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.     

成人先天性巨输尿管症（附15例报告）

报告１５例共１９侧成人先天性巨输尿管，其中左侧９例，右侧２例，双侧４例。９例有腰痛，合并血尿５例；有尿路感染症状５例。手术治疗１１例１４例，其中输尿管成形再植１２例，肾、输尿管切除２例。输尿管梗阻段经组织病理检查１２侧有胶原纤维组织增生，纵肌减少，环肌肥厚或慢性炎症等改变。     

先天性巨输尿管症的超声显像诊断

报道资料完整和经手术与病理证实的１３例１５侧先天性巨输尿管症的超声显像诊断结果，符合率为９２．３％（１２／１３）。巨输尿管症主要声像图表现管腔扩张内径为１．８～４．２ｃｍ，平均３．１ｃｍ，患侧肾多伴有轻度或中度积水。输尿管中下段局部显著扩张者７例，全程呈柱状扩张者６例。扩张输尿管可有迂曲，巨输尿管近狭窄段可呈杵指状、梭形或鼠尾状扩张。实时观察可见输尿管蠕动频率低，蠕动波幅增大，蠕动波向下传递间断或波幅逐渐减小等声像图表现。超声显像诊断本病时，主要应与输尿管机械性梗阻鉴别。     

Operative treatment of megaureter in adults

After a general outline of ideas about surgery of the megaureter, the author presents one-year postoperative results for 3 adult patients with megaureter, operated on by Prof. Hardy Hendren during his visit to Warsaw. The tapering of the ureters and reimplantation were done in all 3 cases. Though the presented material is too small to draw a definite conclusion, the results obtained in adults by a world-renowned specialist of surgery of megaureter in children can be helpful in the choice of a line of conduct in this diffucult urological problem.     

Direct nipple ureteroneocystostomy in adults with primary obstructed megaureter

PURPOSE: We evaluated the results of direct nipple ureteroneocystostomy technique in adults with primary obstructed megaureter. MATERIALS AND METHODS: Five patients with a mean age of 32.5 years had a total of 6 primary obstructed megaureters with complaints of flank pain. Two patients were female and 3 were male. In patient 1 disease was bilateral, in 3 it was on the right side and in 1 it was on the left side. In male patient 1 a thick walled right ureter could be directly inserted into the bladder without eversion or tapering. The left nipple was created with spatulation. In 2 cases the ureters were thin walled (2 mm or less) and the nipples were created without spatulation. In the other 2 cases the nipples were created following spatulation since the ureteral walls were thicker (2 to 3 mm). RESULTS: Mean followup was 36 months. Flank pain complaints resolved in the postoperative period. Early postoperative Whitaker tests revealed nonobstructed renal units. At later followup visits excretory urograms and/or ultrasound showed partially resolved hydroureteronephrosis. CONCLUSIONS: Ease of application, a high success rate and no need to taper or plicate the ureter, or prepare a submucosal tunnel might be the reasons to consider the direct nipple technique for megaureters of different etiologies.     

成人先天性巨输尿管症的临床治疗

目的 探讨成人先天性巨输尿管症的临床治疗方法,评估开放手术与腹腔镜手术的临床治疗效果.方法 成人先天性巨输尿管症24例,13例行开放输尿管中下段裁剪输尿管膀胱再植术,11例行腹腔镜输尿管成形术.结果 开放手术时间2.0～3.0 h,出血60～120ml.住院时间8～12天.腹腔镜手术时间2.0～4.0 h,出血20～40ml.住院时间5～8天.两组均于术后3个月拔除双J管,无腰痛和发热.随访3个月～5年输尿管均无梗阻.结论 腹腔镜输尿管成形术出血少,损伤小,住院时间短,效果好.     

The congenital refluxing megaureter: experiences with surgical reconstruction.

A series of 17 children with congenital refluxing megaureters underwent ureteric tailoring and reimplantation. The results obtained suggest that such surgery should be restricted to those cases in which an obstructive element is introduced as a result of the reflux or in which ascending infection is further damaging the already prenatally defective kidney parenchyma.     

小儿腹腔镜下巨输尿管成形术

目的 探讨小儿腹腔镜下输尿管铲状乳头膀胱再植术的可行性和临床效果.方法采用经膀胱外途径行腹腔镜下输尿管铲状乳头膀胱再植术治疗先天性梗阻性巨输尿管症患儿11例.年龄11个月～13岁,平均(5.3±3.9)岁.左侧4例,右侧7例.其中输尿管出口闭锁1例、单纯性输尿管出口狭窄9例、开放输尿管膀胱再植术后(Cohen手术)输尿管出口狭窄1例.B超和IVU示重度肾积水7例、中度肾积水4例. 结果 11例手术均获成功.手术时间70～190 min,平均(103.O±35.3)min.术中出血10～40 ml,平均(18.0±9.5)ml.术后住院时间7～10 d,平均(8.0±1.4)d.无尿漏发生.术后6周拔除双J管,膀胱镜或输尿管镜下见膀胱输尿管吻合口已黏膜化,乳头收缩抗反流效果满意;11例平均随访6(3～24)个月,B超复查患侧肾积水减轻;IVU示成形输尿管排尿好,无梗阻,症状基本消失;膀胱造影未见膀胱输尿管反流. 结论 在熟练掌握腹腔镜操作技术后,应用经膀胱外途径腹腔镜下输尿管铲状乳头膀胱再植术治疗小儿梗阻性巨输尿管症创伤小、抗反流效果好,是治疗小儿梗阻性巨输尿管症的微创新途径.     

Endoureterotomy for congenital primary obstructive megaureter: preliminary report

BACKGROUND AND PURPOSE: Congenital obstructive megaureter (COMU) may be unilateral or bilateral and may present in later years of life. If the obstruction is not relieved in good time, deleterious effects on the proximal ureter and kidney are well known. Stones may complicate the situation further. So far, the only treatment that has been available is to disconnect the ureter proximal to the site of obstruction, remove any stone, and reimplant the ureter into the bladder after any necessary tailoring. After noticing the encouraging results of endopyelotomy for congenital ureteropelvic junction obstruction, we decided to use the same principle for the management of COMU, as similar functional pathology is present in both situations. PATIENTS AND METHODS: Our technique was applied in six ureters in five adult patients. After cystoscopic evaluation of the bladder and ureteral orifice, a guidewire was advanced up the ureter, and the lower ureter was dilated. A ureteroscope or other suitable endoscopic instrument was passed, and the obstructed segment of the ureter was incised at the 6 o'clock position with pure cutting current. All of the layers of the ureter were incised in the long axis through the entire obstructive segment, so as to expose the periureteral areolar tissue. If necessary, a similar cut was made at the 12 o'clock position. Utmost care was taken not to incise the bladder mucosa. A double-J stent was inserted for 3 weeks. RESULTS: Follow-up for 1 to 4 years showed free drainage of urine into the bladder, with marked reduction in proximal stasis and freedom from recurrent infection and pain. CONCLUSION: Although our series is small and follow- up is relatively short, it appears that endoureterotomy is a safe and effective treatment for COMU.     

The association of congenital megaureter and ureteropelvic junction obstruction

Megaureter and ureteropelvic junction obstruction are not uncommon urinary tract congenital abnormalities. While each has been known to be associated with other urogenital anomalies, their association together is rarely reported. This report will describe the diagnosis and management of such a case.     

成人先天性巨输尿管症30例报告

目的 :探讨成人先天性巨输尿管症的诊治方法。方法 :报告 30例成人先天性巨输尿管症 ,其中左侧 18例 ,右侧 7例 ,双侧 5例。 30例中行肾输尿管切除术 2例 ;行输尿管膀胱吻合术 2 8例 ,其中先行肾造瘘术 ,二期行输尿管膀胱吻合术 5例。结果 :2 8例输尿管膀胱吻合术中 ,成功 2 6例 ,成功率 92 .9%。结论 :对静脉肾盂造影不显影的患肾应行同位素肾动态显像 ,以决定手术方案。治疗本症的原则是解除梗阻 ,尽量保留肾功能。理想的手术方式是输尿管裁剪加输尿管膀胱吻合术     

成人先天性巨输尿管症(附35例报告)

目的：探讨成人先天性巨输尿管症的诊治特点，方法：35例成人先天性巨输尿管症，其中左侧14,全然中侧11例，双侧10例，所有病例均行B超，IVU检查，手术治疗31例39侧，其中肾输尿管切除术12例，输尿管剪裁整形或折叠后行输尿管膀胱再植术27例，结果：29例获得随访，随访时间1－5年，术侧肾输尿管积水37侧减轻，2侧无明显变化。结论：B超，IVU检查是成人先天性巨输尿管症的首先检查方法。治疗本症的原则是解除梗阻，尽量保留肾功能，手术方式是输尿管剪裁或折叠加输尿管膀胱吻合术，肾功能良好者也可保守治疗。     

成人先天性巨输尿管症(附12例报告)

目的：探讨成人先天性巨输尿管症的病因、病理、诊断及治疗。方法：对12例成人先天性巨输尿管症患者的临床资料进行分析总结。结果：12例14侧成人先天性巨输尿管，其中佐侧8例，右侧2例，双侧2例。9例行输尿管裁剪成形和再植术；1例行肾输尿管切除术；2例保守治疗，定期复查随访。取得较好效果。结论：引起成人先天性巨输尿管症原因少数是单一的，多数常常与几种因素有关。可根据不同的情况选择不同的治疗方法。