Zigman A, Yazbeck S, Emil S, Nguyen L
Département de Chirurgie, Hĵpital Sainte-Justine, and the Montreal Children's Hospital, Montreal, Quebec, Canada.
J Pediatr Surg. 2000 Nov;35(11):1540-2. doi: 10.1053/jpsu.2000.18302.
Renal vein thrombosis (RVT) is a rare cause for pediatric surgical consultation. The purpose of this study is to review the Montreal experience in the 1990s with RVT.
A retrospective chart review was conducted from 1990 through 1999.
Twenty-three cases were identified by Duplex ultrasound scan. Mean length of follow-up was 42 months. Eighty-three percent (83%) of cases were diagnosed within the first month of life. In utero thrombosis was suspected in 22% and was associated with caval thrombosis and factor V Leiden. Known risk factors were present in 87%. The "diagnostic triad" of flank mass, gross hematuria, and thrombocytopenia was present in only 13% at the time of diagnosis. Long-term renal function impairment was detected in 100% of those who did not receive heparin, and in 33% of those who did receive heparin. No patient required dialysis. One patient required nephrectomy for recurrent pyelonephritis.
RVT occurs more commonly than anticipated. Because the "classic" triad of signs usually is absent at presentation, the presence of either a flank mass, hematuria, or thrombocytopenia in a patient with risk factors should prompt investigation for RVT. Factor V Leiden is a risk factor for in utero RVT. Anticoagulation improves renal outcome. Patients with RVT require long-term follow-up.
肾静脉血栓形成(RVT)是小儿外科会诊的罕见病因。本研究的目的是回顾20世纪90年代蒙特利尔地区RVT的诊治经验。
对1990年至1999年的病历进行回顾性分析。
通过双功超声扫描确诊23例。平均随访时间为42个月。83%的病例在出生后第一个月内确诊。22%的病例怀疑存在宫内血栓形成,且与腔静脉血栓形成和凝血因子V莱顿突变有关。87%的病例存在已知危险因素。诊断时仅有13%的病例出现胁腹部肿块、肉眼血尿和血小板减少的“诊断三联征”。未接受肝素治疗的患者中100%出现长期肾功能损害,接受肝素治疗的患者中33%出现长期肾功能损害。无一例患者需要透析治疗。1例患者因复发性肾盂肾炎需要行肾切除术。
RVT的发生率比预期的更高。由于就诊时通常不存在“经典”体征三联征,因此对于有危险因素的患者,若出现胁腹部肿块、血尿或血小板减少,应警惕RVT并进行检查。凝血因子V莱顿突变是宫内RVT的危险因素。抗凝治疗可改善肾脏预后。RVT患者需要长期随访。