重复肾畸形患儿82例临床特点及诊治情况

Clinical manifestations and treatment of duplex kidneys in 82 children

目的探讨重复肾畸形的临床特点、治疗方案选择及预后。方法对2008年1月至2015年5月中国医科大学附属盛京医院手术治疗的82例重复肾畸形患儿临床资料进行回顾性分析。手术方法：行保留肾单位手术30例，其中上位肾输尿管膀胱非共鞘移植12例，上下位肾输尿管共鞘移植4例，行尿道镜囊肿电切术12例，下肾肾盂输尿管成形术1例，左上输尿管下肾盂吻合、下肾盂输尿管成形术1例；行切除肾单位手术52例，其中行上位肾及其输尿管切除术43例，上位肾及其输尿管切除、下位肾输尿管膀胱移植术8例，上位肾及其输尿管切除、下位肾输尿管狭窄切除、输尿管端端吻合术1例。结果54例获得随访，随访率65.9%，随访时间2个月～4年，平均随访时间9.53个月。切除肾单位患儿术后临床症状均消失，其中4例发现输尿管末端小囊肿但较术前明显减小。保留肾单位患儿术后临床症状均消失，复查彩超肾积水均明显缓解，行尿道镜囊肿电切术12例患儿中3例出现2度膀胱输尿管返流。结论重复肾主要临床表现包括反复泌尿系统感染，正常排尿间歇尿淋漓，腹痛，发现尿道外口肿物，腹部包块，肾积水进行性加重。如有上述临床表现则需要考虑重复肾畸形的可能。重复肾常合并心脏畸形及隐睾，术前应完善睾丸查体及心脏彩超检查。手术分为保留肾单位手术及切除肾单位手术，手术方案的选择应根据患儿临床表现及辅助检查结果综合分析决定。重复肾畸形总体预后良好，但需进一步随访。

ObjectiveTo investigate the clinical characteristics, treatment options and prognosis of duplex kidneys in children.MethodsData were retrospectively collected from all patients who were hospitalized at Shengjing Hospital Affiliated to China Medical University from January 2008 to May 2015.The surgery to retain moiety nephron was performed in 30 cases： 12 cases underwent non common sheath ureter reimplantation, 4 cases of the patients had common sheath ureter reimplantation, 12 patients underwent transurethral resection of the cyst, 1 case of them had pyeloureterostomy, and the other patients underwent lower-pole pyeloplasty.Removal of renal moiety was performed in 52 cases： 43 patients underwent upper-pole heminephrectomy, 8 patients underwent upper pole heminephrectomy and lower-pole ureter reimplantation, and 1 case of the patients had upper-pole heminephrectomy and lower-pole ureteroplasty.ResultsA total of 54 cases were followed up, the follow-up rate reached 65.9%, the follow-up time lasting 2 months to 4 years, with an average time of 9.53 months.All clinical symptoms disappeared after remove moiety operation.There were small ureteral cysts but compared with the preoperative condition, the symptoms reduced significantly in 4 cases.All clinical symptoms disappeared in patients whose nephron was retained after operation, and hydronephrosis was alleviated.De novo 2 degree of vesicoureteral reflux was found in 3 out of 12 patients who underwent transurethral puncture of the cyst.ConclusionsThe clinical manifestations of duplex kidney have some characteristics which include recurrent urinary tract infection, normal intermittent voiding urine dripping, abdominal pain, urethral orifice mass, abdominal mass and aggravation of hydronephrosis.Duplex kidney is often associated with cardiac malformation and cryptorchidism.The preoperative testicular examination and echocardiography should be performed.The choice of operation depends on the clinical manifestation, auxiliary examination results and comprehensive analysis.The prognosis of duplex kidney is well, but patients need to be further followed up after operation.