Evaluation of Anterior Nutcracker Syndrome in Children: One Center Experience

Yıl 2025, Cilt: 19 Sayı: 2, 87 - 91, 24.03.2025

Saliha Yavuz Eravcı , Tülin Hakan Demirkan , Mihriban İnözü , Begüm Avcı , Özlem Yüksel Aksoy , Zehra Aydın , Umut Selda Bayrakçı , Fatma Şemsa Çaycı , Serdal Kenan Köse

Öz

Objective: Nutcracker syndrome (NCS), caused by the compression of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the abdominal aorta, is a rare clinical condition. While renal Doppler ultrasonography has been used to diagnose NCS, there have been few studies linking LRV compression measurements to symptoms in pediatric patients. This long-term retrospective study aimed to evaluate the clinical characteristics of NCS and its Doppler ultrasonographic measurements in children.

Material and Methods: Demographic, clinic, and laboratory data were collected from the medical records of patients with NCS. All renal Doppler ultrasonography (DUS) findings of the study population were also reviewed.

Results: A total of 50 patients with NCS were identified with a mean follow-up of 6.3 years. The proportion of proteinuria was 70%, and 36% of the patients exhibited symptoms of hematuria. Varicocele was also observed in 55.5% of male patients. While patients with hematuria were diagnosed earlier, patients with varicocele and proteinuria were diagnosed later in life. The mean SMA angle of patients in the supine position was significantly lower patients with varicocele (23.80±3.04° vs 27.87±4.49°, p=0.049). The estimated glomerular filtration rate (eGFR) did not differ in patients with hematuria and in patients with variocele. On the other hand, patients with proteinuria had a lower eGFR than those without (129.85±18.48 ml/min/1.73 m² vs 141.82±20.72 ml/min/1.73 m², p=0.030). The Doppler ultrasonographic parameters of the LRV did not change according to the gender, but the SMA angle decreases significantly with aging.

Conclusion: Although hematuria seems to be common, proteinuria is also common in NCS and SMA angle should thought to be important in children with varicocele.

Anahtar Kelimeler

Doppler ultrasonography, Hematuria, Nutcracker syndrome, Proteinuria, Varicocele

Kaynakça

• Cakici E, Yazılıtaş F, Cinar HG, Can G, Kurt Sukur EG, Gungor T et al. Nutcracker Syndrome in Children: the Role of Doppler Ultrasonography in Symptomatic Patients. Turkish J Pediatr Dis 2019; 13:348-52.
• Gulleroglu K, Gulleroglu B, Baskin E. Nutcracker syndrome. World J Nephrol 2014;3:277-81.
• Orczyk K, Wysiadecki G, Majos A, 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 2017:1746570.
• 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.
• Taktak A, Hakan Demirkan T, Acar B, Gu RG, Köksoy A, Uncu N, et al. Clinico-radiological correlation of nutcracker syndrome: a single centre experience. Arch Argent Pediatr 2017;115:165-8.
• Kolber MK, Cui Z, Chen CK, Habibollahi P, Kalva SP. Nutcracker syndrome: diagnosis and theraphy. Cardiovasc Diagn Ther 2021;11:1140-9.
• Nalcacioglu H, Ceyhan Bilgici M, Tekcan D, Genc G, Bostanci Y, Yakupoglu YK et al. Nutcracker Syndrome in Children: Role of Doppler Ultrasonographic Indices in Detecting the Pattern of Symptoms. J Clin Med 2018; 7:214.
• Meyer J, Rother U, Stehr M, Meyer A. Nutcracker syndrome in children: Apperance, diagnostics, and treatment- A systematic review. J Pediatr Surg 2022;57:716-72.
• 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.
• 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.
• Kim TM, Cho JY, Kim SY, Kim SH. Diagnostic accuracy of the jetting sign and a dilatation ratio of left renal vein in CT urography for detecting anterior nutcracker syndrome. Clin Radiol 2021;76:510-8.
• Kim SH. Doppler US and CT Diagnosis of Nutcracker Syndrome. Korean J Radiol 2019;20:1627-37.
• 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.
• 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.
• Basaran ES, Yilmaz AÇ, Gungor Ö, Tayfur AÇ, Büyükkaragoz B. Clinical Profile and Renal Ultrasound Characteristics of Children With Nutcracker Syndrome in Turkey. Indian Pediatr 2022;59:28-30.
• Velásquez-Jones L, Medeiros M, Patińo Ortega M, Guerrero Kanan R, Valadez-Reyes MT, Valverde-Rosas S et al. Nutcracker syndrome: Cause of non-glomerular hematuria and massive proteinuria. Bol Med Hosp Infant Mex 2014;71:298-302.
• Dunphy L, Penna M, Tam E, El-Kafsi J. Left renal vein entrapment syndrome: nutcracker syndrome! BMJ Case Rep 2019;12:e230877.
• Granata A, Distefano G, Sturiale A, Figuera M, Foti PV, Palmucci S, Basile A. From Nutcracker Phenomenon to Nutcracker Syndrome: A Pictorial Rewiev. Diagnostics (Basel) 2021;11:101.
• Orczyk K, Łabetowicz P, Lodziński S, Stefańczyk L, Topol M, Polguj M. The nutcracker syndrome. Morphology and clinical aspects of the important vascular variations: a systematic study of 112 cases. Int Angiol 2016;35:71-7.
• 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.
• Akdemir I, Mekik Akar E, Yılmaz S, Çakar N, Fitöz S, Özçakar ZB. Nutcracker syndrome in pediatrics: initial findings and long-term follow up results. Pediatr Nephrol 2024;39:799-806.
• Lin L, Zhang K, Yang X, Lin L, Li X, Qiu L. Orthostatic proteinuria due to inferior vena cava interruption without nutcracker phenomenon in an old obese female: a case report and literature review. BMC Nephrol 2023;24:225.
• Ozçakar ZB, Yalçınkaya F, Fitöz S, Cipe G, Soygür T, Ozdemir H et al. Nutcracker syndrome manifesting with severe proteinuria: a challenging scenario in a single-kidney patient. Pediatr Nephrol 2011;26:987-90.
• 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;65:280-3.
• Altugan FS, Ekim M, Fitöz S, Ozçakar ZB, Burgu B, Yalçınkaya F et al. Nutcracker syndrome with urolithiasis. J Pediatr Urol 2010;6:519-21.
• Ismailoglu T. The Nutcracker Syndrome. J Radiol Case Rep 2022;16:17-23.
• 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.
• Reddy DK, Shekar P A. Nutcracker Syndrome-A Rare but Important Cause of Varicocele in Adolescent Boys. Urology 2020;141:143-6.
• Hangge PT, Gupta N, Khurana A, Quencer KB, Albadawi H, Alzubaidi SJ et al. Degree of Left Renal Vein Compression Predicts Nutcracker Syndrome. J Clin Med 2018;7:107.