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Nutcracker syndrome in children: review of symptom, diagnosis, and treatment

  • Diana S. Kalantar (Department of Environmental Science, Policy, and Management, Rausser College of Natural Resources, University of California Berkeley) ;
  • Se Jin Park (Department of Pediatrics, Daejeon Eulji Medical Center, Eulji University School of Medicine) ;
  • Jae Il Shin (Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine)
  • Received : 2023.10.11
  • Accepted : 2023.12.09
  • Published : 2023.12.31

Abstract

Renal nutcracker syndrome (NCS) is the entrapment of the left renal vein between the abdominal aorta and superior mesenteric artery. Although uncommon in pediatric patients, early diagnosis is crucial to avoid potential severe complications, such as anemia or renal vein thrombosis. NCS presents a variety of symptoms, most commonly including "Triade's symptoms"-hematuria, proteinuria, and flank pain. Diagnosis and treatment include invasive and noninvasive management, although due to a lack of pediatric clinical studies, management is widely variable. Conservative diagnosis and treatment are recommended as a first-line option for pediatric patients; however, invasive surgical treatment may be recommended based on symptom severity. This review aims to provide a comprehensive overview of NCS in children to better understand the widely variable incidence, occurrence, and management from early on to allow for early-onset management.

Keywords

References

  1. Kolber MK, Cui Z, Chen CK, Habibollahi P, Kalva SP. Nutcracker syndrome: diagnosis and therapy. Cardiovasc Diagn Ther 2021;11:1140-9. https://doi.org/10.21037/cdt-20-160
  2. Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc 2010;85:552-9. https://doi.org/10.4065/mcp.2009.0586
  3. Miro I, Serrano A, Perez-Ardavin J, March JA, Polo A, Conca MA, et al. Eighteen years of experience with pediatric nutcracker syndrome: the importance of the conservative approach. J Pediatr Urol 2020;16:218.
  4. Granata A, Distefano G, Sturiale A, Figuera M, Foti PV, Palmucci S, et al. From nutcracker phenomenon to nutcracker syndrome: a pictorial review. Diagnostics (Basel) 2021;11:101.
  5. Agarwal A, Litra F, Barr LL. A rare cause of abdominal and flank pain in children: nutcracker syndrome. Cureus 2021;13:e16422.
  6. El-Sadr AR, Mina E. Anatomical and surgical aspects in the operative management of varicocele. Urol Cutaneous Rev 1950;54:257-62.
  7. de Schepper A. "Nutcracker" phenomenon of the renal vein and venous pathology of the left kidney. J Belge Radiol 1972;55:507-11.
  8. Chait A, Matasar KW, Fabian CE, Mellins HZ. Vascular impressions on the ureters. Am J Roentgenol Radium Ther Nucl Med 1971;111:729-49. https://doi.org/10.2214/ajr.111.4.729
  9. Meyer J, Rother U, Stehr M, Meyer A. Nutcracker syndrome in children: appearance, diagnostics, and treatment: a systematic review. J Pediatr Surg 2022;57:716-22. https://doi.org/10.1016/j.jpedsurg.2021.12.019
  10. Mallat F, Hmida W, Othmen MB, Mosbah F. Mixed nutcracker syndrome with left renal vein duplication: a severe and exceptional presentation in an 18-year-old boy. Urol Ann 2015;7:244-7. https://doi.org/10.4103/0974-7796.150494
  11. Vianello FA, Mazzoni MB, Peeters GG, Fossali EF, Camozzi P, Bianchetti MG, et al. Micro- and macroscopic hematuria caused by renal vein entrapment: systematic review of the literature. Pediatr Nephrol 2016;31:175-84. https://doi.org/10.1007/s00467-015-3045-2
  12. Oteki T, Nagase S, Hirayama A, Sugimoto H, Hirayama K, Hattori K, et al. Nutcracker syndrome associated with severe anemia and mild proteinuria. Clin Nephrol 2004;62:62-5. https://doi.org/10.5414/CNP62062
  13. Basaran EG, Yilmaz AC, Gungor O, Tayfur AC, Buyukkaragoz B. Clinical profile and renal ultrasound characteristics of children with nutcracker syndrome in Turkey. Indian Pediatr 2022;59:28-30. https://doi.org/10.1007/s13312-022-2415-7
  14. Reddy DK, Shekar P A. Nutcracker syndrome: a rare but important cause of varicocele in adolescent boys. Urology 2020;141:143-6. https://doi.org/10.1016/j.urology.2020.04.008
  15. Shin JI, Lee JS. ACE inhibition in nutcracker syndrome with orthostatic proteinuria: how about a hemodynamic effect? Pediatr Nephrol 2007;22:758-60. https://doi.org/10.1007/s00467-006-0421-y
  16. Alaygut D, Bayram M, Soylu A, Cakmakci H, Turkmen M, Kavukcu S. Clinical course of children with nutcracker syndrome. Urology 2013;82:686-90. https://doi.org/10.1016/j.urology.2013.03.048
  17. Mazzoni MB, Kottanatu L, Simonetti GD, Ragazzi M, Bianchetti MG, Fossali EF, et al. Renal vein obstruction and orthostatic proteinuria: a review. Nephrol Dial Transplant 2011;26:562-5. https://doi.org/10.1093/ndt/gfq444
  18. Li H, Sun X, Liu G, Zhang Y, Chu J, Deng C, et al. Endovascular stent placement for nutcracker phenomenon. J Xray Sci Technol 2013;21:95-102. https://doi.org/10.3233/XST-130356
  19. Jiang Y, Gan Z, Wang Q, Chen Y, Jiang Y. Bibliometric and visual analysis of research on nutcracker syndrome from 1974 to 2021: a systematic review. Medicine (Baltimore) 2022;101:e29939.
  20. Park SJ, Shin JI. Comment on: nutcracker and SMA syndromes: what is the normal SMA angle in children? (Eur J Radiol. 2012;81(August (8)):e854-61). Eur J Radiol 2013;82:1034.
  21. Beinart C, Sniderman KW, Tamura S, Vaughan ED, Sos TA. Left renal vein to inferior vena cava pressure relationship in humans. J Urol 1982;127:1070-1. https://doi.org/10.1016/S0022-5347(17)54230-5
  22. Park SJ, Lim JW, Cho BS, Yoon TY, Oh JH. Nutcracker syndrome in children with orthostatic proteinuria: diagnosis on the basis of Doppler sonography. J Ultrasound Med 2002;21:39-46. https://doi.org/10.7863/jum.2002.21.1.39
  23. Cheon JE, Kim WS, Kim IO, Kim SH, Yeon KM, Ha IS, et al. Nutcracker syndrome in children with gross haematuria: Doppler sonographic evaluation of the left renal vein. Pediatr Radiol 2006;36:682-6. https://doi.org/10.1007/s00247-006-0145-y
  24. Shin JI, Park JM, Lee JS, Kim MJ. Effect of renal Doppler ultrasound on the detection of nutcracker syndrome in children with hematuria. Eur J Pediatr 2007;166:399-404. https://doi.org/10.1007/s00431-006-0252-7
  25. Nishimura Y, Fushiki M, Yoshida M, Nakamura K, Imai M, Ono T, et al. Left renal vein hypertension in patients with left renal bleeding of unknown origin. Radiology 1986;160:663-7. https://doi.org/10.1148/radiology.160.3.3737903
  26. Kim SH, Cho SW, Kim HD, Chung JW, Park JH, Han MC. Nutcracker syndrome: diagnosis with Doppler US. Radiology 1996;198:93-7. https://doi.org/10.1148/radiology.198.1.8539413
  27. Atasoy D, Cansu A, Bekircavusoglu AF, Ozdogan EB, Ahmetoglu A. The utility of magnetic resonance angiography in children with nutcracker syndrome. Turk J Med Sci 2021;51:2396-402. https://doi.org/10.3906/sag-2101-14
  28. Kim SH. Doppler US and CT diagnosis of nutcracker syndrome. Korean J Radiol 2019;20:1627-37. https://doi.org/10.3348/kjr.2019.0084
  29. Ahmed K, Sampath R, Khan MS. Current trends in the diagnosis and management of renal nutcracker syndrome: a review. Eur J Vasc Endovasc Surg 2006;31:410-6. https://doi.org/10.1016/j.ejvs.2005.05.045
  30. Min KW, Lee OK, Kim MK. Nutcracker syndrome combined with superior mesenteric artery syndrome in a pediatric patient: a case report. Child Kidney Dis 2018;22:75-80. https://doi.org/10.3339/jkspn.2018.22.2.75
  31. Arthurs OJ, Mehta U, Set PA. Nutcracker and SMA syndromes: what is the normal SMA angle in children? Eur J Radiol 2012;81:e854-61. https://doi.org/10.1016/j.ejrad.2012.04.010
  32. Gulleroglu NB, Gulleroglu K, Uslu N, Baskin E. Left renal vein entrapment in postural proteinuria: the diagnostic utility of the aortomesenteric angle. Eur J Pediatr 2022;181:3339-43. https://doi.org/10.1007/s00431-022-04551-3
  33. Fitoz S, Ekim M, Ozcakar ZB, Elhan AH, Yalcinkaya F. Nutcracker syndrome in children: the role of upright position examination and superior mesenteric artery angle measurement in the diagnosis. J Ultrasound Med 2007;26:573-80. https://doi.org/10.7863/jum.2007.26.5.573
  34. Li S, Liu Q, Wang J, Pang X, Zhang Y, Cheng Y, et al. Association between left renal vein entrapment and varicocele recurrence: a cohort study in 3042 patients. Sci Rep 2018;8:10534.
  35. Takahashi Y, Ohta S, Sano A, Kuroda Y, Kaji Y, Matsuki M, et al. Does severe nutcracker phenomenon cause pediatric chronic fatigue? Clin Nephrol 2000;53:174-81.
  36. Lin TH, Lin CC, Tsai JD. Superior mesenteric artery syndrome and nutcracker syndrome. Pediatr Neonatol 2020;61:351-2. https://doi.org/10.1016/j.pedneo.2019.12.003
  37. Du MC, Chen HY, Zhang YX, Zhang LB. Pica with superior mesenteric artery syndrome and nutcracker syndrome as imaging manifestation: a case report. Asian J Surg 2022;45:2038-9. https://doi.org/10.1016/j.asjsur.2022.04.084
  38. England J, Li N. Superior mesenteric artery syndrome: a review of the literature. J Am Coll Emerg Physicians Open 2021;2:e12454.
  39. Gungorer V, Ozturk M, Arslan S. A rare cause of recurrent abdominal pain: the coexistence of Wilkie's syndrome and nutcracker syndrome. Arch Argent Pediatr 2023;121:e202102373.
  40. Ananthan K, Onida S, Davies AH. Nutcracker syndrome: an update on current diagnostic criteria and management guidelines. Eur J Vasc Endovasc Surg 2017;53:886-94. https://doi.org/10.1016/j.ejvs.2017.02.015
  41. Stewart JM, Gewitz MH, Weldon A, Munoz J. Patterns of orthostatic intolerance: the orthostatic tachycardia syndrome and adolescent chronic fatigue. J Pediatr 1999;135:218-25. https://doi.org/10.1016/S0022-3476(99)70025-9
  42. Juhn JH, Yoo BW, Lee JS, Kim MJ. A case of nutcracker syndrome associated with orthostatic proteinuria and idiopathic chronic fatigue in a child. J Korean Soc Pediatr Nephrol 2001;5:64-8.
  43. Shin JI, Lee JS. Can chronic fatigue symptoms associated with nutcracker phenomenon be treated with aspirin? Med Hypotheses 2007;69:704-5. https://doi.org/10.1016/j.mehy.2007.01.011
  44. Shin JI, Park JM, Shin YH, Lee JS, Kim MJ, Jeong HJ. Nutcracker syndrome combined with IgA nephropathy in a child with recurrent hematuria. Pediatr Int 2006;48:324-6. https://doi.org/10.1111/j.1442-200X.2006.02212.x
  45. Strul N, Vaessen S, Collard L, Ghuysen MS, Khamis J, Brisbois D, et al. Clinical case of the month: nutcracker syndrome in association with a painful nephrologic disease. Rev Med Liege 2007;62:73-6.
  46. Shin JI, Lee SM, Park JM, Shin YH, Lee JS, Kim MJ. Doppler ultrasonographic detection of nutcracker syndrome in a young child with intussusception: a case report. Acta Paediatr 2005;94:1510-3. https://doi.org/10.1111/j.1651-2227.2005.tb01830.x
  47. Shin JI, Park JM, Shin YH, Lee JS, Kim MJ. Superimposition of nutcracker syndrome in a haematuric child with Henoch-Schonlein purpura. Int J Clin Pract 2005;59:1472-5. https://doi.org/10.1111/j.1742-1241.2005.00590.x
  48. Rao J, Yang J, Liu Z, Wang L, Liu L, Yin Z, et al. Right retrocaval ureter and left nutcracker syndrome: a case report. Urology 2008;71:1226.
  49. Altugan FS, Ekim M, Fitoz S, Ozcakar ZB, Burgu B, Yalcinkaya F, et al. Nutcracker syndrome with urolithiasis. J Pediatr Urol 2010;6:519-21. https://doi.org/10.1016/j.jpurol.2010.05.014
  50. Preza Fernandes J, Amorim R, Gomes MJ, Oliveira V, Reis A, Ribeiro-Castro J. Posterior nutcracker syndrome with left renal vein duplication: a rare cause of haematuria in a 12-year-old boy. Case Rep Urol 2012;2012:849681.
  51. Yavuz S, Ece A, Corapli M, Ilter C, Guven R. Nutcracker syndrome complicating with renal abscess. J Pak Med Assoc 2016;66:470-2.
  52. Scholbach T. From the nutcracker-phenomenon of the left renal vein to the midline congestion syndrome as a cause of migraine, headache, back and abdominal pain and functional disorders of pelvic organs. Med Hypotheses 2007;68:1318-27. https://doi.org/10.1016/j.mehy.2006.10.040
  53. Al-Zoubi NA, Al-Ghalayini IF, Al-Okour R. Nutcracker syndrome associated with celiacomesentric trunk anomaly: case report. Int J Nephrol Renovasc Dis 2017;10:285-8. https://doi.org/10.2147/IJNRD.S146814
  54. Nishio Y, Kawano Y, Hara S. Nutcracker syndrome complicated with intestinal malrotation. BMJ Case Rep 2019;12:e231230.
  55. Perez-Ardavin J, Serrano Durba A, Miro I, Conca Baena MA, March-Villalba JA, Polo Rodrigo A, et al. Spontaneous spermatic vein thrombosis in pediatric patients: a condition to be considered. Cir Pediatr 2020;33:99-101.
  56. Chang CT, Hung CC, Ng KK, Yen TH. Nutcracker syndrome and left unilateral haematuria. Nephrol Dial Transplant 2005;20:460-1. https://doi.org/10.1093/ndt/gfh482
  57. Zhang H, Li M, Jin W, San P, Xu P, Pan S. The left renal entrapment syndrome: diagnosis and treatment. Ann Vasc Surg 2007;21:198-203. https://doi.org/10.1016/j.avsg.2006.10.021
  58. Velasquez CA, Saeyeldin A, Zafar MA, Brownstein AJ, Erben Y. A systematic review on management of nutcracker syndrome. J Vasc Surg Venous Lymphat Disord 2018;6:271-8. https://doi.org/10.1016/j.jvsv.2017.11.005
  59. Shin JI, Park JM, Lee SM, Shin YH, Kim JH, Lee JS, et al. Factors affecting spontaneous resolution of hematuria in childhood nutcracker syndrome. Pediatr Nephrol 2005;20:609-13. https://doi.org/10.1007/s00467-004-1799-z
  60. Reed NR, Kalra M, Bower TC, Vrtiska TJ, Ricotta JJ, Gloviczki P. Left renal vein transposition for nutcracker syndrome. J Vasc Surg 2009;49:386-94. https://doi.org/10.1016/j.jvs.2008.09.051
  61. Chuang CK, Chu SH, Lai PC. The nutcracker syndrome managed by autotransplantation. J Urol 1997;157:1833-4. https://doi.org/10.1016/S0022-5347(01)64872-9
  62. Xu D, Liu Y, Gao Y, Zhang L, Wang J, Che J, et al. Management of renal nutcracker syndrome by retroperitoneal laparoscopic nephrectomy with ex vivo autograft repair and autotransplantation: a case report and review of the literature. J Med Case Rep 2009;3:82.
  63. Thompson PN, Darling RC, Chang BB, Shah DM, Leather RP. A case of nutcracker syndrome: treatment by mesoaortic transposition. J Vasc Surg 1992;16:663-5. https://doi.org/10.1016/0741-5214(92)90176-9
  64. Benrashid E, Turley RS, Mureebe L, Shortell CK. Gonadal vein transposition in the treatment of nutcracker syndrome. J Vasc Surg 2016;64:845.
  65. Shaper KR, Jackson JE, Williams G. The nutcracker syndrome: an uncommon cause of haematuria. Br J Urol 1994;74:144-6. https://doi.org/10.1111/j.1464-410X.1994.tb16575.x
  66. de Macedo GL, Dos Santos MA, Sarris AB, Gomes RZ. Diagnosis and treatment of the nutcracker syndrome: a review of the last 10 years. J Vasc Bras 2018;17:220-8. https://doi.org/10.1590/1677-5449.012417
  67. Hao J, Shi H, Xu H, Zhu J, Zhou J, Du T. Ultrasound-assisted microsurgical left spermatic-inferior epigastric vein anastomosis for treating nutcracker syndrome-associated varicocele. Int Urol Nephrol 2019;51:1925-32. https://doi.org/10.1007/s11255-019-02226-x
  68. Amaral J, Honjo O, Hannick JH, Rickard M, Lorenzo AJ. In situ gonadal vein valvulotomy and side-to-side gonado-iliac bypass for the management of nutcracker syndrome in an adolescent with a solitary kidney and absence of pelvic congestion. Urology 2019;126:200-3. https://doi.org/10.1016/j.urology.2018.12.033
  69. Ha TS, Lee EJ. ACE inhibition can improve orthostatic proteinuria associated with nutcracker syndrome. Pediatr Nephrol 2006;21:1765-8. https://doi.org/10.1007/s00467-006-0206-3