成人先天性巨输尿管症的诊断治疗

目的探讨成人先天性巨输尿管症（CM）的诊断治疗.方法回顾性分析19例成人CM的临床资料.男6例,女13例,平均年龄36岁.左侧11例,右侧5例,双侧3例.B超、IVU、RGP及MRU等检查,输尿管全段扩张伴肾盂积水16例,患肾无功能4例,仅输尿管下段扩张3例,扩张输尿管最大管径3.3 cm,无输尿管狭窄.尿道膀胱造影（MCUG）及尿动力学检查,无神经性膀胱及膀胱输尿管返流.结果 19例患者中行肾输尿管切除2例,肾造瘘2例,1例1年后行输尿管膀胱再植术,术后5年无异常,另1例4个月后行肾输尿管切除.保守观察3例,随访11～19年,病变无进行性加重.间断性双J管置入3例,随访5～9年,肾功能正常.输尿管裁剪修整,膀胱再植9例,6例随访3～17年无异常;吻合口狭窄及膀胱输尿管返流各1例,再次术后随访6年无异常;1例术后3年行患肾及输尿管切除.结论成人CM诊断主要依据影像学和尿动力学检查,治疗原则为及早手术、去除病因、解除梗阻、保留和恢复肾功能,输尿管裁剪修整、膀胱再植术为有效手术方法.

Diagnosis and treatment of congenital megaureter in adults

Objective To investigate the diagnosis and treatment of the congenital megaureter(CM) in adults.Methods Retrospective analysis was carried out in 19 adult patients of CM(6 men and 13 women;mean age,36 years).Of the 19 cases,CM was found on the left side in 11 cases,on the right in 5 cases,and bilaterally in 3 cases.Ultrasonography,IVU,RGP and MRU revealed whole ureter distension and pelvis hydrops in 16 cases,involved kidney without function in 4 cases,lower ureter distension in 3 cases.The maximum caliber of the distended ureter was 3.3 cm and no ureter stricture was found.Urodynamics and MCUG showed no neurogenic bladder and vesicoureteral reflux.Results Of the 19 cases,2 cases underwent nephrectomy and total ureterectomy.Nephropyelostomy was performed in 2 cases,of whom,1 case underwent ureterovesical reimplantation 1 year after operation and maintained normal renal function during the 5 years' follow-up,the other underwent nephrectomy and total ureterectomy 4 months after nephropyelostomy.Three cases received regular check-up for 11-19 years and kept no obvious change in their renal condition.The double-J catheter stent was placed intermittently in 3 cases during 5 to 9 years' follow-up,and the kidney function was normal.Nine cases were treated by ureter tailoring and trimming,and bladder reimplantation;among them,6 cases were normal with their renal function during 3-17 years' follow-up;1 case underwent nephrectomy and total ureterectomy 3 years after ureter reimplantation;the other 2 suffered from stoma stricture and vesicoureteral reflux,respectively and got normal during the 6 years' follow-up after re-operation.Conclusions Imaging examinations of IVU,MRU,MCUG,and urodynamics are the optimal methods for the diagnosis of CM in adults.The therapeutic strategy for CM in adults is early surgical treatment of relieving obstruction and conserving renal function,which can be achieved by ureter tailoring and bladder reimplantation.