Miró Ignacio, Serrano Agustín, Pérez-Ardavín Javier, March José-Antonio, Polo Alba, Conca María-Ángeles, Sangüesa Cinta, Veiga Diana, Carazo Elena, Domínguez Carlos
Hospital Universitari I Politecnic La Fe, Valencia, Spain, Avinguda Fernando Abril Martorell, 106 46026, Valencia (Valencia), Spain.
Hospital Universitari I Politecnic La Fe, Valencia, Spain, Avinguda Fernando Abril Martorell, 106 46026, Valencia (Valencia), Spain.
J Pediatr Urol. 2020 Apr;16(2):218.e1-218.e6. doi: 10.1016/j.jpurol.2019.12.003. Epub 2019 Dec 7.
Nutcracker syndrome (NS) defines an entity generated by the compression of the left renal vein resulting in venous hypertension, which transmitted in a retrograde direction may cause hematuria, proteinuria, and varicocele. The literature concerning exclusively pediatric patients is very rare.
To report the authors' experience with pediatric NS in the last 18 years.
This is a retrospective review of the patients followed up in the authors' center with diagnosis of NS based on clinical and imaging tests (ultrasound, computed tomography/magnetic resonance imaging, and phlebography). The primary outcome was the success of the conservative approach in the study patients.
A total of 21 patients were diagnosed with NS and followed up for a mean period of 52.3 months (37.1-67.5). The mean age at diagnosis was 11.7 years (9.9-13.4). The most frequent symptom of presentation was hematuria in 16 patients (76.2%), being macroscopic in 75% patients and related to physical exercise in 42.9% patients. Other symptoms were left varicocele in 7 patients (33%) and proteinuria in 6 patients (28.6%). Mild to moderate cases received conservative treatment (change of physical activity, postural hygiene), which achieved resolution of symptoms in 16 patients (76.2%). Five cases (23.8%) finally needed a more aggressive approach. Two (9.5%) of them required endovascular procedures (intravascular stent in the renal vein in 1 case and embolization of the spermatic vein in 1 case); in one (4.8%) of the patients, transposition of the left renal vein and kidney autotransplantation were performed, and 2 (9.5%) of the patients with mild cases required surgical correction of the varicocele.
Hematuria, usually macroscopic and related to physical exercise, is the most frequent symptom in pediatric patients with NS. The authors advocate studying the aortomesenteric junction by abdominal ultrasound in patients with varicocele or with intermittent macroscopic hematuria. Diagnosis is based on non-invasive tests; phlebography should be reserved for severe cases that require an interventionist attitude. A long period of conservative treatment is the first approach for pediatric patients with NS.
胡桃夹综合征（NS）是指左肾静脉受压导致静脉高压所引起的一种病症，逆行传播的静脉高压可能会导致血尿、蛋白尿和精索静脉曲张。专门针对儿科患者的文献非常罕见。
报告作者在过去18年中治疗儿科胡桃夹综合征的经验。
这是一项对作者所在中心诊断为胡桃夹综合征的患者进行的回顾性研究，诊断基于临床和影像学检查（超声、计算机断层扫描/磁共振成像和静脉造影）。主要结果是研究患者中保守治疗的成功率。
共有21例患者被诊断为胡桃夹综合征，平均随访52.3个月（37.1 - 67.5个月）。诊断时的平均年龄为11.7岁（9.9 - 13.4岁）。最常见的症状是16例患者（76.2%）出现血尿，其中75%为肉眼血尿，42.9%的患者血尿与体育锻炼有关。其他症状包括7例患者（33%）出现左侧精索静脉曲张，6例患者（28.6%）出现蛋白尿。轻度至中度病例接受保守治疗（改变体育活动、姿势卫生），16例患者（76.2%）症状得到缓解。5例患者（23.8%）最终需要更积极的治疗方法。其中2例（9.5%）需要血管内介入治疗（1例为肾静脉内血管支架置入术，1例为精索静脉栓塞术）；1例患者（4.8%）进行了左肾静脉移位和自体肾移植，2例症状较轻的患者（9.5%）需要进行精索静脉曲张手术矫正。
血尿通常为肉眼血尿且与体育锻炼有关，是儿科胡桃夹综合征患者最常见的症状。作者主张对患有精索静脉曲张或间歇性肉眼血尿的患者通过腹部超声检查主动脉肠系膜交界处。诊断基于非侵入性检查；静脉造影应保留用于需要采取干预措施的严重病例。长期保守治疗是儿科胡桃夹综合征患者的首选治疗方法。
