先天性巨输尿管症误诊两例报告

小儿肾积水常见的原因是肾盂输尿管连接部梗阻(PUJO)和先天性巨输尿管症,后者较少见,容易误诊为PUJO。1996年我院收治两例先天性巨输尿管症,以前误诊为     

肾盂输尿管连接部梗阻的诊治进展

肾盂输尿管连接部梗阻(Pelvic ureter junction Obstruction,PUJO)为原发性肾积水最常见的原因。近年来随着医疗技术水平的提高,对本病的诊断及治疗也有了较大的进展。一、PUJO诊断方法的改进 PUJO可引起肾积水,但肾积水并非一定有梗阻存在,如肾盂、肾盏或输尿管的肌肉及弹力组织张力减低,或长期梗阻导致肌肉组织被纤维组织代替,即使解除梗阻,上尿路扩张亦不能完全恢复正常。     

肾盂输尿管连接处梗阻的手术治疗

目的：探讨肾盂输尿管连接处梗阻（PUJO）的手术方法。方法：本组42例中采用非离断性肾盂成形术17例，其中单纯粘连带切断松解术4例，肾盂裁判加连接处松解术5例，肾盂Y－V成形术8例；离线性肾盂成变化，3例加重；离散性肾盂成形术22例，20例肾积水明显改善（90．9％），2例肾积水改善不明显但未加重。两种手术方法的疗效比较采用χ^2检验，P＜0．05，差异有显著性意义。结论：离散性肾盏盂成形术是治疗PUJO的理想方法。     

肾盂成形术治疗肾盂输尿管连接处梗阻

肾盂输尿管连接处梗阻(Pelvic-ureter,junctionObstruction,PUJO)早期诊断、及时治疗,对保护肾功能至关重要.我院1986年至1991年共收治20例PUJO患者,均行肾盂成形术,全获随访,成功率为100%.现报告如下.1 临床资料20例中,男15例,女5例,年龄6～48岁,平均27岁.腰腹部胀痛15例;纳呆、头晕、乏力4例;肉眼血尿3例;镜下血尿5例;尿路感染7例;肾结石8例;慢性肾功能衰竭2例.双肾积水3例;单侧肾积     

肾盂成形术治疗肾盂输尿管连接部梗阻

肾盂输尿管连接部梗阻（PUJO）是引起肾积水的原因之一。早期诊断并及时手术对保护肾功能有重大意义。本院于1996年7月至2004年1月手术治疗38例。报告如下。     

离断性肾盂成形术治疗小儿先天性肾积水

为探讨小儿先天性肾积水手术治疗方法，对１９８８～１９９５年间１１７例肾积水进行总结。离断性肾盂成形术１１２例、肾盂ＹＶ成形术４例，盂管部纵切横缝１例；离断性肾盂成形术一期治愈９９例（８８．４％），延期拔肾造瘘管１０例（９．０％），手术失败３例（２．６％）。离断性肾盂成形术设计合理，成功率高，优于其它手术方法，应作为小儿先天性肾积水的首选手术方法。     

肾盂输尿管连接部梗阻的诊治进展

0 引言 肾盂输尿管连接处梗阻(PUJO)是临床上常见的肾积水的原因,随着临床科研的深入,近年来对该疾病的认识、诊断及治疗方面均有很大进展,现综述如下。     

高频彩超在小儿先天性肛门直肠畸形诊断中的应用

目的:探讨高频彩超在小儿先天性肛门直肠畸形诊治中的应用价值。方法:应用高频彩超和X线对20例肛门直肠畸形患儿进行检查。结果:肛门闭锁和直肠阴道瘘有较典型的声像图表现,同时可以发现合并症,本组10例合并症,超声发现9例。肛门闭锁直肠盲端和直肠阴道瘘定位准确性,超声为90%(18/20),X线为75%(15/20)。结论:超声对肛门直肠畸形的定位高于X线,对常见合并症如先天性心脏病、泌尿生殖系畸形等易检出,是首选的辅助诊断方法。     

小儿先天性输尿管畸形2例分析

目的：提高小儿先天性输尿管畸形的认识及诊断水平。方法：回顺性分析2例经手术证实的先天性巨输尿管和输尿管囊肿畸形患者的临床资料,并结合文献对其发病机制及诊断进行分析讨论。结果：先天性巨输尿管和输尿管囊肿畸形临床无特异性．偶为体检或出现泌尿系症状时才被发现。诊断往往依靠影像学确诊。结论：小儿先天性输尿管畸形并非少见,主要是临床缺乏特异性;在小儿体检或发现泌尿系症状时,臆及时检查,确诊治疗。     

肾盂输尿管连接部梗阻的外科治疗——肾盂成形术

肾盂输尿管连接部梗阻引起的肾积水发病原因虽不太清楚,但多数认为是先天性的,可由于该处粘膜瓣、皱襞、狭窄、扭曲成角、输尿管高位连接、异位(迷走)血管等引起。多见于小儿。成人较少。国外报导一组非小儿组年龄在12～20岁间占26％,30～50岁间占20％。因此本病可发现于各个时期,应予注意。通过同位素肾图检查显示梗阻曲线,尿路造影、静脉滴注肾盂造影出现肾盏肾盂扩大而肾盂输尿管连接部梗阻以下输尿管正常时就可明确诊断。应除外腹膜后纤维化、结石、肿瘤、肾下垂、先天性畸形、邻近肿瘤压迫等引起的肾积水,其中须与膀胱输尿管返     

Management of ureteropelvic junction obstruction in infants and neonates

From June 1980 to October 1985 we performed 22 ureteropyeloplasties on 20 infants and neonates with ureteropelvic junction obstruction. All patients were males less than 2 years old; 12 were less than 1-month-old. The diagnosis was suspected on the basis of maternal ultrasonography in 10 patients (50%), a palpable abdominal mass was the presenting symptom in 7 (35%), and obstruction was detected during evaluation of congenital heart disease in 3 (15%). This change in mode of presentation is expected to become even more pronounced as the use of fetal ultrasonography increases. All patients in whom the diagnosis was suspected antenatally underwent ultrasonography after birth to confirm the presence of hydronephrosis. All 22 obstructed kidneys were repaired by a dismembered technique under optical magnification. There were no operative mortalities. Of 21 postoperative intravenous pyelograms available, 3 demonstrated marked improvement, 17 showed improvement with residual hydronephrosis, and 1 showed deterioration. The methods used to diagnose, treat and evaluate ureteropelvic junction obstruction in 20 infants and neonates are presented. Given the existing clinical and experimental data we advocate early postnatal surgical correction of ureteropelvic junction obstruction to achieve a maximum recovery of renal function.     

Is antibiotic prophylaxis necessary in infants with obstructive hydronephrosis?

PURPOSE: We investigated the relationship between the level of obstruction of the upper urinary tract and the risk and onset of urinary tract infection in infants with severe obstructive hydronephrosis to determine the need for antibiotic prophylaxis. MATERIALS AND METHODS: A total of 105 patients were prenatally diagnosed with severe hydronephrosis (Society for Fetal Urology grade III or IV) due to upper urinary tract obstruction between 1994 and 2004. Of these patients 75 had ureteropelvic junction obstruction and 30 had lower ureteral obstruction. We retrospectively evaluated the clinical course and incidence of urinary tract infection during the first 12 months postnatally without antibiotic prophylaxis. RESULTS: The incidence of overall urinary tract infection during followup was 36.2% (38 of 105 patients), and it demonstrated a higher trend with lower ureteral obstruction than with ureteropelvic junction obstruction (50% vs 30.7%, p=0.063). Most cases of urinary tract infection (92.8%) occurred before age 6 months, with a mean age at onset of 2.6 months. Of 105 patients 77 (73.3%) underwent corrective surgery at a mean age of 3.8 months. The incidence of urinary tract infection before surgical correction was 33.8% at a mean age of 2.1 months. The incidence of urinary tract infection in surgical cases was significantly higher with lower ureteral obstruction than with ureteropelvic junction obstruction (54.2% vs 24.5%, p=0.011). CONCLUSIONS: Urinary tract infection in infants with severe obstructive hydronephrosis has a high incidence, occurs before age 6 months and is more common with lower ureteral obstruction than with ureteropelvic junction obstruction. These findings indicate that infants with severe hydronephrosis due to obstruction of the upper urinary tract should receive antibiotic prophylaxis.     

三维重建CT在肾盂输尿管连接处狭窄诊治中的应用

目的：评价三维重建CT技术在肾盂输尿管连接处狭窄患者诊治中的应用价值。方法：对22例肾盂输尿管连接处狭窄患者术前行三维CT重建,根据术前CT重建所显示的肾脏血管及尿路情况,判定肾盂输尿管连接处狭窄是否为肾迷走血管所致,最后与实际手术情况相比较。结果：22例患者中12例经CT重建检查发现存在迷走血管压迫肾盂输尿管连接处,10例未见明显迷走血管压迫征象。经手术证实,三维重建CT对肾迷走血管致肾盂输尿管连接处狭窄的诊断符合率达100％。结论：三维重建CT能准确评价肾脏血管的变异和病变情况,对于术前准确合理地预制定肾盂输尿管成型手术方案及术中准确快速地进行血管处理有着重要的意义。     

Clinical outcome and follow-up of prenatal hydronephrosis

Hydronephrosis is probably the most common congenital abnormality detected prenatally by ultrasonography This study was performed to determine the cause and outcome of prenatal hydronephrosis in our hospital. A total of 45 infants, with 57 prenatally hydronephrotic renal units, were enrolled into this study. For the purpose of this study, the degree of hydronephrosis was defined as mild, moderate or severe. Postnatal ultrasonography was performed as soon as possible in those with bilateral hyronephrosis and 3-7 days after birth in those with unilateral hydronephrosis. Voiding cystourethrogram was performed in 6-8 weeks time. In the absence of vesicoureteral reflux (VUR), diethylenetriamene penta acetate scan was performed to exclude obstructive uropathy. There were 29 males and 16 females (male:female ratio 1.8:1), and unilateral and bilateral hydronephrosis were seen in 33 (73%) and 12 (27%) of the cases, respectively. Hydronephrosis was caused by ureteropelvic junction obstruction (UPJO) in 20 (44.5%), VUR in 10 (22.2%), ureterovesical junction obstruction in four (8.9 %), posteriorurethral valves in four (8.9 %), UPJO with VUR in two (4.4%) and non-VUR non-obstructive in one (2.2%). During follow-up, 16 patients (35.5%) required operative intervention while seven (15.5%) improved spontaneously. Fetal hydronephrosis needs close follow-up during both ante-natal and postnatal periods. In this study, the most common cause for hydronephrosis were UPJO and VUR. Also seen in this study is the noteworthy point that mild fetal hydronephrosis is relatively benign and does not require surgical intervention in most cases and surgery should be performed only if there is renal function compromise. Prenatal consultation with a pediatric nephrologist and urologist is useful in decreasing parental anxiety and facilitating postnatal management.     

肾穿刺造瘘引流肾盂输尿管成形术治疗小儿先天性肾盂输尿管连接部梗阻

目的:探讨提高治疗小儿先天性肾盂输尿管连接部梗阻(UPJO)疗效的新方法。方法:对38例(43侧)先天性UPJO肾积水患者,先行患肾穿刺造瘘引流,再行肾盂输尿管成形术治疗。结果:37例(42侧)肾盂成形术均获成功,痊愈出院;1例肾功能受损严重,行患肾切除术。随访0.5~5年,经B超及利尿性动态肾核素扫描证实,37例患肾结构与功能都有不同程度的好转和提高。结论:肾穿刺造瘘引流肾盂输尿管成形术,是治疗小儿先天性UPJO的有效方法;但常规IVP检查不显影不能作为肾切除的指征。     

Management of ureteropelvic junction obstruction in neonate

Fetal hydronephrosis demonstrated by maternal ultrasonography should lead to early investigation during the neonatal period. Postnatal confirmation of the diagnosis of ureteropelvic junction obstruction usually can be established by combining the radiologic modalities of ultrasound, diuretic isotope renal scan, and voiding cystourethrography. Rarely should intravenous urography, antegrade pyelography, or cystoscopy and retrograde pyelography be necessary. Findings of high-grade obstruction and/or significantly diminished function on the affected side(s) should prompt early neonatal reconstruction. Pyeloplasty was performed within the first month of life in 17 infants (20 kidneys) diagnosed as having significant ureteropelvic junction obstruction. Early reconstruction in the neonatal period can be performed successfully with minimal complications and a relatively brief period of hospitalization. This may ultimately achieve maximal preservation of renal function.     

Pediatric congenital hydronephrosis (ureteropelvic junction obstruction): Medical management guide

The prevalence of asymptomatic hydronephrosis, now detected by ultrasonography, has increased. However, definitive management guidelines for the management of congenital hydronephrosis have not been established. The Japanese Society of Pediatric Urology created a “medical management guide” based on new findings for physicians practicing pediatric urology. We developed a medical management guide focused on congenital hydronephrosis caused by ureteropelvic junction obstruction. This medical management guide consists of the definition, pathophysiology, epidemiology, diagnosis, classification, treatment using a clinical management algorithm of hydronephrosis and the long-term course of the disease. The aim of hydronephrosis management is to determine whether surgery should be carried out to avoid renal dysfunction, as there is a possibility for improvement without intervention. Ultrasonography is essential to make treatment decisions. Management is determined by a comprehensive assessment, including the degree of hydronephrosis, anterior–posterior diameter of the renal pelvis and, if necessary, a nuclear medicine evaluation of the status of urine drainage and renal function.     

Surgical indications for unilateral neonatal hydronephrosis in considering ureteropelvic junction obstruction

Prenatal hydronephrosis is one of the most common urological congenital abnormalities detected by ultrasound. The incidence ranges from 0.59% to 0.69%. Approximately 50% of these fetuses do not have hydronephrosis on postnatal examination, whereas 25–33% of the rest have persistent hydronephrosis leading to the diagnosis of ureteropelvic junction (UPJ) obstruction. Renal ultrasonography and renal radionuclide scanning are the major modalities used for assessment and follow-up. Three main criteria used to determine the presence of obstruction are: (1) the magnitude of hydronephrosis present on ultrasound, (2) the relative renal function (RRF) measured by renography, and (3) the response of radionuclide washout with furosemide. Unfortunately, it is not always easy to determine obstruction; different types of management have been developed. Without depending on the severity of renal pelvis dilation, percentage of RRF, and response of radionuclide washout in the initial presentation, early surgery to preserve renal function and aggressive observation to prevent unnecessary surgery are two extremes on the spectrum of management for neonatal UPJ obstruction. Relying on renal function in renography, <35–40% or 5–10% of a decrease in the percentage of RRF or on the enlarging of hydronephrosis, respectively, and parenchymal thinning on ultrasonography are the indications for the surgical management to recover renal function in time. In addition to renal function change and imaging progression, the follow-up protocol and family compliance are the other considerations in prevention of impaired renal function. Through more than 40 years of development in the field of UPJ obstruction in infants, there have been several advances in management but controversies remain to be resolved. In this review, we focus on the surgical indications for the UPJ obstruction in this cohort.     

Histological findings of the ureteropelvic junction in congenital hydronephrosis (author's transl)]

Histological investigation of ureteropelvic junction (UPJ) specimens of 21 patients with congenital hydronephrosis revealed in 7 instances pathological changes, which might be responsible for the obstruction. In 15 cases a significant increase of collagenous tissue within the UPJ, along with a relative deficiency of monocytes was noted. Each of the smooth muscle fibers was surrounded by fibrous tissue, which caused interruption of the "nexus" function and therefore loss of muscle contractions. The inability of normal muscle contractions within the UPJ might be a contributing factor to the pathogenesis of congenital hydronephrosis.     

Ureteropelvic junction obstruction repair: when, how, what?

PURPOSE OF REVIEW: To review factors that affect the success of ureteropelvic junction obstruction repair and recent developments in minimally invasive procedures for the repair of ureteropelvic junction obstruction. RECENT FINDINGS: Recent reports and studies further confirm earlier findings that the success rate of endopyelotomy is decreased when a crossing vessel is the primary cause of ureteropelvic junction obstruction, poor renal function and significant hydronephrosis. Various minimally invasive procedures have emerged recently for the treatment of ureteropelvic junction obstruction. These include laparoscopic pyeloplasty, robotically assisted laparoscopic procedures, and percutaneous endopyeloplasty. These procedures offer potential advantages over conventional endopyelotomy, including better success rates in the presence of crossing vessels, wider caliber reconstruction of the ureteropelvic junction, and full-thickness healing with primary intent. SUMMARY: With such a large variety of minimally invasive procedures for the treatment of ureteropelvic junction obstruction available, the treatment choice for ureteropelvic junction obstruction must be based on several factors, including the success and morbidity of the procedures, the surgeon's experience, the cost of the procedure, and the patient's choice.