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Renal cystic diseases are common conditions whose etiology can be highly heterogeneous. They require a correct approach for adequate diagnosis and management. We aimed to illustrate part of the spectrum of renal cystic diseases through some clinical cases managed in our service. We describe 11 clinical cases including clinical entities such as renal multicystic dysplasia, and autosomal dominant and autosomal recessive polycystic renal disease, among other pathologies. Renal cystic diseases are heterogeneous in their clinical presentation, natural history, radiological findings, and genetic and pathophysiological basis. An integral clinical approach is needed to get a clear etiological diagnosis and offer adequate individualized care and follow-up for patients.

Risks of contralateral kidney abnormalities and chronic kidney disease necessitate follow-up for unilateral multicystic dysplastic kidneys (MCDK). A nationwide survey of senior UK pediatricians was conducted. Of the 60 responses obtained, 62% routinely perform a dimercaptosuccinic acid scan to confirm diagnosis. Eight percent routinely perform a cystogram to investigate contralateral vesicoureteric reflux. Sixty-two percent would routinely measure renal function (frequency ranging from once only to "every 2 years"). Twenty-five percent recalled MCDK nephrectomy being performed within the previous 5 years. Respondents voiced concerns that national guidance may result in an overcautious approach but could balance consensus and safe variation, and offer families choice and reassurance. The mean estimated cost of follow-up from birth to 18 years ranged from £258 to £3854. Results demonstrate significant variation in management, highlighting the need for a clear pathway to decrease unwanted variability and to ensure those at high risk of renal sequelae are recognized early, without undue investigatory burden., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder and the fourth cause of death of end-stage renal disease. The disease has a prevalence of 1:400-1:1000 accounting for 10% of patients on dialysis. In most ADPKD patients, bilateral kidneys are similarly affected, with numerous fluid-filled cysts arising from different nephron segments. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic., Case Presentation: We report a case of a 46-year-old Ghanaian male patient who presented with left flank pain and hematuria with high BP and deranged renal function. Abdominal ultrasonography showed both kidneys to be larger than normal and had multiple cysts of varying sizes with the right kidney located in the right iliac fossa. Follow up Abdominopelvic computer tomographic scan (CT-Scan) without contrast showed enlarged kidneys with the renal parenchyma replaced by innumerable cyst of varying sizes. The right kidney was ectopically located in the right aspect of the pelvis. A diagnosis of ADPKD with right pelvic ectopic multicystic kidney was made. He was put on antihypertensives, analgesia for the left flank pain and to have follow up at the urology and nephrology departments., Conclusion: In most ADPKD patients, bilateral kidneys are similarly affected. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic., (© 2023. The Author(s).)

Background: Multicystic dysplastic kidney (MCDK) and unilateral renal agenesis (URA) are the most common reasons for a congenital solitary functioning kidney (SFK). We aimed to assess the presence of abnormalities in the congenital SFK and evaluate kidney function using chrome EDTA (CrEDTA) measurements., Methods: We retrospectively reviewed the medical records of 154 children with MCDK and URA in the period from 2005 to 2022 to analyze results from ultrasound scans and CrEDTA glomerular filtration rate (GFR) examinations., Results: Of 154 children with a solitary kidney due to MCDK (62%) or URA (38%), abnormalities on the congenital SFK were found in 13 children (8%). The abnormalities spontaneously resolved in 6 children (46%). The most common abnormality was hydronephrosis. Compensatory hypertrophy was found in 17% of the children within the first 6 months of life. 116 children (90%) had a standard GFR (sdGFR) above 75% of expected for the age. Out of those with a sdGFR below 75% of expected, 3 (23%) had abnormalities in the congenital SFK. There was no difference in sdGFR between children with MCDK and URA., Conclusions: Our study is the first using CrEDTA for GFR measurements and suggests that most children with a congenital SFK due to MCDK or URA have a kidney function within expected for the age. Compensatory hypertrophy of the SFK is found in a minority of children within the first six months of life, suggesting that this process is developing over time. The prevalence of abnormalities in the SFK seems low, however those with abnormalities (e.g. hydronephrosis) are at higher risk of reduced sdGFR., Competing Interests: Conflicts of interest The authors declare that there is no conflict of interests regarding the publication of this paper., (Copyright © 2023 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Multicystic dysplastic kidney (MCDK) is one of the most common fetal malformations, but its etiology remains unclear. Identification of the molecular etiology could provide a basis for prenatal diagnosis, consultation, and prognosis evaluation for MCDK fetuses. We used chromosome microarray analysis (CMA) and whole-exome sequencing (WES) to conduct genetic tests on MCDK fetuses and explore their genetic etiology. A total of 108 MCDK fetuses with or without other extrarenal abnormalities were selected. Karyotype analysis of 108 MCDK fetuses showed an abnormal karyotype in 4 (3.7%, 4/108) of the fetuses. However, CMA detected 15 abnormal copy number variations (CNVs) (14 pathogenic CNVs, and one variant of unknown significance [VUS] CNVs), in addition to four cases that were consistent with the results of karyotype analysis. Out of the 14 pathogenic CNVs cases, three were of 17q12 microdeletion, two of 22q11.21 microdeletion, 22q11.21 microduplication uniparental disomy (UPD), and one case of 4q31.3q32.2 microdeletion, 7q11.23 microduplication, 15q11.2 microdeletion, 16p11.2 microdeletion, and 17p12 microdeletion. Of the 89 MCDK fetuses with normal karyotype analysis and CMA, 15 were tested by WES. Two (13.3%, 2/15) fetuses were identified by WES as Bardet-Biedl syndrome (BBS) 1 and BBS2. Combined application of CMA-WES to detect MCDK fetuses can significantly improve the detection rate of genetic etiology, providing a basis for consultation, and prognosis evaluation.

Purpose: To compare the prevalence of vesicoureteral reflux (VUR), febrile urinary tract infection (fUTI), and chronic kidney disease (CKD) among patients with multicystic dysplastic kidney (MCDK) diagnosed by renal scintigraphy (RS) versus follow-up renal ultrasound (RUS) alone., Methods: This was a retrospective review of patients seen at a tertiary care center from 2010 to 2020 with MCDK diagnosed by RS or follow-up RUS. Differences in the prevalence of VUR, fUTI, and CKD by cohort were assessed using logistic regression analysis, Pearson X 2 , and Fisher's Exact tests. Temporal trends in diagnostic methods used (RUS versus RUS + RS) were evaluated using the Cochran-Armitage trend test., Results: One-hundred seventy-two patients were included: 50% (n = 86) underwent RUS + RS and 50% (n = 86) underwent RUS alone to diagnose MCDK. Prevalence of VUR, fUTI, and CKD did not significantly vary between groups. Among patients who had a VCUG, 4.4% had contralateral VUR (1.7% RUS + RS group; 7.4% RUS group; p = 0.19) and 14.5% had at least one fUTI (16.3% RUS + RS group; 12.8% RUS group; p = 0.52). Females were significantly more likely to have at least one fUTI (p = 0.04). Four patients (2.3%) developed CKD, all in the RUS + RS cohort (p = 0.12). Diagnosis of MCDK by RUS versus RUS + RS did not significantly vary over time (p = 0.17)., Conclusion: Patients with unilateral MCDK confirmed by RS versus RUS alone do not significantly vary in the prevalence of VUR, fUTI, or CKD. Renal scintigraphy studies may not be necessary in unilateral MCDK diagnosis but continue to be used., (© 2022 Wiley Periodicals LLC.)

We present a clinical imaging question focusing on a newborn with known prenatal history of a multicystic dysplastic kidney, and incidental ipsilateral retrovesical seminal vesical cysts, raising the diagnosis of Zinner syndrome. The diagnosis is challenging in this age group. Surgical management is generally proposed for symptomatic cases at an older age., (Copyright © 2021. Published by Elsevier Inc.)

Objectives: To assess the prenatal growth pattern of the normal kidney contralateral to a multicystic dysplastic kidney (MCDK)., Methods: A retrospective study was conducted in a single referral center over 4 years. Cases diagnosed prenatally as MCDK and confirmed postnatally constituted the study group. For creation of nomograms, only isolated cases of MCDK were included., Results: Sixty-one fetuses had a diagnosis of an MCDK during the study period. After exclusion of cases with associated malformations, 47 fetuses remained, providing 94 measurements for creation of nomograms. The growth pattern of the normal kidney contralateral to an MCDK was linear throughout gestation (percentile = 20.01 + 1.5 gestational age; linear R 2  = 0.753; r = 0.868) and was significantly higher during the third trimester (29-38 weeks' gestation) compared to the second trimester (22-28 weeks' gestation; P < .001). A comparison of the growth pattern of the normal kidney contralateral to the MCDK to the growth pattern of a solitary kidney revealed a significant higher compensatory trend during the third trimester (P < .0001). The mean kidney lengths at 22 and 38 weeks' gestation correlated with the 52nd and 88th and with the 84th and 90th percentiles for the normal kidney contralateral to the MCDK and a solitary kidney, respectively., Conclusions: According to our study, the normal kidney contralateral to an MCDK has a unique growth pattern during intrauterine life, with dominant growth during the third trimester. The exact mechanism for this pattern, in comparison to early renal hypertrophy shown in solitary kidneys, is currently not clear. These data provide relevant information for the multidisciplinary prenatal counseling of future parents regarding the future renal outcome., (© 2020 American Institute of Ultrasound in Medicine.)

Background and Objectives: Children with isolated unilateral multicystic dysplastic kidney (MCDK) or congenital solitary kidney (CSK) undergo serial renal ultrasonography with variable frequency until they are transitioned to adult care. A growing body of literature suggests the value of frequent ultrasonography in this population is limited, providing no benefit to overall outcomes. Despite emerging evidence, ultrasound remains overused, resulting in avoidable health care expenditures and unnecessary use of resources. With our initiative, we aimed to improve quality of care by reducing avoidable ultrasounds in these children., Methods: This was a single-center, prospective, interrupted time series of children <18 years with ultrasound-confirmed isolated unilateral MCDK or CSK in the outpatient nephrology clinic to evaluate the effect of a decision-making algorithm on the proportion of children receiving an avoidable ultrasound. An algorithm depicting a consensus, evidence-based protocol for managing pediatric MCDK or CSK was refined through content expert feedback and usability testing to standardize frequency of ultrasonography. Ultrasounds were deemed necessary after birth, at 6 months, and at 2, 5, 10, and 15 years. Differences pre- and postintervention were determined by using a U chart and t and F tests for significance., Results: The algorithm resulted in a 47% reduction (P < .001) in the proportion of avoidable ultrasounds ordered in children with MCDK and CSK. This reduction was sustainable over a 6-month period and would result in at least $46 000 annual savings., Conclusions: Introduction of a clinical decision-making algorithm was associated with a reduction in avoidable ultrasound testing. Improving adherence across providers may allow for an even more pronounced reduction., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Background/aim: The objective of this study is to determine the clinical features of unilateral multicystic dysplastic kidney (MCDK) patients., Materials and Methods: The demographic, clinical, laboratory, and radiologic features of MCDK patients at Diyarbakır Children’s Hospital and Diyarbakır Gazi Yaşargil Training and Research Hospital between January 2008-June 2019 were retrospectively evaluated., Results: A total of 111 [59 (53.2%) male and 52(46.8%) female] patients with MCDK were followed for a mean period of 41.89 ± 32.03 months. MCDK was located on the left and right sides in 46 (41.4%) and 65 (58.6%) of the children, respectively (p > 0.05). A total of 87 (78.4%) patients had antenatal diagnosis. The mean age at diagnosis was 13.7 ± 34.2 months. Of the 49 voiding cystourethrogram (VCUG)-performed patients, vesicoureteral reflux was detected in 11 patients (22.4%). Other associated urological anomalies in the patients were detected in 12 (10.8%) patients. On Tc-99m dimercaptosuccinic acid (DMSA) scintigraphy which was performed in all patients showed scarring in four children. Eight patients had history of UTI (7.2%). Renal failure, hypertension, and proteinuria were diagnosed in three children (2.7%). Sixty-nine (62%) patients developed compensatory hypertrophy., Conclusion: All cases should be followed up closely and VCUG should be reserved for patients with recurrent UTI and other urological problems indicated by ultrasonography and abnormal DMSA scan results., Competing Interests: none declared., (This work is licensed under a Creative Commons Attribution 4.0 International License.)

Multicystic dysplastic kidney is a rare congenital anomaly of the kidney and urinary tract. The association with the posterior urethral valve is also very rare. Here we present a patient with both entities and prenatal resolution of the cysts.A 10-week old baby was referred for nephrourological work up due to prenatal diagnosis of the left multicystic kidney. He had serial US scans during the pregnancy. Immediately before delivery the cysts were not seen (prenatal resolution). There were no extrarenal anomalies. The first postnatal ultrasound scan revealed normal sized right kidney without dilatation of the pelvicalyceal system. The bladder had normal thickness of the wall. Technetium-99m dimercaptosuccinic acid scan showed no activity on the left side, and the right kidney appeared normal. At two months of age, a poor urinary steam was observed and additional urologic work up was indicated on clinical suspicion of PUV. Voiding urethrocystography revealed posterior urethral valve and the baby underwent cytoscopic valve resection.Conclusion: We present a rare association of two congenital anomalies of the kidney and urinary tract with prenatal involution of the multicystic dysplastic kidney that is extremely rare event as seen in our case. Presence of posterior urethral valve must be suspected in a male baby with a poor urinary stream even when his ultrasound scan of urinary system appears normal., (© 2021 Natasha Aluloska et al., published by Sciendo.)

With the advent of routine prenatal imaging, the number of renal anomalies identified prenatally has significantly increased; however, the underlying etiologies of these anomalies and the clinical significance of these findings remains unclear. This confusion is especially true for the prenatal diagnosis of cystic renal changes. The terms "cystic kidney disease" and "renal cystic dysplasia" encompass myriad renal diseases. Although renal cystic dysplasia in infants shares many similarities with multicystic dysplastic kidney (MCDK), it is important to distinguish MCDK from other etiologies that would lead to renal cysts, to ensure proper patient diagnosis and appropriate counseling regarding risks and to guide clinical management. The purpose of this review is to highlight the multiple etiologies of cystic kidney disease, including genetic associations, associations with underlying syndromes, and associations with underlying anatomical abnormalities. Here we focus on prenatal imaging, associated pathological findings, and clinical significance, with an emphasis on the defining characteristics of MCDK as compared to other forms of cystic renal disease.

Objectives: To assess outcomes of unilateral multicystic dysplastic kidney (MCDK) managed at an Australasian centre over a 15 year period. To assess if MCDK involution could be predicted based on change noted between first two postnatal ultrasound scans 6 months apart., Subjects and Methods: A retrospective study was performed., Results: One-hundred-and-six cases of unilateral MCDK were studied. Eighty-four of these presented antenatally. Twenty-two MCDK cases presented postnatally. Urological anomalies associated with MCDK included vesicoureteric reflux (VUR), ureterocele and contralateral pelviureteric junction obstruction (PUJO). Children undergoing surgical intervention for these anomalies were offered concurrent MCDK nephrectomy. Morbidity associated with MCDK under surveillance included febrile culture-positive urinary tract infection in 20 cases (20.7%), hypertension in four (3.7%) and Wilms' tumor in one (0.9%). Thirty-six cases (34%) underwent complete involution, 32 (30.2%) were in the process of involuting and 38 cases (35.8%) underwent nephrectomy because of failure of involution or associated morbidity. If the MCDK reduced in cranio-caudal interpolar length by 20% or more between the first postnatal USS and the next one 6 months later, then it was very likely to involute spontaneously. If the MCDK did not reduce in cranio-caudal interpolar length by 20% between the first postnatal scan and the next one 6 months later, then it was highly likely to fail to involute, and in our study, correlated with the outcome of nephrectomy., Conclusion: Although MCDK is a benign condition, it should be carefully investigated and followed-up, as involution may not occur in over a third. In some cases, morbidity may occur. Each case of MCDK should be managed on its own merits., Level of Evidence: Level II - Prognosis study, Retrospective., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

Background: Ectrodactyly-ectodermal dysplasia-clefting syndrome 3 (EEC) is one of the six overlapping syndromes caused by mutations in the tumor protein p63 gene (TP63). EEC is suspected when patients have cleft hands or feet, polydactyly, and syndactyly, abnormal development of the ectodermally derived structures, and orofacial clefting. Genitourinary (GU) anomalies have been identified in patients with EEC, yet these are often under-recognized and under-reported. The available literature on sonographic prenatal findings is sparse, especially when considering GU anomalies., Methods: We present the case of a male stillborn fetus, who was found antenatally to have multicystic dysplastic kidneys and anhydramnios. Following the termination of pregnancy, examination and autopsy further revealed unilateral polydactyly and bilateral syndactyly which had not been previously identified on antenatal ultrasound., Results: Whole-exome sequencing (WES) revealed a de novo heterozygous pathogenic variant in exon 5 of the TP63 gene: p.His247Arg: c.740A>G (NM&#95;003722.4) which has been reported in the literature. The His247Arg variant has been published as a pathogenic variant in association with EEC, both with and without orofacial clefting., Conclusion: Our prenatal case expands the phenotypic spectrum of TP63-related disorders in general. In addition, it adds to the phenotype associated with the His247Arg pathogenic variant responsible for EEC. Further, we highlight the importance of WES as a postnatal tool to help clarify unexpected findings, and as a way to add to the spectrum of existing phenotypes of known single-gene disorders., (© 2020 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals LLC.)

Multicystic dysplastic kidney (MCDK) is the most common Congenital Abnormalities of Kidney and Urinary Tract (CAKUT) in clinical practice. Its etiology and pathogenesis are still controversial and obstruction is a generally accepted causative assumption. Obstetric ultrasound is the gold standard for prenatal diagnosis and for the detection of other associated malformations. Prenatal management is based on ultrasound monitoring of pregnancy, of the disease, of the occurrence of other anomalies and of the amount of amniotic fluid. This study aims to report our experience in the Department of Gynecology-Obstetrics 2 at the University Hospital Hassan II-Fez in order to clarify the epidemiology of MCDK, highlight the role of obstetric ultrasound in positive and etiological diagnosis while describing the various sonographic appearances. It is essential to schedule for screening tests during pregnancy in order to assess the evolution of the disease diagnosed in the prenatal period, to implement a strategy in postpartum care and to establish a prognosis., Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêts., (© Hekmat Chaara et al.)

Objective: We purposed to review prenatal diagnoses of ureterocele, to determine the sonographic findings and additional abnormalities, and to illustrate the pregnancy outcomes of these patients., Material and Methods: We reviewed the records of 24 patients with the diagnosis of ureterocele in our referral center between January 2010-March 2017. Prenatal sonographic findings, antenatal course, and postnatal follow-up were obtained., Results: The mean gestational age at first US diagnosis was 24.5 ± 2.9 weeks. 13 (54.1%) of fetuses were female, and 11 (45.9%) were male. Ureterocele was associated with the duplex kidney in 17 (70.8%), MCDK in 5 (20.8%) and hydronephrosis with a single system in 1 (4.2%) and pelvic kidney in 1 (4.2%) fetuses. Postnatal follow-up was achieved in 22 of 24 (91.6%) cases, and mean follow-up interval was 56 ± 14.2. Months. The diagnosis of ureterocele was confirmed in 22 (91.6%) cases postnatally. 15 of 22 (68%) cases were classified as extravesical ureterocele, and 7 (32%) cases were intravesical ureterocele. Postnatal confirmation of duplex kidney achieved in 16 of 17 (94.1%) patients. 17 (77.2%) patients were required surgical intervention, and 5 (22.8%) cases were managed conservatively. 15 of 16 (93.7%) cases who were diagnosed duplex kidney underwent surgery however 2 of 5 (40%) cases which were confirmed MCDK required an operation. Cystoscopic ureterocele incision was the initial approach for the surgical management and performed all of the cases which required surgery. It was curative in 10 of 17 (58.8%) patients and 7 (41.2%) cases needed to further operations. Ureteroselectomy and common-sheath ureteroneocystostomy was performed in 5 (29.1%) cases and. 2 (%11.7%) cases underwent partial nephrectomy., Conclusion: Ureterocele can be accurately diagnosed by prenatal sonography, and it is a significant clue for the diagnosis of a duplex kidney. Postnatal prognosis depends on associated anomaly and presence of reflux and upper pole function., (Copyright © 2019. Published by Elsevier B.V.)

The genitourinary system for reasons unknown is more likely to have birth defects than any other system. The anomaly of collecting system draining the kidney represent mystifying subset of congenital anomalies. Pelviureteric junction obstruction (PUJO) is most common. Chronic obstruction can lead to stasis, urinary infection and stone formation in PUJO. Extrarenal calyces, which is characterised by presence of calyces and renal pelvis outside the renal parenchyma is one of the rare anomalies seen among the collecting system right gonadal vein drains into inferior vena cava. Its altered drainage into right renal vein is rarely seen and reported. Glut of these multiple anomalies in a single case is an extremely rare event. We hereby discuss a case of 40-year-old male patient with combination of all these anomalies and discuss the embryology, presentation and management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

The aim was to compare the renal dynamic scan (RDS) performed with technetium-99-L,L-ethylene dicysteine (Tc-L,L-EC) and technetium-99-mercaptoacetyltriglycine (Tc-MAG3) in children with pelviureteric junction (PUJ) obstruction. A retrospective study was carried out and children with PUJ obstruction who had RDS performed with both Tc-L,L-EC and Tc-MAG3. Children with any intervention in between the two scans or a gap of more than 2 months in between renal scans were excluded. The dose of each radiotracer used was 0.1 mCi/kg (3.7 MBq/kg), with a minimum dose of 1 mCi (37 MBq). RDS was performed using the F+0 protocol. The differential renal function, Tmax, T1/2, drainage pattern, and hepatic uptake of the radiotracer were recorded and compared. A Bland-Altman plot was used to assess agreement between the two radiotracers. Sixteen children were included in the study. A total of 18 obstructed and 14 normal renal units were available to us for study. The values of differential renal function as well as Tmax and T1/2 of the two radiotracers were in agreement. In three obstructed kidneys in which T1/2 on Tc-MAG3 was greater than 20 min, Tc-L,L-EC showed T1/2 values of 13.3 min or less. Tc-L,L-EC showed nonobstructive drainage in three patients who had shown partial obstruction on Tc-MAG3 scan. The hepatic uptake of Tc-L,L-EC was also lower compared with Tc-MAG3. To conclude Tc-L,L-EC is a useful radiotracer for the evaluation of children with PUJ obstruction, with better assessment of drainage and lower hepatic uptake compared with Tc-MAG3.

The case of a 16-year-old male patient with left renal artery stenosis due to fibromuscular dysplasia (FMD) and an atrophic kidney due to a right multicystic dysplastic kidney (MCDK) who presented with refractory hypertension is reported. On continuous Doppler imaging, the peak systolic velocity (PSV) at the stenotic site of the left renal artery was 404 cm/s. The FMD formed a "string-of-beads" appearance on computed tomographic angiography (CTA) and renal artery angiography. Percutaneous transluminal renal angioplasty (PTRA) with a balloon catheter was performed, after which the left renal artery returned to near normal, and his blood pressure decreased to within the normal range. In this case, the main cause of refractory hypertension was considered to have been renal artery stenosis due to left FMD.

Objective: Multicystic dysplastic kidney (MCDK) is one of the most common anomalies detected by prenatal ultrasound. Our objective was to identify factors associated with severe adverse neonatal outcomes of prenatally diagnosed MCDK STUDY DESIGN: A retrospective review of prenatally diagnosed MCDK (1 January 2009 to 30 December 2014) from a single academic center was conducted. The primary outcome was death or need for dialysis among live-born infants. Associations between prenatal characteristics and outcome were analyzed by Fisher's exact test and Mann-Whitney test., Results: A total of 53 cases of prenatally suspected MCDK were included, of which 46 cases were live-born and confirmed postnatally (38 survivors, 8 non-survivors). Prenatally diagnosed extrarenal anomalies, bilateral MCDK, contralateral renal anomalies, and anhydramnios were significantly associated with death or need for dialysis (all p < 0.0001)., Conclusions: Prenatally identified findings are associated with adverse neonatal outcome, and can guide counseling and management planning. In the absence of significant associated findings, prenatally diagnosed unilateral MCDK has a benign neonatal course.

Background: Pelvic dystopia of rudimentary multicystic dysplastic kidney as a rare cause of bedwetting in children., Case Presentation: We report the case of a 14-year-old Kazakh girl who presented with difficulty in starting the stream of urine and intermittent interruption of the urinary stream while voiding as well as bedwetting, caused by a rare congenital disease (pelvic dystopia of rudimentary multicystic dysplastic kidney). The diagnostic workup, differential diagnosis, and management, and a review of the literature are presented. Persistent since she was 2 years old, bedwetting was stressful for both the parents and child. Initially detected radiologically and endoscopically, a bladder mass was thought suspicious for ureterocele, papilloma, or mixed tumor of the urinary bladder, but surprisingly, turned out to be a pelvic dystopia of the rudimentary multicystic dysplastic kidney. Transvesical excision of this mass was performed., Conclusions: The purpose of this case report is to draw attention to the fact that a persistent case of bedwetting which does not respond to conventional therapy should be subject to further examinations to exclude surgical causes of the disease.

Purpose: We sought to define features that describe the dynamic information in diuresis renograms for the early detection of clinically significant hydronephrosis caused by ureteropelvic junction obstruction., Materials and Methods: We studied the diuresis renogram of 55 patients with a mean ± SD age of 75 ± 66 days who had congenital hydronephrosis at initial presentation. Five patients had bilaterally affected kidneys for a total of 60 diuresis renograms. Surgery was performed on 35 kidneys. We extracted 45 features based on curve shape and wavelet analysis from the drainage curves recorded after furosemide administration. The optimal features were selected as the combination that maximized the ROC AUC obtained from a linear support vector machine classifier trained to classify patients as with or without obstruction. Using these optimal features we performed leave 1 out cross validation to estimate the accuracy, sensitivity and specificity of our framework. Results were compared to those obtained using post-diuresis drainage half-time and the percent of clearance after 30 minutes., Results: Our framework had 93% accuracy, including 91% sensitivity and 96% specificity, to predict surgical cases. This was a significant improvement over the same accuracy of 82%, including 71% sensitivity and 96% specificity obtained from half-time and 30-minute clearance using the optimal thresholds of 24.57 minutes and 55.77%, respectively., Conclusions: Our machine learning framework significantly improved the diagnostic accuracy of clinically significant hydronephrosis compared to half-time and 30-minute clearance. This aids in the clinical decision making process by offering a tool for earlier detection of severe cases and it has the potential to reduce the number of diuresis renograms required for diagnosis., (Copyright © 2018 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Introduction: Ureteropelvic junction obstruction (UPJO) is one of the most frequent urological diseases affecting the pediatric population. It can be due to both intrinsic stenosis of the junction and extrinsic causes such as the presence of crossing vessels (CVs), which can be detected by color Doppler ultrasound (CD-US). Magnetic resonance urography (MRU) is a good alternative, but sedation and infusion of a contrast agent are required., Objective: The aim of this study was to analyze the diagnostic accuracy of CD-US and MRU in visualizing CVs in pediatric hydronephrosis, in order to decide the correct diagnostic pathway in the pre-operative phase., Material and Methods: A retrospective review was performed of medical records for all patients who underwent surgical treatment for hydronephrosis from August 2006 to February 2016. Ultrasound and scintigraphy had been performed on all patients. Data about CD-US and MRU were collected. A high-level technology ultrasound scanner and a 1.5 T MR scanner were used. The presence of CVs at surgery was considered the gold standard. Sensitivity, specificity, positive and negative predictive values (NPV) were calculated and reported for both of the imaging techniques., Results: A total of 220 clinical charts were reviewed. Seventy-three CVs were identified at surgery (33.2% of UPJO). The median age was statistically higher in the group with CVs compared to the group without CVs (P < 0.001). The sensitivity and NPV of CD-US in detecting CVs were higher than MRU (sensitivity 93.3% vs. 71.7%, NPV 95.7% vs. 77.6%, respectively)., Discussion: According to the data, CD-US had higher sensitivity and NPV than MRU, resulting in superior detection of CVs. It is important for a surgeon to know that a child has a CV, especially in older children in which the incidence of extrinsic UPJO is higher. The main limitation of this study was the presence of incomplete data, due to the retrospectivity., Conclusions: In the pre-operative phase, the CD-US should be considered as the investigation of choice to detect CVs in children with hydronephrosis (Summary Fig). Moreover, CD-US has lower costs than MRU, and sedation with infusion of contrast agent is unnecessary. For the future, it could be useful to lead a prospective comparison between the two imaging techniques., (Copyright © 2017 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Congenital pelviureteric junction obstruction (PUJO) is one of the most frequent causes of neonatal hydronephrosis. Obstruction at the PUJ has potential severe adverse outcomes, such as renal damage. While pyeloplasty has been established as the definitive treatment, the exact pathophysiology of congenital PUJO remains unknown. Recent research has proposed neuronal innervation defects as an etiological factor in congenital PUJO. We aim to study the expression of various neuronal markers in PUJO specimens compared with controls, and evaluate whether severity of renal disease or dysfunction pre-operatively is related to expression of neuronal markers in resected PUJO specimens., Materials and Methods: All consecutive patients who underwent dismembered pyeloplasty at KK Women's and Children's Hospital, Singapore, for intrinsic PUJO from 2008 to 2012 were included. Patients with other co-occurring renal pathologies were excluded. Controls were obtained from nephrectomy patients with Wilm's tumor or other benign renal conditions during the same period. Specimens were stained immunohistochemically with neuronal markers protein gene product 9.5 (PGP9.5), synaptophysin, and S-100, and with CD-117, a marker for interstitial cells of Cajal (Table). Levels of expression of the markers were assessed semiquantitatively (decreased, increased or no change) in comparison with controls by two independent observers. Pre-operative data of patients' renal anatomical (ultrasonography measurements of renal pelvis size) and functional parameters (differential renal function measured using MAG-3 renal scans) were obtained., Discussion: Thirty-eight PUJO specimens (38 renal units) and 20 controls were studied. Mean patient age at pyeloplasty was 25.3 months (2.9-167.6 months). Median pre-operative pelvic size was 25.0 mm (17.0-50.0 mm). Both PUJO specimens and controls showed great heterogeneity in distribution of innervation. All four immunohistochemical markers were not predictive of significant pre-operative renal pelvis dilation or pre-operative diminished renal function of the operated kidney., Conclusions: There exists marked variability in expression of neuronal markers synaptophysin, PGP9.5, and S-100, and CD-117 in PUJO specimens compared with controls. Our results show no clinical significance of the expression of neuronal markers in predicting degree of pre-operative renal pelvis dilation or differential renal function. The heterogeneity of expression of neuronal markers in PUJO specimens and controls in our population is at variance with prior studies. The etiology of PUJO is likely to be complex and multifactorial., (Copyright © 2017 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Ureteropelvic junction obstruction (UPJO) is the most common cause of hydronephrosis in children. One major challenge in the management of UPJO is to select the patients that must be subjected to early obstruction relief. Currently, there is no gold standard for this assessment. Therefore, the aim of the present study was to evaluate the urinary levels of carbohydrate antigen (CA) 19-9 and normalized CA 19-9 (Ca 19-9/Cr ratio) in UPJO patients before and after surgery and compare them with a control group to assess their potential clinical application as an assisting tool in diagnosis of UPJO patients., Material and Methods: From Jan 2013 to Jun 2016, 30 children with history of inguinal hernia, circumcision, hydrocele, and undescended testis as the control group (group 1) and 30 children with unilateral congenital UPJO (group 2) were enrolled in the study. Random CA 19-9 and random creatinine were measured in the voided urine samples of control group and proven congenital UPJO group preoperatively (group 2A) and at 6 months after dismembered pyeloplasty (group 2B). In addition, the random urinary CA 19-9/CR ratio was evaluated as a marker to normalized urinary CA 19-9., Discussion: The urinary CA 19-9/Cr ratio was significantly greater in the UPJO group than in the control group. The urinary CA 19-9 also was more in group 2A than in group 1; however, it was not statistically significant. The urinary CA 19-9/Cr ratio and renal pelvis anteroposterior diameter decreased significantly in the group 2B 6 month after complete relief of obstruction in comparison with group 2A preoperatively. Urinary CA 19-9 also decreased in 2B group though it was not significant., Conclusion: Urinary CA 19-9/Cr ratio is suggested as a non-invasive marker that can assist in diagnosis and long-term follow-up of UPJO patients. This ratio is superior to urinary CA 19-9 as it is more strongly correlated with UPJ obstruction., (Copyright © 2017 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: We evaluated predictive factors for compensatory hypertrophy and renal outcomes in a large cohort of patients with multicystic dysplastic kidneys., Materials and Methods: We conducted a retrospective review from 1997 to 2016. Contralateral kidney and multicystic dysplastic kidney length were recorded from all ultrasounds as well as creatinine when available. We used generalized estimating equations to determine predictors of contralateral kidney length., Results: A total of 443 children with multicystic dysplastic kidneys were identified based on sonographic findings and lack of function on nuclear scan. Average followup was 3.2 years (IQR 1.5 to 5.7). Median time to involution in patients diagnosed before age 2 years was 5.5 years (95% CI 3.8-7.0). In all patients the median time to contralateral hypertrophy was 2.7 years (95% CI 2.2-3.3), and 90% of patients manifested contralateral hypertrophy by 10 years. After adjusting for age, gender, multicystic dysplastic kidney side and cohort status for each year of involution after age 2 years, the contralateral kidney grows 0.35 cm longer (95% CI 0.01-0.68, p = 0.04) compared to cases without involution. Patients with contralateral hypertrophy had greater creatinine clearance at followup (83 vs 61 ml per minute, p = 0.07), although this finding was not statistically significant due to limited data., Conclusions: The majority of children with multicystic dysplastic kidneys will have contralateral hypertrophy by age 3 years. Multicystic dysplastic kidney involution predicts contralateral kidney growth rate after age 2 years. A small cohort of patients with multicystic dysplastic kidneys will not exhibit contralateral hypertrophy and may be at risk for renal insufficiency., (Copyright © 2018 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Background and Objectives: Joubert syndrome is a genetically heterogeneous ciliopathy associated with >30 genes. The characteristics of kidney disease and genotype-phenotype correlations have not been evaluated in a large cohort at a single center., Design, Setting, Participants, & Measurements: We evaluated 97 individuals with Joubert syndrome at the National Institutes of Health Clinical Center using abdominal ultrasonography, blood and urine chemistries, and DNA sequencing., Results: Patients were ages 0.6-36 years old (mean of 9.0±7.6 years old); 41 were female. Mutations were identified in 19 genes in 92 patients; two thirds of the mutations resided in six genes: TMEM67 , C5orf42 , CC2D2A , CEP290 , AHI1 , and KIAA0586 . Kidney disease was detected in 30%, most commonly in association with the following genes: CEP290 (six of six), TMEM67 (11 of 22), and AHI1 (three of six). No kidney disease was identified in patients with mutations in C5orf42 (zero of 15) or KIAA0586 (zero of six). Prenatal ultrasonography of kidneys was normal in 72% of patients with kidney disease. Specific types of kidney disease included nephronophthisis (31%), an overlap phenotype of autosomal recessive polycystic kidney disease/nephronophthisis (35%), unilateral multicystic dysplastic kidney (10%), and indeterminate-type cystic kidney disease (24%). Early-onset hypertension occurred in 24% of patients with kidney disease. Age at ESRD ( n =13) ranged from 6 to 24 years old (mean of 11.3±4.8 years old)., Conclusions: Kidney disease occurs in up to one third of patients with Joubert syndrome, most commonly in those with mutations in CEP290 , TMEM67 , and AHI1 . Patients with mutations in C5orf42 or KIAA0586 are less likely to develop kidney disease. Prenatal ultrasonography is a poor predictor of kidney involvement in Joubert syndrome. Unilateral multicystic dysplastic kidney and autosomal recessive polycystic kidney disease-like enlarged kidneys with early-onset hypertension can be part of the Joubert syndrome kidney phenotype., (Copyright © 2017 by the American Society of Nephrology.)

Objective: To evaluate outcomes of congenital solitary functioning kidney (SFK) in early childhood., Study Design: A retrospective study of 32 children diagnosed in utero with SFK owing to unilateral renal agenesis or multicystic dysplastic kidney and followed for 1 to 11.5 years., Results: SFK length was in the compensatory hypertrophy range in 45% of fetal sonographic evaluations from mid-pregnancy, and in 85% on postnatal follow-up. Glomerular filtration rate was below normal range in 44.4%, 12.5% and 0% at <1 year, age 1 to 3 years and thereafter, respectively. Hyperfiltration was detected in 18.5% and 82.6% at <1 year and >3 years, respectively. Hypertension was documented in 35% at age 1 to 3 years but in none at an older age. Proteinuria was absent in all children., Conclusion: Congenital SFK is apparently associated with little or no renal damage in infancy or childhood. Compensatory enlargement of the functioning kidney begins in utero and might serve as a prognostic indicator for normal renal function after birth.

Objectives: To establish the diagnostic accuracy of obstetric ultrasound at a tertiary fetal medicine center in the prenatal detection of unilateral and bilateral multicystic dysplastic kidney (MCDK) in fetuses in which this condition was suspected, and to undertake a systematic review of the relevant literature., Methods: This was a retrospective observational study of all cases referred to a regional tertiary fetal medicine unit due to suspicion of either unilateral or bilateral MCDK between 1997 and 2015. Diagnosis was confirmed by postnatal ultrasound reports or postmortem examination. The accuracy of prenatal ultrasound in the diagnosis of MCDK was calculated. Using a systematic search strategy we also performed a review of the literature regarding the prenatal diagnosis and diagnostic accuracy of MCDK., Results: We included 144 women in our analysis; 37 (25.7%) opted for pregnancy termination (TOP) (due to unilateral MCDK with additional abnormalities, suspected bilateral MCDK or severe obstructive uropathy). Complete pre- and postnatal data were available in 126 pregnancies, including 104 livebirths, 19 TOPs with postmortem findings available and three intrauterine fetal deaths. Two infants died shortly after birth (due to known bilateral MCDK or known cranial vault defect). The overall number of cases of MCDK confirmed postnatally was 100; of these, 98 were diagnosed prenatally (true positive), while two were thought to be hydronephrosis prenatally (false negative) and the diagnosis of MCDK was made after birth. In nine cases, the initial antenatal diagnosis of suspected MCDK was revised, either later in pregnancy (n = 2) or postnatally (n = 7) (false positive). Overall, the diagnostic accuracy in our population for the use of antenatal ultrasound to detect MCDK was 91.3%, while that reported in the existing literature was found to range from 53.3% to 100%. MCDK was isolated in the majority (71%) of cases, while in 29% of cases it was found to be associated with other renal and extrarenal fetal abnormalities., Conclusions: Antenatal ultrasound had a diagnostic accuracy of about 91% in the prediction of postnatal MCDK and can therefore be used to guide antenatal counseling. However, prenatal or postnatal revision of the diagnosis occurred in about 7% of cases and parents should be counseled appropriately. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.)

The postnatal treatment of asymptomatic unilateral hydronephrosis due to ureteropelvic junction obstruction remains controversial, and the timing of and indications for surgical intervention are continuously debated. There is no consensus on the best follow-up during expectant management. The various modalities and parameters have been discussed along with their pros and cons and an attempt has been made to clear up the controversies.

Objectives: To assess the need for implementing renal scintigraphy in the diagnosis of the multicystic dysplastic kidney (MCDK) and the safety of its conservative treatment., Material and Methods: A retrospective study of patients with unilateral MCDK was conducted at our centre from January 2005 to August 2015. We calculated the positive predictive value (PPV) of ultrasonography, taking renal scintigraphy and pathology as the gold standard. We calculated a survival curve according to the Kaplan-Meier method to assess the annual probability of spontaneous resolution of the multicystic kidney., Results: Fifty-six patients were identified, 48 (85.7%) of whom had a prenatal diagnosis. Thirty eight (67.9%) of the patients were males, and the left side was affected in 33 (58.9%) of the patients. We observed associated urological abnormalities in 22 (39.29%) patients, with vesicoureteral reflux the most common (8, 14.29%). Seven patients (12.5%) developed renal failure. Forty-nine (87.5%) patients developed compensatory contralateral renal hypertrophy. Of the 33 patients who underwent surgery, the pathology results confirmed the MCDK diagnosis in 32. Compared with scintigraphy and pathology, the PPV of ultrasonography was 100% and 97%, respectively. The rate of spontaneous involution was 5.4% at 3 months of life, 11.3% at 2 years and 38.4% at 5 years., Conclusions: In our experience, the conservative treatment of MCDK, until at least 5 years of age, is safe. Our data suggest that performing scintigraphy is not required for these patients, which means lower radiation exposure, as well as financial savings., (Copyright © 2016 AEU. Publicado por Elsevier España, S.L.U. All rights reserved.)

Crossed renal ectopia is a rare occurrence. The majority of the crossed units are fused to their ipsilateral mate. Pelvi-ureteric junction obstruction (PUJO) in crossed fused moieties has been reported very rarely. Similarly, malignancy arising in the crossed over moieties is a rare occurrence too. Only a few cases have been reported earlier. We are here reporting an unusual case with coexistent PUJO and renal cell carcinoma in the crossed over moiety of right to left crossed fused ectopia. To the best of our knowledge, this is the first reported case with both these pathologies, coexisting, in the crossed over moiety. The patient underwent open radical nephrectomy of the right moiety via a lower midline incision. Surgery in this scenario is very challenging due to complex anatomy with aberrant vasculature, which needs for dissection within the renal parenchyma, similar to nephron-sparing surgery to separate from the normal moiety., Competing Interests: Conflicts of Interest: None declared., (2016 BMJ Publishing Group Ltd.)

Objectives: To review the objective and subjective success rates of robotic-assisted laparoscopic pyeloplasty in symptomatic patients with radiographic findings suggestive of uretero-pelvic junction obstruction (UPJO), but equivocal renal scans (diuretic T1/2 <20 minutes)., Methods: We reviewed 77 patients with symptomatic UPJO, who underwent robotic-assisted laparoscopic pyeloplasty between August 2006 and March 2013. We grouped patients by renal scan findings into 1 of 2 groups, obstructed (diuretic T1/2 ≥20 minutes) or equivocal (diuretic T1/2 <20 minutes). All patients were symptomatic and had radiographic findings suggestive of UPJO (eg hydronephrosis)., Results: Mean age was 40.7 years (range 17-80) with 70% female. UPJO occurred 44% left and 56% right, with 92% presenting with flank pain. Of 77 patients, 45 had obstruction on renal scan, with 41 (91%) having resolution of obstruction postoperatively and 44 of 45 (98%) having complete resolution of their initial symptoms. Thirty-two patients had equivocal findings with mean diuretic T1/2 of 12.6 minutes (range: 5.5-19.26) on renal scan. In this latter group, patients had significantly less of a decrease in their diuretic T1/2 postoperatively (4 vs 64 minutes, P = .018) and reported less pain resolution (53% vs 98%, P ≤.001) than group 1., Conclusion: Many studies have demonstrated excellent success of pyeloplasty, with most series including patients meeting strict diagnostic criteria for obstruction. Our study examines outcomes in patients with clinically symptomatic UPJO and equivocal diuretic renography. In our cohort, equivocal patients were significantly less likely to have subjective resolution of symptoms than patients in the obstructed group., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: To assess the clinical implication of chromosomal microarray analysis (CMA) in prenatal diagnosis of MCDK., Methods: Thirty-seven cases with MCDKs detected by prenatal ultrasound were enrolled in the study; 33 cases were isolated MCDKs and four cases were non-isolated MCDKs. CMA was performed on the Affymetrix CytoScan HD platform. The frequencies of the detected CNVs were compared with 461 cases that underwent CMA for anomalies unrelated to congenital anomalies of kidney and urinary tract (CAKUT) or 124 healthy newborns as controls. All of the annotated CNVs were validated by MLPA or qPCR., Results: Pathogenic CNVs were detected in 13.5% (5/37) of MCDKs. Two 17q12 deletions, one untypical 22q11.2 deletion, and one 22q11.2 duplication were detected in four isolated MCDK cases. Duplication of 1q31.3q44 was identified in a non-isolated MCDK case. Three of the five pathogenic CNVs were inherited. We also validated eight CNVs of uncertain significance only detected in MCDKs and five CNVs with higher frequency in MCDKs., Conclusion: A substantial proportion of MCDKs were associated with pathogenic CNVs. Family members with the same CNV were asymptomatic or of different kind of renal malformations. It may be reasonable to perform CMA when MCDKs are identified prenatally. © 2016 John Wiley & Sons, Ltd., (© 2016 John Wiley & Sons, Ltd.)

Ureteropelvic junction obstruction (UPJO) is defined as an impedance in the normal flow of urine from the renal pelvis into the proximal ureter. This leads to an increase urine pressure in the pelvicalyceal system and as a consequence cause hydronephrosis and damage of renal parenchyma. Presence of anomalous vessels crossing the ureter (crossing vessels) is one of the many reasons of UPJ obstruction. We report a case of 32-year-old female patient with chronic abdominal pain and recurrent episodes of pyelonephritis in the past. Contrast enhanced URO-CT was performed in order to determine the cause of complaints. This examination revealed right pelvicalyceal system dilatation and ipsilateral UPJ obstruction. After urological consultation patient was qualified for surgery, which aimed to decompress right pelvicalyceal system by changing anatomical conditions between the ureter and presumably crossing vessels. We demonstrate in the described case the value of contrast enhanced computed tomography URO-CT) in the diagnostic process of UPJ pathology. A multitude of information that we obtain from URO-CT examination allows the surgeon to reveal the cause of abnormal urine flow and choose the optimal operating method to minimize the risk of adverse events (such as intraoperative bleeding from mutilated crossing vessels).

Most multicystic dysplastic kidneys (MCDKs) are discovered prenatally. There is no consensus regarding initial workup and appropriate follow-up. We present a 9-year-old female who was fetally diagnosed with a MCDK and without follow-up returned with an 18-cm multicystic mass. The patient underwent laparoscopic nephrectomy. Final pathology revealed a dermoid cyst arising in a pediatric kidney, which to our knowledge has not been previously described. Patients with MCDK have hypertension as a possible sequela and possible reflux to their functioning kidney. Voiding cystourethrogram seems reasonable initially, and renal ultrasound is ideally noninvasive. Focused clinical awareness of the solitary kidney is important., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: To assess the clinical characteristics and natural course of segmental multicystic dysplastic kidney (MCDK)., Methods: We retrospectively analyzed the medical records of 40 patients (43 renal units) diagnosed as having segmental MCDK between January 2002 and June 2014. Segmental MCDK was classified as typical when it was localized to the upper pole of a duplex collecting system, and otherwise as atypical. We investigated involution, associated anomalies requiring surgery, and complications of segmental MCDK., Results: Of 43 renal units, 23 were typical and 20 were atypical. During 71.5 (interquartile range: 37.5-84.1) months of median follow-up period, complete and partial involution were observed in 48% and 26% of the typical group and 30% and 35% of the atypical group, respectively. In the typical group, involution was complete significantly earlier (P = .048) and ipsilateral anomalies were more frequently observed (P = .002). The initiation point of involution and contralateral anomalies were not different in the two groups. Hypertension developed in 1 case with contralateral MCDK and 1 case with contralateral renal agenesis., Conclusion: Segmental MCDK is not a rare disease entity, and conservative treatment appears to be sufficient with thorough follow-up, including regular monitoring of blood pressure and renal function, and with ultrasonography., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: To analyze the efficiency of diuretic renography in the management of unilateral ureteropelvic junction obstruction (UPJO) patients, by observing the affected kidney relative renal function (RRF) and drainage in the period of follow-up., Methods: In the study, 76 patients diagnosed as unilateral UPJO were retrospectively collected. Diuretic renography was performed on all the patients initially, and during the period of follow-up. No morphological or functional abnormalities were detected on the contralateral kidney. Changes of affected renal RRF and drainage were observed during the follow-up period. The correlations of initial RRF (RRFinitial) and drainage type with RRF improvement were analyzed., Results: In the operative group (57 cases), the RRFinitial of affected kidney was 40.81%±12.96%, and the RRF in the last follow-up (RRFrecent) was 44.63%±13.21% (P<0.05). Drainage improvements was found in 54.00% of the obstructive patients (27/50), and unchanged in 71.43% of the non-obstructive patients (5/7). In the conservative group (19 cases), the RRFinitial was 46.47%±12.84%, and the RRFrecent was 46.95%±11.86% (P>0.05). One obstructive patient (1/10) was found with improved drainage, and the other 9 obstructive patients (9/10) and all of the non-obstructive patients (9/9) were observed with unchanged drainage. Four patients with deteriorated RRF in the conservative group received surgery. There were no significant differences in the changes of affected renal RRF in different RRFinitial and drainage types in both operative and conservative groups., Conclusion: Diuretic renography could be effectively applied in the follow-up of unilateral UPJO patients. Operation could improve affected kidney's RRF, and better some patients' drainage conditions. However, for those patients with no or minor clinical symptoms, conservative management could be accepted if RRF remains stable during the period of follow up.

Purpose: The main criteria used for deciding on surgery in children with presumed antenatally detected pelviureteric junction obstruction (PPUJO) are the level of hydronephrosis (ultrasonography), the level of differential renal function (DRF) and the quality of renal drainage after a furosemide challenge (renography), the importance of each factor being far from generally agreed. Can we predict, on the basis of ultrasound parameters, the patient in whom radionuclide renography can be avoided?, Methods: We retrospectively analysed the medical charts of 81 consecutive children with presumed unilateral PPUJO detected antenatally. Ultrasound and renographic studies performed at the same time were compared. Anteroposterior pelvic diameter (APD) and calyceal size were both divided into three levels of dilatation. Parenchymal thickness was considered either normal or significantly decreased. Acquisition of renograms under furosemide stimulation provided quantification of DRF, quality of renal drainage and cortical transit., Results: The percentages of patients with low DRF and poor drainage were significantly higher among those with major hydronephrosis, severe calyceal dilatation or parenchymal thinning. Moreover, impaired cortical transit, which is a major risk factor for functional decline, was seen more frequently among those with very severe calyceal dilatation. However, none of the structural parameters obtained by ultrasound examination was able to predict whether the level of renal function or the quality of drainage was normal or abnormal. Alternatively, an APD <30 mm, a calyceal dilatation of <10 mm and a normal parenchymal thickness were associated with a low probability of decreased renal function or poor renal drainage., Conclusion: In the management strategy of patients with prenatally detected PPUJO, nuclear medicine examinations may be postponed in those with an APD <30 mm, a calyceal dilatation of <10 mm and a normal parenchymal thickness. On the contrary, precise estimation of DRF and renal cortical transit should be performed in patients with APD >30 mm, major calyceal dilatation and/or parenchymal thinning.

Purpose: Equivocal ureteropelvic junction obstruction refers to clinical symptoms and/or other radiological suggestions of possible ureteropelvic junction obstruction but with inconclusive results of obstruction on diuretic renogram. We evaluated long-term outcomes in patients with equivocal ureteropelvic junction obstruction treated with minimally invasive pyeloplasty., Materials and Methods: We retrospectively analyzed the records of 125 consecutive patients who underwent minimally invasive pyeloplasty as performed by a single surgeon from May 2004 to July 2013. Of 98 patients with followup those with more than 6-month followup were included in analysis. Equivocal ureteropelvic junction obstruction, defined as half-life less than 20 minutes on diuretic renogram, was identified in 23 patients. All patients underwent transperitoneal minimally invasive pyeloplasty. We evaluated patient demographics, preoperative and postoperative symptoms and renal function., Results: The 16 female and 7 male patients with equivocal ureteropelvic junction obstruction had flank pain and associated hydronephrosis on imaging. At a median followup of 20.2 months (range 7 to 75) 95.7% of patients with equivocal obstruction achieved complete symptom resolution. Mean ± SD preoperative and postoperative half-life was 14.1 ± 3.7 and 7.4 ± 4.2 minutes, respectively, for an improvement of 6.7 minutes (p < 0.001). In 1 patient (4.3%) with equivocal obstruction of a complicated iatrogenic etiology treatment ultimately failed postoperatively and endopyelotomy was required. There was no statistically significant difference in clinical or radiological success between the equivocal obstruction group and the 75 patients treated with minimally invasive pyeloplasty for definitive ureteropelvic junction obstruction (p = 0.44 and 0.07, respectively)., Conclusions: In patients with radiographic equivocal ureteropelvic junction obstruction and flank pain minimally invasive pyeloplasty efficaciously provides symptomatic relief and functional preservation. Results are comparable to those in patients with high grade obstruction., (Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)