NUTCRACKER SYNDROME: A RARE AND UNDERDIAGNOSED PATHOLOGY ? OUR EXPERIENCE, REVIEW OF THE LITERATURE AND PROPOSAL OF A NEW ANATOMICAL CLASSIFICATION OF NUTCRACKER SYNDROME

  • Miguel Machado Instituto de Ciências Biomédicas Abel Salazar— Universidade do Porto
  • Rui Machado Instituto de Ciências Biomédicas Abel Salazar— Universidade do Porto; Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Universitário do Porto
  • Daniel Mendes Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Universitário do Porto
  • Rui de Almeida Instituto de Ciências Biomédicas Abel Salazar— Universidade do Porto; Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Universitário do Porto
Keywords: Nutcracker Syndrome, Nutcracker Phenomenon, Kidney transplant, Kidney auto transplant

Abstract

Objective: Evaluate our experience with treated patients with the diagnosis of Nutcracker Syndrome, literature review and proposal of a new classification for the Nutcracker Syndrome

Introduction: The Nutcracker syndrome (NCS) can result from the left renal vein (LRV) compression between the superior mesenteric artery and the aorta (Anterior NCS), or more rarely by compression of the LRV between the aorta and the lumbar vertebra when the vein passes behind the aorta, (posterior NCS). The association of the two anomalies can occur when a circunaortic renal vein exists. The prevalence of NCS is unknown, but it appears to be rare and under diagnosed.

Methods and Materials: A retrospective descriptive analysis case by case, based on the clinical and imagiological records related to seven patients diagnosed with Nutcracker Syndrome, in the period between January-2011 and March- 2017, in our center . The diagnosis was made by clinical suspicion, after exclusion of other more common etiologies, and the observation of a significant hemodynamic stenosis of the venous kidney drainage by computed tomography. We observed 7 patients, 57,1% (4 patientes) females, with a mean age of 21,7 years with 85,7% in their second or third decade of life. Posterior NCS were observed in 42,9% (3 patients), anterior NCS in 28,6% (2 patients), antero-posterior NCS in 14,3% (1 patient), and compression of a left side vena cava by the aorta when it crosses to right side in 14,3% (1 patient). The most common symptom was macroscopic hematúria (71,4%), followed by flank pain exacerbated by exercise (28,6%) and congestion pelvic syndrome in 14,3%. Tree patients were submitted to surgery, two were treated with a renal auto transplant and one a left ilio-caval stenting. One patient submitted to an auto transplant necessitated a nephrectomy secondary to a renal venous thrombosis. The other four patients followed a conservative management and surveillance. After a mean follow-up of 2,7 years, five patients were asymptomatic, one had a self-limited haematuria episode, and another kept asymptomatic proteinuria.

Discussion/Conclusion: The diagnosis of NCS can be made at any age, particularly in the second and third decade of life and is reported to be more frequent in females. In our experience we observed a female/male ratio of 1,3, and a high proportion of posterior NCS (42,9%). The posterior NCS type was the most common type of NCS, going against the literature that reports only 19 cases since 2017. We describe a fourth type of Nutcracker Syndrome, that we call other anatomic type of NCS and we propose a new anatomical classification of NCS. A policy of wait and see, decreased the necessity of surgery to near one half of the patients (42,9%). The choice of renal auto transplant was due to our long and good experience in kidney transplantation, with more than 2500 cases done. The nephrectomy was done by laparoscopy to reduce the invasiveness, and we should pay attention to the tension of the renal vein, as it can result in thromboses as was one of our cases. Despite the recent reports of good results with stenting of the renal vein, we keep concern about the durability of the stents in this young population and it isn’t our first choice.

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References

1. Shin JI , Lee JS. Nutcracker phenomenon or nutcracker syndrome
Nephrol Dial Transplant. 20, 2005, Vol. 1, pp. 120-129
2. Machado M, Machado R, Mendes D, Almeida R. May-Thurner
Syndrome associated with a Nutcracker Syndrome, clinical case
and literature review. Angiologia e Cirurgia Vascular, 2017 volume
3, numero 1, pp. 52-57
3. El-Sadr AR e Mina A. Anatomical and surgical aspects in the operative
management of varicocele. Urol Cutaneous Rev. 54, 1950, Vol.
5, pp. 257-262.
4. de Schepper A. "Nutcracker" phenomenon of the renal vein and
venous pathology of the left kidney [in Dutch]. J Belge Radiol. 55,
1972, Vol. 5, pp. 507-511.
5. Chait A, et al. Vascular impressions on the ureters. Am J Roentgenol
Radium Ther Nucl Med. 111, 1971, Vol. 4, pp. 729-749
6. Hearin JB. Duodenal ileus with special reference to superior mesenteric
artery compression. Radiology. 86, 1966, Vol. 2, pp. 305-310.
7. Wilson-Storey D , MacKinlay GA. The superior mesenteric artery
syndrome. J R Coll Surg Edinb. 31, 1986, Vol. 3, pp. 175-178.
8. Bedoya R, et al. Clinical and radiological aspects of the superior mesenteric
artery syndrome. J Fla Med Assoc. 73, 1986, Vol. 9, pp. 686-689.
9. Cohen LB, Field SP , Sachar DB. The superior mesenteric artery
syndrome: the disease that isn't, or is it? J Clin Gastroenterol. 7,
1985, Vol. 2, pp. 113-116.
10. Pastershank SP. Left renal vein obstruction by a superior mesenteric
artery. J Can Assoc Radiol. 25, 1974, Vol. 1, pp. 52-54.
11. Barsoum MK, Shepherd RF, Welch TJ. Patient with both Wilkie
syndrome and Nutcracker syndrome. Vasc Med. 13, 2008, Vol. 3,
pp. 247-250
12. Latorre J. Malformaciones congénitas. J Latorre Vilallonga. Sector
iliocava: Anatomía, Fisiopatología, Exploraciones y Tratamiento.
Barcelona : Uriach, 1993, pp. 70-112.
13. 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. 21,
2001, Vol. 2, pp. 373-386.
14. Ali-El-Dein B, et al. Anterior and posterior nutcracker syndrome: a
report on 11 cases. Transplant Proc. 35, 2003, Vol. 2, pp. 851-853.
15. Takahashi Y, et al. Does severe nutcracker phenomenon cause
pediatric chronic fatigue? Clin Nephrol. 53, 2000, Vol. 3, pp. 174-181.
16. Shaper KR, Jackson JE, Williams G. The nutcracker syndrome: an uncommon
cause of haematuria. BR J Urol. 74, 1994, Vol. 2, pp. 144-146.
17. Hohenfellner M, et al. The nutcracker syndrome: new aspects of
pathophysiology, diagnosis and treatment. J Urol. 146, 1991, Vol.
3, pp. 685-688.
18. Fu WJ, et al. Diagnosis of the nutcracker phenomenon by multislice
helical computed tomography angiography. Chin Med J (Engl).
117, 2004, Vol. 12, pp. 1873-1875.
19. Wendel RG, Crawford ED, Hehman KN. The "nutcracker" phenomenon:
an unusual cause for renal varicosities with hematuria. J
Urol. 123, 1980, Vol. 5, pp. 761-763.
20. Shokeir AA, el-Diasty TA, Ghoneim, MA. The nutcracker syndrome:
new methods of diagnosis and treatment. Br J Urol. 74, 1994,
Vol. 2, pp. 139-143.
21. Cope C, Isard HJ. Left renal vein entrapment: a new diagnosis finding
in retroperitoneal disease. Radiology. 92, 1969, Vol. 4, pp. 867-872.
22. Zerhouni EA, et al. Elevated pressure in the left renal vein in
patients with varicocele: preliminary observations. J Urol. 123,
1980, Vol. 4, pp. 512-513.
23. Ariyoshi A , Nagase K. Renal hematuria caused by "nutcracker"
phenomenon: a more logical surgical management. Urology. 35,
1990, Vol. 2, pp. 168-170.
24. Rudloff U, et al. Mesoaortic compression of the left renal vein
(nutcracker syndrome): case reports and review of the literature.
Ann Vasc Surg. 20, 2006, Vol. 1, pp. 120-129.
25. Beinart C, et al. Left renal vein hypertension: a cause of occult
hematuria. Radiology. 145, 1982, pp. 647-650.
26. MacMahon HE , Latorraca R. Essential renal hematuria. J Urol. 71,
1954, pp. 667-676.
27. Pytel A. Renal fornical hemorrhages: their pathogenesis and
treatment. J Urol. 83, 1960, pp. 783-789.
28. Low AI , Matz LR. Haematuria and renal fornical lesions. Br J Urol.
44, 1972, pp. 681-691.
29. Shin JI, et al. Effect of renal Doppler ultrasound on the detection
of nutcracker syndrome in children with hematuria. Eur J Pediatr.
166, 2007, Vol. 5, pp. 399-404.
30. Ekim M, et al. The "nutcracker phenomenon" wit orthostatic
proteinuria: case reports. Clin Nephrol. 65, 2006, pp. 280-283.
31. Poyraz AK, et al. Evaluation of left renal vein entrapment using
multidetector computed tomography. Acta Radiol. 54, 2013, pp.
144-148.
32. Unlu M, 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. 41, 2007, Vol. 1, pp. 42-46.
33. Takebayashi S, et al. Diagnosis of the nutcracker syndrome with
color Doppler sonography: correlation with flow patterns on
retrograde left renal venography. AJR Am J Roentgenol. 172, 1999,
Vol. 1, pp. 39-43.
34. Kurklinsky AK , Rooke TW. Nutcracker Phenomenon and Nutcracker
Syndrome. Mayo Clin Proc. 85, 2010, Vol. 6, pp. 552-559.
35. Wolfish NM, McLaine PN , Martin D. Renal vein entrapment syndrome:
frequency and diagnosis - a lesson in conservatism. Clin
Nephrol. 26, 1986, pp. 96-100.
36. Stewart BH , Reiman G. Left renal venous hypertension "nutcracker"
syndrome. Managed by direct renocaval re-implantation.
Urology. 20, 1982, pp. 365-369.
37. Kim SH, et al. Nutcracker syndrome: diagnosis with Doppler US.
Radiology. 198, 1996, pp. 93-97.
38. Beinart C, et al. Left renal to inferior vena cava pressure relationship
in humans. J Urol. 127, 1982, pp. 1070-1071.
39. Reed NR, et al. Left renal vein transposition for nutcracker
syndrome. J Vasc Surg. 49, 2009, p. 386.
40. Hohenfellner M, et al. Transposition of the left renal vein for
treatment of the nutcracker phenomenon: long-term follow-up.
Urology. 2002, pp. 354-357.
41. Shokeir AA et al. The Nutcracker Syndrome: new methods of diagnosis
and treatment. Br J Urol. 74,1994, pp. 139-43.
42. Hartung O, et al. Laparoscopic transposition of the left renal vein
into the inferior vena cava for nutcracker syndrome. J Vasc Surg.
49, 2009, pp. 1319-1323.
43. Chung BI , Gill IS. Laparoscopic splenorenal venous bypass for
nutcracker syndrome. J Vasc Surg. 49, 2009, pp. 1319-1323.
44. Neste MG, Narasimham DL , Belcher KK. Endovascular stent placement
as a treatment for renal venous hypertension. J Vasc Interv
Radiol. 7, 1996, p. 859.
45. Chen S, et al. Endovascular stenting for treatment of nutcracker
syndrome: report of 61 cases with long-term followup. The Journal
of Urology. 2011, Vol. 186, pp. 570-575.
46. Marone EM, et al. Surgical treatment of posterior nutcracker
syndrome. J Vasc Surg. 54, 2011, pp. 844-7.
47. Ananthan K, Onida S, Davies A H. Nutcracker Syndrome: An Update
on Current Diagnostic Criteria and Management Guidelines Eur
J Vasc Endovasc Surg (2017) 53, 886-894
48. Chuang CK, Chu SH , Lai PC . The nutcracker syndrome managed by
autotransplantation. The Journal of Urology. 157, 1997, pp. 1833-
1834.
Published
2018-06-07
Section
Original Article

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