先天性肾盂输尿管连接处梗阻的诊断与治疗

目的：总结肾盂输尿管连接处理梗阻（PUJO）引起的小儿先天性 肾积水的诊断及治疗经验。方法：回顾性分析22例由PUJO引起的小儿先天性肾积水的临床资料。结果：术前诊断符合率90.9%(20/22),手术治愈率90.0%(18/20)。结论：B超为由PUJO引起的先天性肾积水首选的诊断方法;电视监视IVU为轻度由PUJO 引起的先天性肾积水的主要诊断方法;CT为中重度由PUJO引起的先天性肾积水的主要诊断方法。Anderson-Hynes成形术为其首选的治疗方法。     

Neuroblastoma masquerading as congenital ureteropelvic junction obstruction.

A case of perirenal neuroblastoma causing intrarenal obstruction of the pelvis of the kidney presented as a small hydronephrosis at 3 days of age. The tumor grew rapidly over the next 48 hr, diagnosis was confirmed by ultrasonography, and the mass was removed surgically.     

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.     

Structural changes of smooth muscle in congenital ureteropelvic junction obstruction

Background/Purpose

Ureteropelvic junction (UPJ) obstruction is the most common cause of congenital hydronephrosis. Previous studies have reported that the excess amount of collagen restricting mobility and resiliency of the UPJ is the result of an impaired collagen production by anomalous smooth muscle cells (SMCs). Our purpose was to evaluate the role of SMC differentiation in the pathogenesis of UPJ obstruction.

Methods

Surgical specimens of UPJ from 21 patients (8 girls/13 boys) who were subjected to dismembered pyeloplasty were examined immunohistochemically using monoclonal antibodies against smooth muscle (SM) myosin heavy chain isoforms including SM1, SM2, and SMemb. The age ranged from 1 month to 13 years. Ureteropelvic walls taken from 14 forensic autopsy cases, with no urological abnormalities, served as age-matched control group.

Results

The immunohistochemical expression of SM1 and SM2 in UPJ obstruction was significantly increased when compared with controls (P < .05). In contrast, there was no statistical difference of expression of SMemb.

Conclusion

Our findings supported the hypothesis that the primary anomaly in UPJ obstruction may be attributed to a malfunction of SMCs in the ureter.     

The distribution of interstitial cells of Cajal in congenital ureteropelvic junction obstruction

Purpose

The authors analysed the distribution of c-kit-positive interstitial cells of Cajal (ICCs) in obstructed ureteropelvic junction (UPJ) and its age-related changes.

Methods

Twenty specimens were obtained from children with intrinsic ureteropelvic junction obstruction (UPJO), at the average age of 8.1 years (8 months–16.8 years), fixed in formalin and embedded in paraffin. Five control samples were taken from children at the average age of 2.3 years (2.4 months–7.4 years). All specimens were analysed by the immunohistochemistry test with light microscopy with respect to c-kit expression. The distribution of c-kit-positive ICCs in the two groups was compared and the correlation between the distribution of c-kit-positive ICCs and the patients’ age in UPJO cases was analysed. The results were examined by Yates’ χ² test, Mann–Whitney U test, and t test for Pearson’s correlation coefficient. A P value < 0.05 was considered as statistically significant.

Results

No statistically significant differences were found in the distribution of c-kit-positive ICCs between UPJO and the control group. No correlation was established between the age of patients with UPJO and the distribution of c-kit-positive ICCs.

Conclusion

No distributional difference found in obstructed and unobstructed UPJ seems to indicate that UPJO is not associated with anomalous distribution of c-kit-positive ICCs. Age-related changes in the expression of c-kit-positive ICCs are equally distributed in obstructed UPJ.     

肾盂输尿管交界部功能性梗阻

自１９８５～１９９１年间共收治先天性肾盂输尿管交界部（ＵＰＪ）功能性梗阻患者３７例，病理检查而确诊，均经手术治疗。其中近期７例手术标本作了电镜检查，结果证实本病的病因为ＵＰＪ处平滑肌细胞之间有大量胶原纤维组织堆积，平滑肌细胞本身发育不良所致传导功能障碍。治疗应尽量切除病变的ＵＰＪ组织。     

先天性肾盂输尿管交界部梗阻的影像学诊断方法比较

为提高对先天性肾盂输尿管交界部梗阻(UPJO)的诊断,评价影像诊断的价值,对80例先天性UPJO的病人的影像学检查资料进行回顾性比较分析,结果表明所有病人均作了静脉肾盂造影及B超检查;50例行逆行肾盂输尿管造影;20例曾行腹部CT检查;利尿性肾核素检查25例。75例外科手术治疗。先天性UPJO可分为两大类,动力性和机械性梗阻。动力性梗阻在临床及影像学表现上无特异性,机械性梗阻有其特征性的影像学表现。因此认为尿路造影诊断可以明确诊断,优于CT、B超、同位素的诊断价值。     

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。     

Percutaneous pyeloplasty (endopyelotomy) for congenital ureteropelvic junction obstruction.

Endopyelotomy was performed in 30 patients with congenital primary ureteropelvic junction obstruction; 4 patients had high insertion of the ureter and 8 patients had caliceal stones. Clinical and radiologic success was achieved in 25 patients. There were five failures, all of whom subsequently had successful open pyeloplasty. The theoretical and experimental foundations of the procedure and fine points of the operative technique are presented. Endopyelotomy appears to be valuable for primary ureteropelvic junction obstruction just as it is for secondary obstruction.     

Dominantly inherited ureteropelvic junction obstruction

We report on siblings from two families with unilateral ureteropelvic junction obstruction. HLA studies were undertaken and found to be a useful marker between affected members. We believe that incomplete penetrance with variable expression is the most probable mode of transmission of this disorder.     

Renal biopsy in congenital ureteropelvic junction obstruction: evidence for parenchymal maldevelopment

The renal histologic changes associated with congenital ureteropelvic junction obstruction (UPJO) and the relationship to clinical imaging have not been well studied. In order to better understand the histologic alterations of congenital UPJO and their relationship with clinical imaging and outcomes, we examined renal biopsies from 61 patients undergoing pyeloplasty for congenital UPJO. Glomeruli were analyzed for various injury patterns and the tubulointerstitium was examined for tubular atrophy/simplification and fibrosis. Two methods were used to evaluate tubular mass: glomerular density and morphometric measurement of tubular size and density. Control specimens were obtained from age-matched autopsy specimens without renal pathology. Glomerular changes were identified in 73% of all biopsies and were present in a range from 1.7 to 91% of glomeruli in each patient. Overt tubulointerstitial changes were present in 26% of all biopsies. Fibrosis was noted to occur with tubulointerstitial changes in a significantly greater fraction of children over the age of 1 year (P=0.026). Increased glomerular density was associated with severe hydronephrosis (P<0.02). Normal glomerular density was inversely correlated with age (P<0.001), but this relationship was more variable in UPJO (P<0.01). Among patients with intact differential function preoperatively (>45%), postoperative functional decline was predicted only by increased glomerular density. 20 biopsies without overt tubulointerstitial changes were analyzed morphometrically and showed a significant reduction in proximal tubular (PT) size, but unchanged density. Distal tubular (DT) size was unchanged in UPJO, but density was increased. The PT/DT ratio was therefore markedly decreased in UPJO (P<0.0001). Both PT and DT sizes were significantly larger in children with a diuretic renogram washout time less than 20 min than those with greater than 20 min, a common threshold for functionally significant obstruction (P<0.05). Capsular thickness was significantly increased in UPJO. In all, 36% of biopsies had a thickness >0.5 mm and this was associated with greater degrees of tubulointerstitial changes and glomerular alterations. Congenital UPJO produces a variety of renal parenchymal changes, which may in part reflect abnormal development. Some of these alternations are seen in clinical imaging and may help predict outcomes, but there is significant discordance between conventional imaging and histological findings.     

Changes in interstitial cell of Cajal-like cells density in congenital ureteropelvic junction obstruction

Purpose  

The authors examined the number of interstitial cells of Cajal-like cells (ICC-LCs) in obstructed ureteropelvic junction (UPJ) in comparison with normal UPJ specimens and age-related changes.     

后腹腔镜治疗儿童先天性肾盂输尿管连接部梗阻的价值

目的:探讨后腹腔镜肾盂成形术治疗儿童先天性肾盂输尿管连接部梗阻(UPJO)的可行性、手术技巧及临床应用价值。方法:回顾性分析26例先天性UPJO患儿经后腹腔镜肾盂成形术治疗的临床资料。20例患儿因患侧腰腹胀痛就诊,2例因血尿就诊,4例体检时发现。并发肾盂结石4例。在后腹腔镜下游离肾盂及输尿管上段,斜行切除狭窄段,用5-0可吸收线全层缝合输尿管近端与肾盂做V-Y吻合。留置支架管及肾盂造瘘管。结果:26例手术均获得成功。随访3～18个月,复查静脉尿路造影示吻合口无狭窄,肾积水、肾功能均得到改善。结论:采用后腹腔镜肾盂成形术治疗儿童先天性UPJO效果显著,安全可行,较开放手术创伤小,可以替代开放手术成为儿童先天性UPJO的首选治疗方式。     

腹腔镜手术治疗肾盂输尿管连接部狭窄19例

目的 评价腹腔镜手术治疗肾盂输尿管连接部狭窄（ureteropelvic junctiono bstruction，UPJO）的疗效。方法 采用经腹路径对19例UPJO行离断式。肾盂输尿管成形术。打开侧腹膜，以。肾下极为标志游离出。肾盂输尿管连接部，切除狭窄部分，端端吻合肾盂输尿管并留置双J管。结果 19例手术全部成功，手术时间110～240min，平均150min。术中出血量50～100ml，平均80ml，无严重并发症发生。术后住院6～10d，平均7．8d。19例随访3～15个月，平均6个月，14例静脉尿路造影（intrarenous urography，IVU）无吻合口狭窄。结论 腹腔镜下离断式肾盂输尿管成形术治疗UPJO有效、可行，可以替代开放手术。     

Management of neonatal ureteropelvic junction obstruction

No topic in urology is more contentious than the management of neonatal ureteropelvic junction obstruction. Those favoring early diagnosis and correction of obstruction in early infancy cite excellent return of function and superior surgical results compared with delayed repair. Others believe that hydronephrosis improves or resolves with growth in many instances. In the most widely quoted series, almost all such patients improved. This article attempts to separate opinion from fact. The arguments for early surgery and for observation, even when obstruction has been diagnosed, are reviewed. Several experiences with long-term surveillance are summarized.     

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.     

Non-invasive markers of ureteropelvic junction obstruction

Non-invasive prognosis of the clinical progression of disease is of high interest, especially in newborn and children. Neonatal ureteropelvic (UPJ) junction obstruction needs close and invasive surveillance to determine the necessity of pyeloplasty. A number of groups have initiated research with the aim to find non-invasive biomarkers for UPJ obstruction. Two different strategies have been followed. One strategy, based on the knowledge obtained in animal models of UPJ obstruction, has identified a number of individual urinary markers of severe UPJ obstruction. Combining these markers might allow prediction of which patients will require surgery and in which patients UPJ obstruction will spontaneously resolve. The other strategy is based on urinary proteomics. In this strategy the entire urinary proteome is probed for a set of biomarkers that correlates with the degree of UPJ obstruction. In subsequent steps, these sets of urinary biomarkers are used for prediction of the clinical evolution of UPJ obstruction patients. This proteomic-based strategy allowed prediction, several months in advance, of the clinical evolution of neonates with UPJ-obstruction. Both strategies will be complementary and will hopefully replace in the near future the invasive follow-up of newborns with UPJ obstruction.