成人先天性巨输尿管症20例诊治分析

Diagnosis and treatment of congenital megaureter in adults

目的探讨成人先天性巨输尿管症(CM)的诊断和治疗方法。方法回顾性分析20例成人CM的临床资料,其中左侧13例,右侧5例,双侧2例。主要临床表现有间歇性腰痛及肉眼血尿,反复尿频、尿急、尿痛,发热及腹部肿块等。实验室检查尿常规异常及尿培养阳性等。B超均显示患侧输尿管扩张和肾积水。静脉尿路造影(IVU)提示肾积水13例,肾脏不显影5例。磁共振泌尿系成像示肾积水5例。手术治疗19例,其中Ⅰ期行输尿管裁剪整形加输尿管膀胱再植术14例;行肾输尿管切除术2例;肾造瘘3例,其中1例1个月后行肾输尿管切除术,2例3个月后行输尿管裁剪修整加输尿管膀胱再植术。间断性双J管置入1例。术后随访3个月至5年。结果行输尿管下段裁剪整形加输尿管膀胱再植术治疗的16例,患侧肾积水均减轻,肾功能好转或正常;先后期行肾输尿管切除术的3例,术后对侧肾代偿良好;置入双J管的1例,肾积水减轻。结论 B超、IVU等检查是成人CM的主要诊断方法。治疗原则为及早手术以去除病因、解除梗阻、保留和恢复肾功能。输尿管裁剪修整加输尿管膀胱再植术是治疗成人CM的首选手术方式。

Objective To investigate the methods of diagnosis and treatment for adult congenital megaureter（CM）.Methods Retrospective analysis was carried out in 20 adult patients with CM.Of 20 cases,13 cases were in the left side,5 cases in the right side,and 2 cases in bilateral.The common clinical manifestations of CM included intermittent lumbago and gross hematuria,recurrent frequent micturition,urgent micturition,disuria,fever and abdominal mass,etc.Laboratory positive finds included abnormal routine uronoscopy,positive urine culture,etc.Ultrasonography revealed ureter distension and hydronephrosis in sick side.Intravenous urography（IVU） revealed moderate to severe hydronephrosis in 13 cases and kidney with no image in 5 cases.Magnetic resomance urography revealed hydronephrosis in 5 cases.Nineteen cases were treated by surgical treatment,and 1 case was placed double-J stent intermittently.Of 19 cases treated by surgical treatment,14 cases were treated with ureter tailoring and ureterovesical reimplantation by one-stage;2 cases underwent nephrectomy and total ureterectomy;3 cases underwent nephropyelostomy,among whom 1 case underwent nephrectomy and total ureterectomy after 1 month,and 2 cases underwent ureter tailoring and ureterovesical reimplantation after 3 months.All patients treated by operation were followed up for 3 months to 5 years.Results The hydronephrosis of the operated side reduced,and the kidney function was recovered in 16 cases who underwent ureterovesical reimplantation.The contralateral renal function compensated well in 3 cases who underwent nephrectomy and total ureterectomy.The hydronephrosis reduced in 1 case who placed double-J stent.Conclusions Ultrasonography and IVU are the main methods for the diagnosis of adult CM.The principle of treatment is that early operation should be performed for eliminating the cause of disease,relieving obstruction,recovering and conserving renal function.The ureter tailoring and ureterovesical reimplantation is the first choice of operative pattern for treating adult CM.