重肾双输尿管合并下肾肾盂输尿管连接部梗阻性肾积水的诊疗分析

目的 探讨小儿重肾双输尿管合并下肾肾盂输尿管连接部梗阻性肾积水的临床特点及诊疗方法.方法 回顾性分析本院近10年来收治的190例重肾双输尿管畸形病例中,6例合并下肾肾盂输尿管连接部梗阻性肾积水患儿的临床资料,包括年龄、性别、临床症状、解剖结构及处理方法等.结果 重肾双输尿管畸形患儿190例,合并下肾肾盂输尿管连接部梗阻性肾积水6例,占3.16%.其中男5例,女1例,年龄10 d至3岁7个月,平均13.8个月.围产期行B超检查发现肾积水4例,泌尿系感染1例,腹部包块1例.病变位于左侧3例,右侧3例;3例为重肾完全型双输尿管,3例为重肾Y型输尿管,其中1例为右重肾Y型输尿管合并下肾肾盂输尿管连接部狭窄及下肾输尿管膀胱连接部狭窄.3例重肾完全型双输尿管病例中,1例行上组肾及输尿管切除＋下组肾离断性肾盂成形术,2例行下组肾离断性肾盂成形术;3例重肾Y型输尿管中,2例行上肾输尿管下肾盂端侧吻合＋下组肾离断性肾盂成形术,1例行上组肾及输尿管切除＋下组肾离断性肾盂成形术＋输尿管膀胱再植术.术后随访3~18个月,平均12个月,B超及IVP显示肾积水明显好转,无并发症.结论 重肾双输尿管合并下肾肾盂输尿管连接部梗阻性肾积水发病率低,易误诊.术前B超、IVP及MRU是有效的辅助检查手段.临床应根据患儿肾功能及解剖异常情况制定个体化的手术方案.

Diagnosis and Treatment of Lower Pole Pelvic-ureteric Junction Obstruction in Duplicated Systems

Objetive To explore the management of the pelvic-ureteric junction obstruction （PUJO） of the lower pole in duplicated systems. Methods The clinical datum of 6 patients with PUJO of the lower pole in duplicated systems in 190 patients in recent decade were retrospectively analysed, reviewing each case for age, sex, presenting symptoms, anatomy and type of management and so on. Results The incidence of the PUJO of lower pole in duplicated systems was 3.16% （ male 5 and female 1, age ranged from 10 days to 3 years and 7 months old with mean age of 13. g months）. In four of the patients the presentation was prenatal hy- dronephrosis, one presented with urinary infection and one with abdominal mass. Duplicated kidneys located at the left side in 3 patients and other three at the right side. Three presented completely duplicated systems and other three had ＂Y＂ type with mid-ureteric confluence in who one had stricture at ureterovesieal junction in ＂Y＂ type. In 3 cases with completely duplicated systems, 1 patient underwent with resection of the upper renal moiety and lower pyeloureterostomy and 2 with lower moiety pyeloureterostomy. In 3 patients with incompletely duplicated systems, 2 cases underwent with lower pelvis-upper ureterostomy and lower pyeloureterostomy, and resection of the upper renal moiety and lower moiety pyeloureterostomy and reterovesieal reinplantation was per- formed in the child who had stricture at ureterovesieal junction in ＂Y＂ type. During the follow-up within 3 to 18 months （mean 12 months） all of the patients improved without complication on B-ultrasonography,intravenous pyelography （IVP）. Conclusions The incidence of lower pole PUJO in duplicated systems is very low and the ratio of diagnostic errors is high. B-ultrasonography ,IVP and magnetic resonance urography （MRU） are helpful for diagnosis. The operative methods should be individualized and decided by the lesions of renal function and the anatomy.