Child Kidney Dis.  2023 Dec;27(2):89-96. 10.3339/ckd.23.018.

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

Affiliations
  • 1Department of Environmental Science, Policy, and Management, Rausser College of Natural Resources, University of California Berkeley, Berkeley, CA, USA
  • 2Department of Pediatrics, Daejeon Eulji Medical Center, Eulji University School of Medicine, Daejeon, Republic of Korea
  • 3Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea

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 firstline 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.

Keyword

Aorta, abdominal; Mesenteric artery, superior; Renal nutcracker syndrome; Renal veins

Figure

  • Fig. 1. Illustration of renal nutcracker syndrome, depicting the compression of the LRV between the abdominal aorta and the SMA. LRA, left renal artery; LRV, left renal vein; SMA, superior mesenteric artery.

  • Fig. 2. Images of Doppler ultrasonography. (A) The diameters of left renal vein in the aortomesenteric portion (0.13 cm) and the hilar portion (0.69 cm). (B) The peak velocity in the aortomesenteric portion (95 cm/sec). (C) The peak velocity in the hilar portion (18 cm/sec). (D) The aortomesenteric angle (18°). Reused from Min et al. Child Kidney Dis 2018;22:75-80 [30].

  • Fig. 3. Abdomen and pelvis computed tomography (enhancement). (A) Left renal vein was compressed by the aorta and the superior mesenteric artery (aorto-superior mesenteric artery distance: 4.6 mm). (B) Aortomesenteric angle was 18° in the sagittal plane. Reused from Min et al. Child Kidney Dis 2018;22:75-80 [30].


Reference

References

1. Kolber MK, Cui Z, Chen CK, Habibollahi P, Kalva SP. Nutcracker syndrome: diagnosis and therapy. Cardiovasc Diagn Ther. 2021; 11:1140–9.
Article
2. Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc. 2010; 85:552–9.
Article
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.
Article
5. Agarwal A, Litra F, Barr LL. A rare cause of abdominal and flank pain in children: nutcracker syndrome. Cureus. 2021; 13:e16422.
Article
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.
Article
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.
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.
Article
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.
Article
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.
Article
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.
Article
14. Reddy DK, Shekar P A. Nutcracker syndrome: a rare but important cause of varicocele in adolescent boys. Urology. 2020; 141:143–6.
Article
15. Shin JI, Lee JS. ACE inhibition in nutcracker syndrome with orthostatic proteinuria: how about a hemodynamic effect? Pediatr Nephrol. 2007; 22:758–60.
Article
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.
Article
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.
Article
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.
Article
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.
Article
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.
Article
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.
Article
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.
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.
Article
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.
Article
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.
Article
26. Kim SH, Cho SW, Kim HD, Chung JW, Park JH, Han MC. Nutcracker syndrome: diagnosis with Doppler US. Radiology. 1996; 198:93–7.
Article
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.
Article
28. Kim SH. Doppler US and CT diagnosis of nutcracker syndrome. Korean J Radiol. 2019; 20:1627–37.
Article
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.
Article
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.
Article
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.
Article
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.
Article
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.
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.
Article
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.
Article
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.
Article
38. England J, Li N. Superior mesenteric artery syndrome: a review of the literature. J Am Coll Emerg Physicians Open. 2021; 2:e12454.
Article
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.
Article
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.
Article
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.
Article
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.
Article
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.
Article
47. Shin JI, Park JM, Shin YH, Lee JS, Kim MJ. Superimposition of nutcracker syndrome in a haematuric child with Henoch-Schönlein purpura. Int J Clin Pract. 2005; 59:1472–5.
Article
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.
Article
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.
Article
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.
Article
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.
Article
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.
Article
54. Nishio Y, Kawano Y, Hara S. Nutcracker syndrome complicated with intestinal malrotation. BMJ Case Rep. 2019; 12:e231230.
Article
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.
Article
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.
Article
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.
Article
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.
Article
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.
Article
61. Chuang CK, Chu SH, Lai PC. The nutcracker syndrome managed by autotransplantation. J Urol. 1997; 157:1833–4.
Article
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.
Article
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.
64. Benrashid E, Turley RS, Mureebe L, Shortell CK. Gonadal vein transposition in the treatment of nutcracker syndrome. J Vasc Surg. 2016; 64:845.
Article
65. Shaper KR, Jackson JE, Williams G. The nutcracker syndrome: an uncommon cause of haematuria. Br J Urol. 1994; 74:144–6.
Article
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.
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.
Article
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.
Article
69. Ha TS, Lee EJ. ACE inhibition can improve orthostatic proteinuria associated with nutcracker syndrome. Pediatr Nephrol. 2006; 21:1765–8.
Article
Full Text Links
  • CKD
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr