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目的:探讨先天性巨输尿管症的诊治特点。方法:9例先天性巨输尿管症,其中左侧4例,右侧2例,双侧3例。主诉症状不典型,最终经B超、KUB+IVP、膀胱镜逆行插管造影、CT、MRU等检查确诊。采用输尿管中、下段裁剪、抗逆流输尿管膀胱再植术5例。1例先行肾盂穿刺造瘘术,3个月后行输尿管膀胱再植术。因肾重度积水,功能严重受损而行肾、输尿管切除术1例。1例行输尿管末端切开术。1例行保守治疗,定期更换双J管。结果:输尿管膀胱再植术6例(包括先行肾盂穿刺造瘘术,3个月后再行输尿管膀胱再植术的患者),均于6～12周后拔除支架管或双J管(幼儿患者约6周拔除支架管导尿管,成人患者约2～3个月拔除双J管)。术后随访经B超及静脉肾盂造影检查,显示患侧输尿管扩张度和肾积水均明显减轻。1例行输尿管末端内切开术的患者在术后3个月拔除并更换双J管1次,复查B超亦提示恢复良好。保守治疗的1例患者到目前为止,病情尚无恶化征象。结论:B超和KUB+IVP检查是诊断先天性巨输尿管症的首选检查方法,但MRU近年体现出更多的诊断优势。治疗本症的原则是解除梗阻,尽量保留肾功能。手术方式以输尿管剪裁或折叠加输尿管膀胱吻合术为主,但腹腔镜和内镜手术也逐渐受到重视。肾功能尚好者也可行扩张或放置内支架等保守治疗。
Objective: To investigate the diagnosis and treatment of congenital giant ureter. Methods: Nine cases of congenital megalitis, including 4 cases on the left, 2 cases on the right and 3 cases on both sides. The main symptoms of atypical, the final by the B-, KUB + IVP, cystoscopy retrograde intubation, CT, MRU and other tests confirmed. In the ureter, the lower cut, anti-reflux ureter bladder replantation in 5 cases. 1 case of first pelvis puncture fistula, 3 months after ureteral bladder replantation. Due to severe hydronephrosis, dysfunction and renal function, ureteral resection in 1 case. A routine ureteral incision. A routine conservative treatment, regular replacement of double J tube. Results: 6 cases of ureteric bladder replantation (including the first peritoneal laparoscopic ostomy and ureteric bladder replantation after 3 months) were both removed stent or double J tube in 6-12 weeks About 6 weeks removal of stent catheterization, adult patients about 2 ~ 3 months to remove double J tube). Postoperative follow-up by ultrasonography and intravenous pyelography, showed ipsilateral ureteral dilatation and hydronephrosis were significantly reduced. A routine endoscopic ureteral endotracheal resection in 3 months after removal and replacement of double J tube 1 times, review of B-also prompted a good recovery. One patient with conservative treatment has so far not shown any signs of worsening. Conclusions: B-ultrasound and KUB + IVP are the first choice for the diagnosis of giant ureteral congenital ureteral diseases. However, MRU has shown more diagnostic advantages in recent years. The principle of treatment of this disease is to lift the obstruction, try to retain renal function. Surgical approach to ureter cut or fold plus ureteral anastomosis based, but laparoscopic and endoscopic surgery has gradually been taken seriously. Good renal function is also feasible to expand or place the stent and other conservative treatment.