小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.

Diagnosis and management of congenital unilateral dysplastic kidney

Objective To present the experiences of diagnosis and management of unilateral dysplastic kidney in children. Methods Between 1988 and 2009,83 children including 31 boys and 52 girls aged from 45 days to 11 years old (mean age, 70 months old) underwent nephrectomy for unilateral dysplastic kidney at this center. Among these patients,30 patients complicated with ectopic ureter,15 with ureteropelvic junction obstruction (UPJO), 5 with megaureter, 9 with ureterocele, and 4 with vesico-ureteric reflux (VUR). Sixteen patients also presented abnormalities of the contralateral kidneys including UPJO was found in 6 patients,megaureter in 6,duplicative kidney in 1 and VUR in 3. Ultrasonography,excretory urography and 99mTc-DTPA renal scintigraphy were employed to examine the kidney on all patients. Enhanced computerized tomography (CT) was performed on 35 patients, and delayed enhanced three dimensional CT (3D-CT) on 39 patients. All cases underwent nephrectomy.The surgical indications were determined when patients reached to 1 of the 3 criteria: symptomatic nonfunctional dysplastic kidneys (55 cases), or asymptomatic multicystic dysplastic kidneys without any improvement during follow-up or even increasing in cysts' size (27 cases), or dysplastic kidneys with poor function and hydronephrosis (1 case). Results Ultrasonography located the dysplastic kidneys in 51 cases. IVP failed to visualize the affected kidney in all cases. Forty-four dysplastic kidneys were identified by 99mTc-DTPA renal scintigraphy. Enhanced CT located dysplastic kidney in 28 of 35 cases. Delayed enhanced 3D-CT could locate dysplastic kidney in all 39 cases. The pathology diagnosis of all resected kidneys confirmed the diagnosis of dysplastic kidney, and no malignant tissues were found.Among the 4 children with preoperative hypertension, 2 didn't have any improvement after surgery.Conclusions Delayed enhanced 3D-CT is accurate and specific to visualize the dysplastic kidneys. Ne-phrectomy is recommended to the patients with symptomatic non-functional dysplastic kidneys, or asymptomatic multicystic dysplastic kidneys. The patients' kidney function and hypertension should be closely followed up after surgery.