Ubetagoyena Arrieta M, Areses Trapote R, Arruebarrena Lizarraga D
Sección de Nefrología Pediátrica, Hospital Universitario Donostia, San Sebastián, España.
An Pediatr (Barc). 2011 Nov;75(5):329-33. doi: 10.1016/j.anpedi.2011.05.011. Epub 2011 Jul 2.
To analyse kidney position and fusion anomalies, as well as associated urinary tract and extra-renal pathologies in a cohort from a tertiary hospital.
The medical records of 84 children with renal ectopia (RE) and horseshoe kidney (HK) in our hospital from 1999 to 2010 were retrospectively reviewed.
Prenatal diagnosis was made in 53 patients (63.09%). Of the 84 patients surveyed 35 had unilateral simple renal ectopia (SRE), 1 had bilateral SRE; 2 had crossed renal ectopia (CRE) without fusion, 17 had CRE with fusion, and 29 had HK. Average age was 30 days at diagnosis (IQR: 0-12 months). In the individuals with renal ectopia (RE), the incidence of associated urinary tract pathologies was 20.23% in the ectopic kidney (EK) (17 out of 84) and 17.85% in the contralateral kidney (CK) (15 out of 84). The most frequent urological pathology was vesico-ureteral reflux (VUR), 4 individuals had multicystic dysplastic kidneys (MCDK). In 4 patients renal agenesis was detected in the CK. In patients with HK, the incidence of associated urinary tract pathologies was 16.66% (14 out of 84), and the most frequent associated urological anomaly was also VUR. Of the 84 patients 21(25.45%) had extra-renal pathology. Anomalies of the skeletal system were detected in 9/84(10.71%), genital anomalies 5/84 (5.95%), cardiac anomalies 3/84 (3.57%), and digestive disease 1/84 (1.19%). Facial malformations were noted in 3/84 patients (3.57%), endocrine disorders 2/84 (2.38%), and 8/84 children (9.52%) suffered from polymalformative syndrome. During follow-up, the most frequent complication of the urinary tract was infection, observed in 10/84 patients (11.91%).
When renal ectopia or horseshoe kidney is detected, associated renal and urinary anomalies and structural extra-renal malformations should be evaluated. Patients need long-term follow-up and should be examined regularly for potential complications.
分析一家三级医院队列中肾脏位置及融合异常情况,以及相关的泌尿道和肾外病变。
回顾性分析我院1999年至2010年收治的84例肾异位(RE)和马蹄肾(HK)患儿的病历。
53例(63.09%)患儿为产前诊断。在84例受调查患儿中,35例为单侧单纯肾异位(SRE),1例为双侧SRE;2例为无融合交叉肾异位(CRE),17例为有融合交叉肾异位,29例为马蹄肾。诊断时平均年龄为30天(四分位间距:0 - 12个月)。在肾异位(RE)患儿中,异位肾(EK)相关泌尿道病变发生率为20.23%(84例中有17例),对侧肾(CK)相关泌尿道病变发生率为17.85%(84例中有15例)。最常见的泌尿系统病变是膀胱输尿管反流(VUR),4例有多囊肾发育不良(MCDK)。4例患儿对侧肾存在肾缺如。马蹄肾(HK)患儿中,相关泌尿道病变发生率为16.66%(84例中有14例),最常见的相关泌尿系统异常也是VUR。84例患儿中有21例(25.45%)存在肾外病变。骨骼系统异常在9/84(10.71%)患儿中被检测到,生殖器异常5/84(5.95%),心脏异常3/84(3.57%),消化系统疾病1/84(1.19%)。3/84例患儿(3.57%)存在面部畸形,内分泌紊乱2/84(2.38%),8/84例患儿(9.52%)患有多发畸形综合征。随访期间,泌尿系统最常见的并发症是感染,10/84例患儿(11.91%)出现该情况。
当检测到肾异位或马蹄肾时,应评估相关的肾脏和泌尿道异常以及肾外结构畸形。患儿需要长期随访,并应定期检查是否存在潜在并发症。