52例先天性巨输尿管症诊疗体会

Diagnosis and Treatment of Congenital Megaureter of 52 Cases

目的：探讨先天性巨输尿管症（CM）的诊断、治疗及病理特点。方法：回顾性分析52例CM患者的临床资料,其中22例为左侧,19例为右侧,11例为双侧。所有患者均通过超声等影像学检查而确诊。保守治疗2例2侧,经皮肾穿刺造瘘术2例2侧;手术治疗48例59侧,其中输尿管裁剪输尿管膀胱吻合术41例52侧,肾输尿管切除术3例3侧,输尿管D-J管置入术4例4侧。结果：57侧（48例）术后病理为输尿管黏膜上皮及平滑肌组织增生伴炎细胞浸润,部分伴有黏膜上皮脱落（2侧）,肌间神经丛减少（6侧）,还可见上皮结构排列紊乱。在随访完全（12-65个月）的45例患者中,54侧（43例）肾输尿管积水不同程度缓解。结论：通过超声、静脉肾盂造影等影像学检查有助于明确诊断先天性巨输尿管症,治疗原则为早期诊断、早期去除梗阻、最大限度保护肾功能,而输尿管裁剪、输尿管膀胱再植术、肾穿刺造瘘术等方式是治疗的合理选择。

Objective：To investigate the diagnosisn,treatment and pathological features of congenital megaureter.Method：The clinical data of 52 cases of congenital megaureter were retrospectively analyzed,22 cases were left,19 cases were right,11 cases were bilateral.All patients were checked by ultrasound and IVU examination to make a clear diagnosis.59 sides of 48 cases were for the surgical treatment,in which anastomosis of ureter and bladder ureter cut 52 sides of 41 cases,3 sides of 3 cases were adjusted to kidney and ureteral resection,4 cases of 4 sides were placed double-J.Result：57 side（48 cases） showed ureteral epithelial and smooth muscle hyperplasia accompanied with inflammation.45 patients were followed up after 12 to 65 months,43 patients with 54 sides were seen to reduce the side ipsilateral kidney and hydroureter.Conclusion：Ultrasound and IVU examination could contribute to the diagnosis of congenital megaureter.Surgical treatment,conservative treatment are appropriate medthod to maximize the protection of renal function.