ÇOCUKLARDA NUTCRACKER SENDROMU: DOPPLER ULTRASONOGRAFİNİN SEMPTOMATİK HASTALARDAKİ ROLÜ

Öz

Amaç: Bu çalışmanın amacı, Nutcracker sendromu tanısı almış pediatrik hastaların klinik özelliklerini değerlendirmek ve klinik belirtilerle sol renal ven doppler ultrasonografi bulguları arasında bir ilişki olup olmadığını belirlemektir.

Gereç ve yöntemler: Ocak 2015- Aralık 2018 tarihleri arasında, Nutcracker sendromu tanısı alan hastaların klinik verileri geriye dönük olarak incelendi. Hastalar semptomatik (yan ağrısı, makroskopik hematüri veya her ikisi) ve semptomatik olmayan (tesadüfen saptanan mikroskobik hematüri ve proteinüri) olarak gruplandırıldı ve bu iki gruptaki doppler ultrasonografi bulguları değerlendirildi.

Bulgular: Nutcracker sendromu tanısı olan 41 hasta (28 kız ve 11 erkek hasta, ortalama yaş 13,6±3,37 yıl) çalışmaya dahil edildi. 26 (%63,4) hastada asemptomatik Nutcracker sendromu bulundu ve bu hastaların %46,1’i mikroskobik hematüri ve %42,3’ü izole proteinüri değerlendirilmesi için başvurdu. En sık görülen belirtiler sol yan ağrısı (%33,3), sol yan ağrısı ile hematüri birlikteliği (%26,6) idi. Sol renal ven hiler bölüm çapının aortomesenterik bölüm çapına oranının ortalama değeri 5,07±1,02 idi. Sol renal ven aorta-mezenterik/hiler bölge pik akım oranı ortalaması 7,17±1,86 saptandı. Pik akım oranları asemptomatik çocuklarda semptomatik gruba göre anlamlı derecede yüksekti (p=0.041).

Çıkarım: Nutcracker sendromu tanısı, proteinüri ve hematüri gibi belirtilerin varlığında düşünülmeli ve böbrek biyopsisinden önce mutlaka ekarte edilmelidir. Ek olarak çalışmamız, sol renal vende artmış kompresyonun çoğunlukla mikroskobik hematüri ve proteinüri olan hastalarda gözlendiğini göstermiştir. 

Anahtar Kelimeler

• Çocuk,Doppler ultrasonografi,Nutcracker sendromu,Sol renal ven

NUTCRACKER SYNDROME IN CHILDREN: THE ROLE OF DOPPLER ULTRASONOGRAPHY IN SYMPTOMATIC PATIENTS

Öz

Objective: The aim of this study is to evaluate the clinical characteristics of pediatric patients with Nutcracker syndrome and to determine whether there is a relationship between clinical signs and left renal vein doppler ultrasonography findings.

Materials and methods: Between January 2015 and December 2018, clinical data of patients diagnosed with Nutcracker syndrome were retrospectively reviewed. Patients were devided into two groups as symptomatic (loin pain, macroscopic hematuria or both) and non-symptomatic (incidentally detected microscopic hematuria and proteinuria), and the doppler ultrasonography findings in both group were evaluated.

Results: Fourty-one patients (28 female and 11 male patients, mean age 13,6±3,37 years) with the diagnosis of Nutcracker syndrome were included the study. Asymptomatic Nutcracker syndrome was found in 26 (63.4%) patients; in whom 46.1% were admitted for the evaluation of proteinuria and 42.3% for isolated proteinuria. The most frequent symptoms were left flank pain (33.3%), left flank pain and hematuria together (26.6%). The mean ratio of the diameter of the hilar portion of the left renal vein to that of the aortomesenteric portion was 5,07±1,02. The mean peak velocity ratio of the left renal vein aorta-mesenteric/hiler portion was found to be 7,17±1,86. The peak velocity ratios of the LRV (p = 0.041) were significantly higher in asymptomatic children with Nutcracker syndrome than in the symptomatic group.

Conclusion: The diagnosis of Nutcracker syndrome should be considered in the presence of symptoms such as proteinuria and hematuria and should be absolutely ruled out before attempting renal biopsy. In addition, our study showed that increased compression of the left renal vein is mostly observed in proteinuria and microscopic hematuria.

Anahtar Kelimeler

• Child,Doppler ultrasonography,Nutcracker syndrome

Kaynakça

1. 1. De Schepper, A. “Nutcracker” phenomenon of the renal vein and venous pathology of the left kidney. J Belge Radiol. 1972 Sep-Oct;55(5):507-11.
2. 2. Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc. 2010 Jun;85(6):552-9. doi: 10.4065/mcp.2009.0586. Review. PMID: 20511485.
3. 3. Ananthan K, Onida S, Davies AH. Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines. Eur J Vasc Endovasc Surg. 2017 Jun;53(6):886-94. doi: 10.1016/j.ejvs.2017.02.015. Epub 2017 Mar 27. Review. PMID: 28356209
4. 4. Venkatachalam S, Bumpus K, Kapadia SR, Gray B, Lyden S, Shishehbor MH. The nutcracker syndrome. Ann Vasc Surg. 2011 Nov;25(8):1154-64. doi: 10.1016/j.avsg.2011.01.002. Epub 2011 Mar 25. Review. PMID: 21439772.
5. 5. Shin JI, Lee JS. Nutcracker phenomenon or nutcracker syndrome? Nephrol Dial Transplant. 2005 Sep;20(9):2015. PMID:16091381.
6. 6. 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 Jan;172(1):39-43. DOI: 10.2214/ajr.172.1.9888735.
7. 7. Alaygut D, Bayram M, Soylu A, Cakmakcı H, Türkmen M, Kavukcu S. Clinical course of children with nutcracker syndrome. Urology. 2013 Sep;82(3):686-90. doi: 10.1016/j.urology.2013.03.048. Epub 2013 Jun 2.
8. 8. Del Canto Peruyera P, Vaquero Lorenzo F, Vallina-Victorero Vazquez MJ, Alvarez Salgado A, Vicente Santiago M, Botas Velasco M, et al. Recurrent hematuria caused by nutcracker syndrome. Ann Vasc Surg. 2014 May;28(4):1036.e15-9. doi: 10.1016/j.avsg.2013.05.021. Epub 2013 Dec 7.

9. 9. He Y, Wu Z, Chen S, et al. Nutcracker syndrome--how well do we know it? Urology. 2014 Jan;83(1):12-7. doi: 10.1016/j.urology.2013.08.033. Epub 2013 Oct 18.
10. 10. 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 Jan;21(1):39-45.
11. 11. 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 May;166(5):399-404. DOI:10.1007/s00431-006-0252-7. Epub 2006 Oct 19.
12. 12. Kim SH, Cho SW, Kim HD, Chung JW, Park JH, Han MC. Nutcracker syndrome: Diagnosis with Doppler US. Radiology. 1996 Jan;198(1):93-7.
13. 13. Cheon JE, Kim WS, Kim IO, et al. Nutcracker syndrome in children with gross haematuria: Doppler sonographic evaluation of the left renal vein. Pediatr Radiol. 2006 Jul;36(7):682-6. DOI: 10.1007/s00247-006-0145-y. Epub 2006 Apr 26.
14. 14. Shin JI, Baek SY, Lee JS, Kim MJ. Follow-up and treatment of nutcracker syndrome. Ann Vasc Surg. 2007 May;21(3):402. Epub 2007 Apr 6. No abstract available. PMID: 17419006.
15. 15. Shin JI, Lee JS, Kim MJ. The prevalance, physical characteristics and diagnosis of nutcracker syndrome. Eur J Vasc Endovasc Surg. 2006 Sep;32(3):335-6. DOI: 10.1016/j.ejvs.2006.04.030. Epub 2006 Jun 15.
16. 16. Lopatkin NA, Morozov AV, Lopatkina LN. Essential renal haemorrhages. Eur Urol. 1978;4(2):115-9.
17. 17. Ekim M, Bakkaloglu SA, Tumer N, Sanlidilek U, Salih M. Orthostatic proteinuria as a result of venous compression (nutcracker phenomenon): a hypothesis testable with modern imaging techniques. Nephrol Dial Transplant. 1999 Apr;14(4):826-7.
18. 18. Gulleroglu K, Gulleroglu B, Baskin E. Nutcracker syndrome. World J Nephrol. 2014 Nov 6;3(4):277-81. doi: 10.5527/wjn.v3.i4.277.
19. 19. Noorani A, Walsh SR, Cooper DG, Varty K. Entrapment syndromes. Eur J Vasc Endovasc Surg. 2009 Feb;37(2):213-20. 20. Park SJ, Shin JI. Renal doppler ultrasonography in the diagnosis of nutcracker syndrome. Eur J Pediatr. 2013 Jan;172(1):135-6. doi: 10.1007/s00431-012-1859-5. Epub 2012 Nov 20. No abstract available. PMID: 23179952.
20. 21. Fitöz 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 May;26(5):573-80
21. 22. Hangge PT, Gupta N, Khurana A, et.al. Degree of Left Renal Vein Compression Predicts Nutcracker Syndrome. J Clin Med. 2018 May 8;7(5). pii:E107. doi: 10.3390/jcm7050107.
22. 23. Wang L, Yi L, Yang L, et al. Diagnosis and surgical treatment of nutcracker syndrome: a single-center experience. Urology. 2009 Apr;73(4):871-6. doi: 10.1016/j.urology.2008.11.043. Epub 2009 Feb 4. PMID: 19193424.
23. 24. Shin JI, Park JM, Lee SM, Shin YH, Kim JH, Lee JS, Factors affecting spontaneous resolution of hematuria in childhood nutcracker syndrome. Pediatr Nephrol. 2005 May;20(5):609-13.
24. 25. Cuéllar i Calàbria H, Quiroga Gómez S, Sebastià Cerqueda C, Boyé de la Presa R, Miranda A, Alvarez-Castells A. Nutcracker or left renal vein compression phenomenon: Multidetector computed tomography findings and clinical significance. Eur Radiol. 2005 Aug;15(8):1745-51.
25. 26. Orczyk K, Wysiadecki G, Majos M, Stefańczyk L, Topol M, Polguj M. What each clinical anatomist has to know about left renal vein entrapment syndrome (Nutcracker syndrome) A review of the most important findings. Biomed Res Int. 2017;2017:1746570