Renal function and vesicoureteric reflux in children with ureteroceles

A total of 215 ureteroceles were present in 41 children with single-system and 154 children with double-system ureteroceles. Males were predominant in the single-system group while the reverse was true in patients with double-system ureteroceles. Single-system ureteroceles occurred more commonly on the left side. In double-system ureteroceles there was minimal renal function in 74% of the ureterocele-bearing moieties, 26% of the ipsilateral lower moieties, and 3% of the contralateral kidneys. Vesicoureteric reflux (VUR) was present in 17% of the ureterocele-bearing ureters, 54% of the ipsilateral second ureters, and 28% of the contralateral ureters. In the single-system ureteroceles, 20% of the ipsilateral kidneys had minimal renal function and 9% of both the ipsilateral and contralateral ureters had VUR. Differences in laterality, VUR, and function of the subservient renal tissue suggest that single-system ureteroceles are a different disease entity from the double-system type and should be considered separately in any discussion of the management of ureteroceles.     

Single sysem ectopic ureters and ureteroceles associated with dysplastic kidney

Eight children forming an uncommon subgroup of renal obstructive dysplasia are presented. Each child had a nonfunctioning dysplastic kidney with a single collecting system with ectopic ureteral insertion and/or ureterocele. Five of the children had classic multicystic dysplastic kidneys, one had they hdyronephrotic type of multicystic dysplasia kidney had two had hypoplastic kidneys. Other significant medical problems in 5 of the 8 children (63%Z) included VACTERL association, congenital heart diseaise and other genitourinary malformations. Unlike some children with unilateral multicystic dysplasia kidney, this subgroup of children ahs an increased risk of infection. The must be correctly identified on imaging so that tailored clinical management decisions can be made and associated anomalies detected.Presented at the 1990 meeting o the RSNA     

Ectopic ureterocele in nonduplicated collecting systems

Ectopic ureteroceles in nonduplicated collecting systems are rare; only 34 well-documented cases have been reported in the English-language literature. We report two cases of a single-system ectopic ureterocele associated with a nonfunctional ipsilateral kidney in two boys. Nephroureterectomy and excision of the ureterocele were performed. One of the patients had a ventricular septal defect and the other had Sotos' syndrome. An ectopic ureterocele in a nonduplicated collecting system differs from a simple ureterocele or an ectopic ureterocele of a duplicated collecting system because of its prevalence in males, the frequent presence of a nonfunctional ipsilateral kidney, and the high incidence of associated anomalies such as congenital heart disease, crossed renal ectopia, and vas deferens abnormalities. Offprint requests to: R. Gonzalez     

The impact of fetal screening on indications for cystourethrography in infants

To evaluate cystourethrography in the era of fetal screening, we evaluated retrospectively the clinical and imaging findings of all children up to the age of 1 year who underwent a cystourethrogram in a 5-year period (1987–1992). There were 292 children, 64 neonates (<1 month, 51 boys, 13 girls) and 228 infants (1 month–1 year, 111 boys, 117 girls). Hydronephrosis detected prenatally and confirmed after birth by US was the indication for cystourethrography in 88 children (72 boys, 16 girls). This 4.5 to 1, male to female ratio is very unusual compared to children with urinary tract infection, the great majority of whom are girls. The findings based on imaging studies in these 88 children with hydronephrosis included 31 with vesicoureteral reflux (VUR) (in 4 this was secondary), 23 with obstruction at the ureteropelvic junction (UPJ), 13 with multicystic dysplastic kidney, 2 with obstruction at the ureterovesical junction, 1 with ectopic ureterocele and 1 with posterior urethral valves, both the latter without reflux. Eleven children had normal postnatal studies and six children were lost to follow-up. Urinary tract infection (UTI) was the indication in 163 children (62 boys, 101 girls). Forty-one children were examined for other causes. Although most cases of hydronephrosis were detected on fetal screening, UTI was still the most common indication for cystourethrography and some significant abnormalities were found in these symptomatic children.     

小儿输尿管膨出症的治疗--附91例分析

目的 选用创伤小，疗效好的术式治疗输尿管膨出症，减法二次手术。方法 91例输尿管膨出症，男25例，女66例，其中70例（76.9%)并发于重肾双输尿管畸形，其中左上输尿管膨出症40例，右上27例，双上3例，影像学检查；双上肾积水4例，患侧上肾积水52例，患侧上下肾积水10例，患侧上，下及对侧都有肾积水4例。21例单一系统输尿管膨出症中，左侧5例，右侧11例，双侧5例。影像学检查；患侧肾积水10例，双侧肾积水5例，患肾功能严重受损5例。上尿路正常1例。结果 78例（85.7%)术后平均随访2年。重肾组；（1）上肾部切除48例52侧，治愈36例，失访7例，需加做输尿管残留切除3例，需再经尿道戳穿输尿管膨出2例；（2）切除输尿管膨出及输尿管膀胱再吻合6例，治愈5例。术后加做上肾部切除1例；（3）经尿道戳穿输尿管膨出15例，治愈9例，失访4例。需加做上肾部切除2例；（4）输尿管膨出自行缩小，症状消失1例。单一系统21例中15例经尿道戳穿输尿管膨出，治愈12例，失访2例，需加做输尿管膀胱再植1例，1例经输尿管膨出切除，输尿管膀胱再植治愈，发育不良肾切除5例。治愈2例，有尿失禁症状2例，仍时有尿路感染1例。全组1次手术治愈65例。结论 输尿管膨出症的治疗须根据患肾功能决定，如患肾功能严重受损，首选患肾或肾部切除，反之则经尿道做膨出部下缘戳孔术，对单一系统肾发育不良并发异位输尿管膨出的女性，其位置低至尿道远端，因该处尿道肌肉缺损造成失禁，须进一步治疗。     

Abnormal contralateral kidney in unilateral multicystic dysplastic kidney disease

We performed a retrospective study of infants with unilateral multicystic dysplastic kidney (MCDK) in order to investigate the associated urological abnormalities. We examined the records of seven patients, in five of whom a diagnosis had been made prenatally using ultrasonography. Our investigation focused on the associated urological abnormalities, particularly on the contralateral side, and the results of voiding cystourethrography (VCUG). Four of the seven patients (57%) had urological abnormalities other than MCDK: three exhibited vesicoureteral reflux (VUR) of the contralateral side including one patient who also had an ipsilateral ectopic ureter, and the fourth patient had a ureterocele of the ipsilateral side. The results indicate that contralateral VUR, was the most common abnormality associated with MCDK. Two infants had high-grade VUR and underwent anti-reflux surgery soon after the diagnosis. Both have remained free of recurrent urinary tract infection.     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

Routine voiding cystourethrography is of no value in neonates with unilateral multicystic dysplastic kidney

OBJECTIVES: To determine if two successive ultrasound examinations could rule out the presence of clinically significant contralateral anomalies in neonates with multicystic dysplastic kidney (MCDK), thereby avoiding unnecessary voiding cystourethrography (VCUG). STUDY DESIGN: We followed 76 newborn infants with antenatally discovered MCDK. Two successive neonatal renal ultrasound examinations were performed, one within the first week and one at around 1 month of life. VCUG and isotopic studies were performed in all infants. RESULTS: Urologic anomalies of the contralateral kidney were present in 19 of 76 children (25%): vesicoureteral reflux (VUR) in 16 (21%), ureteropelvic junction obstruction in 2 (3%), and renal duplex kidney in 1 (1%). Sixty-one infants (80% of total) had normal contralateral urinary tract on the 2 successive neonatal renal ultrasound scans. Among them, 4 of 61 (7%) infants presented with low-grade VUR on VCUG that had resolved spontaneously before 2 years of age. The sensitivity, specificity, positive predictive value, and negative predictive value of two successive ultrasound scans in the neonatal period to predict contralateral urological anomalies on VCUG were 75%, 95%, 80%, and 93%, respectively. CONCLUSIONS: In infants with antenatally diagnosed MCDK, two successive normal neonatal renal ultrasound scans will rule out clinically significant contralateral anomalies, thereby rendering the need for a neonatal VCUG unnecessary.     

Prenatal diagnosis of primary megaureter]

22 of 114 infants with prenatal diagnosis of urinary tract malformations (15 boys) had obstruction of the ureterovesical junction caused by primary megaureter (n = 14), ureterocele (n = 7), or ectopic ureter (n = 1). Infants with infravesical obstruction or refluxing systems were excluded. All infants had a normal renal function and probably only 5 of them (23%) would have been diagnosed during infancy without prenatal diagnosis. A total number of 26 renal units was evaluated. Six infants had ipsilateral duplication with additional contralateral duplication (n = 2) or vesicoureteric reflux (n = 2). A nonfunctioning kidney or upper pole of duplication was noted in 7 units (5 with ureteroceles); in two instances a moderately dysplastic kidney was present. One boy with urinary infections developed renal scarring during follow-up. Conservative treatment was performed in 14 renal units while 12 units of 11 children had 22 operations (9 temporary diversions) at a mean age of 4,6 months. A severe operative complication was noted in one infant. Conservative treatment is justified in many infants with primary obstructive megaureters but severe renal dysplasia may be present even neonatally. Yet we feel that prenatal diagnosis benefitted most infants, especially those with the most severe malformations.     

Multicystic dysplastic kidney: Impact of imaging modality selection on the initial management and prognosis

ObjectiveTo evaluate the impact of imaging modalities on the evaluation and prognosis of children with multicystic dysplastic kidney (MCDK) disease.Patients and methodsA retrospective analysis of all children with MCDK diagnosed from 2004 until 2012 was performed. The study included 63 patients for whom all postnatal imaging modalities were available: renal bladder ultrasound (RBUS), dimercaptosuccinic acid scan (DMSA) and voiding cystourethrogram (VCUG). Cases with major congenital abnormalities or incomplete data were excluded. Abnormalities in the contralateral kidney and the fate of MCDK were also addressed.ResultsAt diagnosis, the average age was four-and-a-half months. The majority of cases were detected antenatally (87%). Postnatal RBUS and DMSA scans established the diagnosis of MCDK in 92% and 98% of patients, respectively. DMSA showed photopenic areas in the contralateral kidneys in 10% of patients; all of them had hydronephrosis and were confirmed to have vesicoureteral reflux (VUR). Contralateral VUR was detected in 16 patients; 63% of them had hydronephrosis. After a mean follow-up of three-and-a-half years, involution occurred in 62% of patients and the involution rate was inversely proportional to the initial size.ConclusionsThe classical appearance of MCDK on RBUS was sufficient to establish the diagnosis in most patients. DMSA scan was more accurate in confirming the diagnosis and evaluating the contralateral kidney. Selective screening for VUR in patients with contralateral hydronephrotic kidney should be considered.     

A spectrum of segmental multicystic renal dysplasia

Background. Multicystic renal dysplasia (MCDK) is a common anomaly well described in the literature, but less well described when involving only a portion of a kidney. Objective. To present the imaging spectrum, natural history and associated anomalies of six kidneys with segmental MCDK. Materials and methods. Five children with segmental MCDK (one with bilateral segmental MCDK) referred to our hospital between 1989 and 1996 were reviewed. All had at least one ultrasound examination. Four had a voiding cystogram and three had renal scintigraphy. Results. Four children had antenatal diagnosis of cystic renal abnormality. In two, with obvious duplex kidneys and associated ureteroceles, the diagnosis of upper moiety MCDK was obvious either antenatally or immediately postnatally. In the other three there were diagnostic difficulties. One patient had bilateral widespread cysts obscuring the functioning renal portions. Another presented in utero with a large ureterocele and a cystic upper pole that had involuted by birth. The fifth had a nephrectomy at 3 years for a multiloculated cystic mass. Varying degrees of involution occurred in the five kidneys seen early. Reflux was demonstrated into the ipsilateral functioning lower moiety and midpole. Conclusion. In these children as in other studies, the commonest presentation of segmental MCDK is in the upper pole of a duplex kidney associated with a ureterocele at the end of the atretic ureter. Atypical segmental MCDK may present a diagnostic dilemma and should be included in the differential diagnosis of multiloculated cystic masses and cystic kidneys. Received: 16 June 1998 Accepted: 16 November 1998     

核素利尿肾动态显像在儿童肾重复畸形中的临床应用

为评价99Tc^m-EC利尿肾动态显像在儿童肾重复畸形中的临床价值，对23例肾生复畸形住院患儿99Tc^m-EC利尿肾动态显像（RDI），分析其影像特征和主要功能指标的变化；设计可反映重复上半肾功能的指标（D/N），并以之与临床病理分型比较。结果显示：肾重复畸形在RDI连续叠加影像中有4种表现；上肾稀疏、上肾缺损、肾影增长、肾盂分离；RDI的灵敏度、特异性、准确性分别为73.3%、93.2%、81.4%；RDI对合并输尿管囊肿检出率较好，并可检出部分输尿管扩张积水；利尿肾图显示尿路梗阻类型均为非机械性梗阻。临床病理分型为发育不良型，其上肾D/N值均小于30%，发育型均大于70%，积水型为30%-70%。对术后随访的病例，RDI在双肾形态和功能转归方面有肯定的判断。说明99Tc^m-EC肾动态显像对肾重复畸形有较好的诊断价值可帮助判断重复上半肾功能，为临床床前选择手术方式提供重要依据可作为术后随访复查的主要检查手段。     

Congenital ureteroceles: an indication for screening?

In the pediatric population, ureteroceles may present with different clinical pictures, including obstruction and lithiasis. We report two pairs of twins; one of each set had ureteroceles, and the other a related urinary tract malformation (ureterocele, polycystic kidney disease). These cases raise the question of whether the siblings of children with ureteroceles should be screened for urogenital abnormalities. Received: November 1996 Accepted: 13 January 1997     

Dysplastic multicystic kidney: should the classic treatment (nephrectomy) be changed after prenatal diagnosis?

We reviewed 60 cases of multicystic renal dysplasia managed in our institution during a 10-year period, 54 of which were prenatally detected; 59 were unilateral dysplastic multicystic kidneys (DMK) and 1 bilateral. The mean gestational age at diagnosis was 28.1 weeks. Associated anomalies were present in 17 cases; 4 of these infants died. In 19 cases, a nephrectomy was initially performed. Among the 37 infants initially managed nonoperatively, 4 were lost to follow-up. The DMK was removed in 17 instances after 18 months of age (mean age 26.8 months), and 16 children with spontaneous regression of the lesion were followed. In 9 cases, complete disappearance of the dysplastic kidney was observed. Of the 36 kidneys removed, most were of the dysplastic type; a ureter was present in 21 cases and no nodular renal blastema was found. When opacification of the specimen was performed, communication between cysts was the most frequent finding. The critical question of the harmlessness of nonoperative management of DMKs has to be answered by a large-scale, long-term, multicenter study. Correspondence to: E. Sapin     

Evaluation of a three-year (1988-1990) prenatal screening of malformative uropathies in the department of Puy-de-D?me]

During a 3-year period, 93 prenatal diagnoses of kidney or urinary tract abnormalities were carried out in the French district of Puy-de-D?me. Sixty-nine mothers were resident in this area giving an incidence of 2.8 out of 1000 births. The pregnancy was interrupted in 10 cases, there were 2 stillbirths and three infants died within two months of life. The most frequent abnormalities were: hydronephrosis (48% of cases), megaureter with or without ureter duplication (19%) and multicystic dysplasia (16%). The prenatal diagnosis was confirmed after birth in 82% of cases. Of the 56 infants with obstructive uropathies, 17 underwent a pyeloplasty within three months of life, 32 had conservative treatment, of whom 4 were operated on afterwards, and seven could not be traced. Of the seven infants who had normal ultrasound scan at birth, three had abnormal scan during the follow-up one of whom was operated on. Nephrectomy was not performed in any of the 11 cases of multicystic dysplasia: one patient was lost to follow up, three had stable lesions and in seven cases, the size of the cysts decreased.     

Micturating cystourethrograms are not necessary for all cases of antenatally diagnosed hydronephrosis

ObjectiveSince 1995 we have, at our centre, adopted a selective approach to performing micturating cystourethrograms (MCUGs) on patients with antenatally diagnosed hydronephrosis. This study reviews the outcome of this policy.MethodsWe carry out MCUGs only if any of the following features are present on ultrasound: bilateral hydronephrosis, ureteric dilatation, renal scarring, bladder wall thickness greater than 5 mm, or presence of a duplex system or ureterocele. Patients with simple unilateral hydronephrosis are excluded, and are managed with 6 months’ trimethoprim prophylaxis and ultrasound surveillance with a minimum of 3 years’ follow up.ResultsFifty-five patients were referred with an antenatal diagnosis of hydronephrosis between 1999 and 2002; 26 (47%) did not have an MCUG. Of these, five had increasing hydronephrosis and required surgery for pelvi-ureteric junction obstruction, and three had a multicystic dysplastic kidney on postnatal scanning. In the remaining 18 patients, the hydronephrosis resolved spontaneously, with no renal scars or asymmetry. During follow up, none of these patients had a urinary tract infection.ConclusionWe believe that vesico-ureteric reflux in most antenatally diagnosed hydronephrotic kidneys is physiological rather than pathological, and resolves with time without causing long-term renal damage. This is a separate entity from, rather than a precursor of, the pathological symptomatic refluxing kidney in older, mainly female children. Taking a more conservative approach to the postnatal investigation of antenatally diagnosed hydronephrotic kidneys has not resulted in any missed damaged kidneys, but has reduced the number of invasive investigations performed. A careful protocol and detailed postnatal ultrasonography are important to prevent missed pathological cases.     

Renal dysplasia and MRI: a clinician's perspective

Renal dysplasia is a common abnormality in children. The role of MRI in evaluating children with renal dysplasia is evolving. More information is clearly necessary before MRI replaces conventional imaging modalities. In order to appropriately use MRI, the radiologist must have an understanding of the clinical questions that are important in the management of children with renal dysplasia. This review provides background information on renal dysplasia for the pediatric radiologist. The focus is on unilateral disease, especially multicystic dysplastic kidneys, and bilateral dysplasia, which is the most common cause of kidney failure in children. The emphasis is on the important clinical issues, and the potential of MRI as a methodology for providing clinically useful information not otherwise available from other imaging modalities.     

Cystic dysplasia of the testis associated with multicystic dysplastic kidney

Cystic dysplasia of the testis (CDT) is a rare congenital defect characterized by the formation of multiple irregular cystic spaces in the mediastinum testis. Co-existent genitourinary lesions have commonly been associated with this lesion and have included absence of the ipsilateral kidney, duplication anomalies, and cryptorchidism. We describe the first case in which multicystic dysplastic kidney (MCDK) is associated with CDT.