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Newly-identified symptoms of left renal vein entrapment syndrome mimicking orthostatic disturbance

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Abstract

Background

In addition to the urinary abnormalities, symptoms of left renal vein entrapment between the aorta and superior mesenteric artery (left renal vein entrapment syndrome, LRVES) may include abdominal and flank pain as well as chronic fatigue. We investigated various LRVES symptoms in this study.

Methods

In 53 pediatric LRVES patients treated at our department, 22 had a score of 5 points or higher on orthostasis. Initial evaluation of LRVES by abdominal ultrasonography showed a stenotic-to-prestenotic vein diameter ratio of 0.2 or less. Definitive diagnosis was made by computed tomography and magnetic resonance angiography. Cortisol, catecholamine (CA), and brain natriuretic peptide (BNP) were also measured.

Results

The frequency of LRVES was 2.5 times higher in girls than in boys. Low or very low body mass indexes were seen in both sexes. The most common initial finding was urine abnormalities, followed by dizziness and malaise. In 6 patients, orthostasis precluded school attendance. Ten patients had orthostasis scores above 12. Patients unable to attend school had either low levels of plasma or urinary cortisol. Midodrine significantly decreased orthostasis scores. Some patients required treatment with fludrocortisone. Plasma CA, renin, and BNP levels were all normal.

Conclusions

Locally excessive venous pressure may cause reversible adrenal dysfunction with transitory Addisonian symptoms. Children with cryptogenic malaise or severe orthostasis should be evaluated for LRVES.

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References

  1. De Schepper A. “Nutcracker” phenomenon of the renal vein and venous pathology of the left kidney. J Belge Radiol 1972;55:507–511.

    PubMed  Google Scholar 

  2. Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc 2010;85:552–529.

    Article  PubMed  Google Scholar 

  3. Bhanji A, Malcolm P, Karim M. Nutcracker syndrome and radiographic evaluation of loin pain and hematuria. Am J Kidney Dis 2010;55:1142–1145.

    Article  PubMed  Google Scholar 

  4. Natale F, Granato C, Aronne L, Di Marco GM, Lo Priore E, Mocerino R, et al. Nutcracker syndrome. Eur J Echocardiogr 2009;10:993.

    Article  PubMed  Google Scholar 

  5. Mohamed M, Siham S, Souad C, Hassan J, Ahmed A, Mohamed B, et al. The nutcracker syndrome. Nephrol Ther 2009;5:58–60.

    Article  PubMed  Google Scholar 

  6. Ozcan A, Gonul II, Sakallioglu O, Oztas E. Nutcracker syndrome in a child with familial Mediterranean fever (FMF) disease: renalultrastructural features. Int Urol Nephrol 2009;41:1047–1053.

    Article  PubMed  Google Scholar 

  7. Kim SH, Cho SW, Kim HD, Chung JW, Park JH, Han MC. Nutcracker syndrome: diagnosis with Doppler US. Radiology 1996;198:93–97.

    PubMed  CAS  Google Scholar 

  8. Takahashi Y, Akaishi K, Sano A, Kuroda Y. Intra-arterial digital subtraction angiography for children with idiopathic renal bleeding: a diagnosis of nutcracker phenomenon. Clin Nephrol 1988;30:134–140.

    PubMed  CAS  Google Scholar 

  9. Karaosmanoğlu D, Karcaaltincaba M, Akata D, Ozmen M. Unusual causes of left renal vein compression along its course: MDCT fi ndings in patients with nutcracker and pelvic congestion syndrome. Surg Radiol Anat 2010;32:323–327.

    Article  PubMed  Google Scholar 

  10. Romera-Villegas A, Vila-Coll R, Cairols-Castellote M, Poveda-Monge R, Masuet-Aumatell C, Grinyó-Boira M. The importance of a standing position in the diagnosis of Nutcracker phenomenon by duplex sonography. Int Angiol 2009;28:461–468.

    PubMed  CAS  Google Scholar 

  11. 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;53:174–181.

    PubMed  CAS  Google Scholar 

  12. Takemura T, Iwasa H, Yamamoto S, Hino S, Fukushima K, Isokawa S, et al. Clinical and radiological features in four adolescents with nutcracker syndrome. Pediatr Nephrol 2000;14:1002–1005.

    Article  PubMed  CAS  Google Scholar 

  13. Takahashi Y, Sano A, Matsuo M. An ultrasonographic classification for diverse clinical symptoms of pediatric nutcracker phenomenon. Clin Nephrol 2005;64:47–54.

    PubMed  CAS  Google Scholar 

  14. Tanaka H, Yamaguchi H, Matushima R, Tamai H. Instantaneous orthostatic hypotension in children and adolescents: a new entity of orthostatic intolerance. Pediatr Res 1999;46:691–696.

    Article  PubMed  CAS  Google Scholar 

  15. Takemura T, Murakami K, Miyazato H, Yagi K, Yoshioka K. Expression of Fas antigen and Bcl-2 in human glomerulonephritis. Kidney Int 1995;48:1886–1892.

    Article  PubMed  CAS  Google Scholar 

  16. Uchiyama T. Childhood hypertension in Japanese. In: Guidelines for the Management of Hypertension 2009. Tokyo: The Japanese Society of Hypertension, 2009:83–86.

    Google Scholar 

  17. Unlu M, Orguc S, Serter S, Pekindil G, Pabuscu Y. Anatomic and hemodynamic evaluation of renal venous fl ow in varicocele formation using color Doppler sonography with emphasis on renal vein entrapment syndrome. Scand J Urol Nephrol 2007;41:42–46.

    Article  PubMed  Google Scholar 

  18. Takebayashi S, Ueki T, Ikeda N, Fujikawa A. 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.

    PubMed  CAS  Google Scholar 

  19. Shintaku N, Takahashi Y, Akaishi K, Sano A, Kuroda Y. Entrapment of left renal vein in children with orthostatic proteinuria. Pediatr Nephrol 1990;4:324–327.

    Article  PubMed  CAS  Google Scholar 

  20. Ekim M, Ozçakar ZB, Fitoz S, Soygür T, Yüksel S, Acar B, et al. The “nutcracker phenomenon” with orthostatic proteinuria: case reports. Clin Nephrol 2006;65:280–283.

    PubMed  CAS  Google Scholar 

  21. Venkatachalam S, Bumpus K, Kapadia SR, Gray B, Lyden S, Shishehbor MH. The nutcracker syndrome. Ann Vasc Surg 2011;25:1154–1164.

    Article  PubMed  Google Scholar 

  22. Shin JI, Lee JS. Can chronic fatigue symptoms associated with nutcracker phenomenon be treated with aspirin. Med Hypotheses 2007;69:704–705.

    Article  PubMed  CAS  Google Scholar 

  23. Menard MT. Nutcracker syndrome: when should it be treated and how? Perspect Vasc Surg Endovasc Ther 2009;21:117–124.

    Article  PubMed  Google Scholar 

  24. Cohen F, Amabile P, Varoquaux A, Piquet P, Bartoli JM, Vidal V, et al. Endovascular treatment of circumaortic nutcracker syndrome. J Vasc Interv Radiol 2009;20:1255–1257.

    Article  PubMed  Google Scholar 

  25. Tanaka H, Waga S. Spontaneous remission of persistent severe hematuria in an adolescent with nutcracker syndrome: seven years’ observation. Clin Exp Nephrol 2004;8:68–70.

    Article  PubMed  Google Scholar 

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Correspondence to Tsukasa Takemura.

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Koshimichi, M., Sugimoto, K., Yanagida, H. et al. Newly-identified symptoms of left renal vein entrapment syndrome mimicking orthostatic disturbance. World J Pediatr 8, 116–122 (2012). https://doi.org/10.1007/s12519-012-0349-1

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  • DOI: https://doi.org/10.1007/s12519-012-0349-1

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