoogle Scholar
  31. 31.
    Rogers A, Beech A, Braithwaite B. Transperitoneal laparoscopic left gonadal vein ligation can be the right treatment option for pelvic congestion symptoms secondary to nutcracker syndrome. Vascular. 2007;15(4):238–40.CrossRefGoogle Scholar
  32. 32.
    Wendel RG, Crawford ED, Hehman KN. The “nutcracker” phenomenon: an unusual cause for renal varicosities with haematuria. J Urol. 1980;123(5):761–3.CrossRefGoogle Scholar
  33. 33.
    Takebayashi S, Ueki T, Ikeda N, et al. Diagnosis of the nutcracker syndrome with color Doppler sonography: correlation with flow patterns on retrograde left renal venography. AJR Am J Roentgenol. 1999;172:39–43.CrossRefGoogle Scholar
  34. 34.
    Braedel HU, Schindler E, Polsky MS. Selective renal phlebography in the diagnosis of renal pelvic and ureteric varices. Br J Urol. 1977;49(5):365–70.CrossRefGoogle Scholar
  35. 35.
    Fitoz S, Ekim M, Ozcakar ZB, et al. Nutcracker syndrome in children: the role of upright position examination and superior mesenteric artery angle measurement in the diagnosis. J Ultrasound Med. 2007;26(5):573–80.CrossRefGoogle Scholar
  36. 36.
    Stavros AT, Sickler KJ, Menter RR. Color duplex sonography of the nutcracker syndrome (aortomesenteric left renal vein compression). J Ultrasound Med. 1994;13(7):569–74.CrossRefGoogle Scholar
  37. 37.
    Zhang H, Li M, Jin W, et al. The left renal entrapment syndrome: diagnosis and treatment. Ann Vasc Surg. 2007;21(2):198–203.CrossRefGoogle Scholar
  38. 38.
    Sayfan J, Halevy A, Oland J, et al. Varicocele and left renal vein compression. Fertil Steril. 1984;41(3):411–7.CrossRefGoogle Scholar
  39. 39.
    Takahashi Y, Sano A, Matsuo M. An ultrasonographic classification for diverse clinical symptoms of pediatric nutcracker phenomenon. Clin Nephrol. 2005;64(1):47–54.CrossRefGoogle Scholar
  40. 40.
    Takahashi Y, Sano A, Matsuo M. An effective “transluminal balloon angioplasty” therapy for pediatric chronic fatigue syndrome with nutcracker phenomenon. Clin Nephrol. 2000;53(1):77–8.PubMedGoogle Scholar
  41. 41.
    Kim SH, Cho SW, Kim HD, et al. Nutcracker syndrome: diagnosis with Doppler US. Radiology. 1996;198:93–7.CrossRefGoogle Scholar
  42. 42.
    Fu W, et al. Diagnosis of the nutcracker phenomenon by multislice helical computed tomography angiography. Chin Med J. 2004;117:1873–5.PubMedGoogle Scholar
  43. 43.
    Kim KW, Cho JY, Kim SH, et al. Diagnostic value of computed tomographic findings of nutcracker syndrome: correlation with renal venography and renocaval pressure gradients. Eur J Radiol. 2011;80(3):648–54.CrossRefGoogle Scholar
  44. 44.
    Andrianne R, Limet R, Waltregny D, et al. Hematuria caused by nutcracker syndrome: preoperative confirmation of its presence. Prog Urol. 2002;12:1323–6.PubMedGoogle Scholar
  45. 45.
    He Y, Wu Z, Chen S, et al. Nutcracker syndrome—how well do we know it? Urology. 2014;83(1):12–7.CrossRefGoogle Scholar
  46. 46.
    Shokeir AA, el-Diasty TA, Ghoneim MA. The nutcracker syndrome: new methods of diagnosis and treatment. Br J Urol. 1994;74(2):139–43.CrossRefGoogle Scholar
  47. 47.
    Tanaka H, Waga S. Spontaneous remission of persistent severe haematuria in an adolescent with nutcracker syndrome: seven years’ observation. Clin Exp Nephrol. 2004;8(1):68–70.CrossRefGoogle Scholar
  48. 48.
    Reed NR, Kalra M, Bower TC, et al. Left renal vein transposition for nutcracker syndrome. J Vasc Surg. 2009;49:386–93; discussion: 393-4.CrossRefGoogle Scholar
  49. 49.
    Ha T-S, Lee E-J. ACE inhibition can improve orthostatic proteinuria associated with nutcracker syndrome. Pediatr Nephrol. 2006;21(11):1765–8.CrossRefGoogle Scholar
  50. 50.
    Neste MG, Narasimham DL, Belcher KK. Endovascular stent placement as a treatment for renal venous hypertension. J Vasc Interv Radiol. 1996;7:859.CrossRefGoogle Scholar
  51. 51.
    d’Archambeau O, Maes M, De Schepper AM. The pelvic congestion syndrome: role of the “nutcracker phenomenon” and results of endovascular treatment. JBR-BTR. 2004;87:1–8.PubMedGoogle Scholar
  52. 52.
    Erben Y, Gloviczki P, Kalra M, et al. Treatment of nutcracker syndrome with open and endovascular interventions. J Vasc Surg Venous Lymphat Disord. 2015;3(4):389–96.CrossRefGoogle Scholar
  53. 53.
    Hartung O, Azghari A, Barthelemy P, et al. Laparoscopic transposition of the left renal vein into the inferior vena cava for nutcracker syndrome. J Vasc Surg. 2010;52:738–41.CrossRefGoogle Scholar
  54. 54.
    Chuang CK, et al. The nutcracker syndrome managed by autotransplantation. J Urol. 1997;157:1833–4.CrossRefGoogle Scholar
  55. 55.
    Salehipour M, et al. The role of renal autotransplantation in treatment of nutcracker syndrome. Saudi J Kidney Dis Transpl. 2010;21:237–41.PubMedGoogle Scholar
  56. 56.
    Thompson PN, Darling RC 3rd, Chang BB, et al. A case of nutcracker syndrome: treatment by mesoaortic transposition. J Vasc Surg. 1992;16:663–5.CrossRefGoogle Scholar
  57. 57.
    Chung BI, Gill IS. Laparoscopic splenorenal venous bypass for nutcracker syndrome. J Vasc Surg. 2009;49(5):1319–23.CrossRefGoogle Scholar
  58. 58.
    Barnes RW, Fleisher HL 3rd, Redman JF, et al. 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.CrossRefGoogle Scholar
  59. 59.
    Hohenfellner M, D’Elia G, Hampel C, et al. Transposition of the left renal vein for treatment of the nutcracker phenomenon: long-term follow-up. Urology. 2002;59:354–7.CrossRefGoogle Scholar
  60. 60.
    Chen S, Zhang H, Shi H, et al. Endovascular stenting for treatment of nutcracker syndrome: report of 61 cases with long-term follow up. J Urol. 2011;186:570–5.CrossRefGoogle Scholar
  61. 61.
    Wang X, Zhang Y, Li C, et al. Results of endovascular treatment for patients with nutcracker syndrome. J Vasc Surg. 2012;56:142–8.CrossRefGoogle Scholar
  62. 62.
    Cohen F, Amabile P, Varoquaux A, et al. Endovascular treatment of circumaortic nutcracker syndrome. J Vasc Interv Radiol. 2009;20:1255–6.CrossRefGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2018

Authors and Affiliations

  • Ajay Savlania
    • 1
  • Shivanesan Pitchai
    • 2
  1. 1.Postgraduate Institute of Medical Education and ResearchTrivandrumIndia
  2. 2.Sree Chitra Tirunal Institute for Medical Sciences and TechnologyTrivandrumIndia

Personalised recommendations