Prenatal diagnosis and management of monozygotic twins discordant for severe fetal abnormalities.

OBJECTIVE: We present prenatal diagnosis and management of monozygotic (MZ) twins discordant for severe fetal abnormalities. CASE REPORT: A 36-year-old woman underwent amniocentesis at 18 weeks of gestation because of advanced maternal age, and hydrops fetalis, a giant cystic hygroma of 5 × 3.5 cm and left hydronephrosis in a co-twin. The other co-twin was structurally normal. Amniocentesis revealed a karyotype of 46,XY in both co-twins. Simultaneous polymorphic DNA marker analysis using the DNAs extracted from maternal blood and uncultured amniocytes confirmed MZ twinning. The woman underwent a successful selective fetal reduction by radiofrequency ablation at 22 weeks of gestation. At 28 weeks of gestation, premature rupture of membranes occurred, and a 1280-g normal male baby and a 275-g dead malformed co-twin were delivered. The normal co-twin was phenotypically normal and was doing well at age seven weeks. CONCLUSIONS: Prenatal diagnosis of MZ twins discordant for structural abnormalities should include a differential diagnosis of MZ twinning, and a zygosity test is necessary under such a circumstance.

Significance of third trimester ultrasound in detecting congenital abnormalities of kidney and urinary tract-a prospective study.

INTRODUCTION: Antenatal hydronephrosis is defined as dilatation of the renal pelvis with or without dilatation of calyces in the developing fetus. Although second trimester scan is more sensitive in detecting congenital abnormalities of kidney and urinary tract (CAKUT), it has been suggested that the third trimester scan is more predictive of postnatal outcome. OBJECTIVES: This study aims to determine the incidence of antenatal hydronephrosis at our institution and the significance of third trimester scan in detecting CAKUT postnatally. STUDY DESIGN: It is a prospective observational study. During the 3-year study period from June 2013 to May 2016, all cases of antenatal hydronephrosis, defined as renal pelvic diameter (RPD) ≥4 mm in the second trimester and ≥7 mm in the third trimester scan, diagnosed in the fetal medicine unit of our hospital, were included and were followed up postnatally for 6 months. RESULTS: In the fetal medicine unit, 32,443 women were screened for anomalies, and hydronephrosis was detected in 269 cases. Incidence of antenatal hydronephrosis was observed to be 0.83% in our institution. In second trimester scan, of 80 cases with the left hydronephrosis, only 22 (27.5%) had postnatal CAKUT and of 70 cases with the right hydronephrosis, 18 (25.7%) had CAKUT. In the third trimester scan, it was observed that with RPD >10 mm, on the left side hydronephrosis, 87% had CAKUT on postnatal scan, and 85% with the right hydronephrosis had CAKUT. CONCLUSION: Sixty percent cases of hydronephrosis detected in the second trimester scan resolve in utero. In the third trimester scan with RPD <10 mm, the possibility of postnatal CAKUT is 23%, whereas if RPD is > 10 mm, then the possibility of postnatal CAKUT is 86%.

Prenatal Urinary Tract Dilatation.

Urinary tract dilatation (UTD) is the most common congenital anomaly detected on prenatal ultrasonography (US), affecting 1% to 3% of all pregnancies. This article focuses on the prenatal detection of UTD and the postnatal evaluation and management based on the UTD grading system risk assessment. Prophylactic antibiotics and postnatal imaging are discussed. The recent management trend is for a more conservative approach to minimize unnecessary testing and exposures to the fetus and neonate while detecting those who may have clinically significant disorder. The renal bladder US remains a critical part of the evaluation and helps guide further investigations.

Isolated low grade prenatally detected unilateral hydronephrosis: do we need long term follow-up?

ABSTRACT Purpose: To assess the need for postnatal evaluation and the medium term outcome in patients with isolated unilateral low grade prenatally detected hydronephrosis. Materials and Methods: We prospectively selected 424 patients (690 kidney units) with a prenatal diagnosis of urinary tract dilatation between 2010 and 2013. We included only those patients with isolated unilateral low-grade hydronephrosis who underwent at least 2 postnatal ultrasound examinations. The Society for Fetal Urology (SFU) grading system was utilized for assessment of the hydronephrosis. We excluded patients with bilateral dilation or other urological abnormalities. The fate of hydronephrosis including resolution, stability or worsening was documented. Results: A total of 66 infants (44 boys and 22 girls) with antenatally diagnosed unilateral urinary tract dilation (23 right and 43 left) were identified. Ultrasounds showed SFU grade 1 hydronephrosis in 32 patients (48%) and SFU grade 2 hydronephrosis in 34 (52%). After a mean follow-up period of 32 months (range 12 to 60), 37 patients (56%) had complete resolution of hydronephrosis while the remaining 29 were stable (44%). None of our patients developed UTIs during follow-up and none required surgical intervention. Conclusions: Prenatally detected, isolated unilateral low-grade hydronephrosis usually have a favorable prognosis. All cases in our cohort showed either stability or resolution of hydronephrosis without any harmful consequences. Based on our findings on medium-term in this category of patients, long-term follow-up is not warranted.

Isolated low grade prenatally detected unilateral hydronephrosis: do we need long term follow-up?

PURPOSE: To assess the need for postnatal evaluation and the medium term outcome in patients with isolated unilateral low grade prenatally detected hydronephrosis. MATERIALS AND METHODS: We prospectively selected 424 patients (690 kidney units) with a prenatal diagnosis of urinary tract dilatation between 2010 and 2013. We included only those patients with isolated unilateral low-grade hydronephrosis who underwent at least 2 postnatal ultrasound examinations. The Society for Fetal Urology (SFU) grading system was utilized for assessment of the hydronephrosis. We excluded patients with bilateral dilation or other urological abnormalities. The fate of hydronephrosis including resolution, stability or worsening was documented. RESULTS: A total of 66 infants (44 boys and 22 girls) with antenatally diagnosed unilateral urinary tract dilation (23 right and 43 left) were identified. Ultrasounds showed SFU grade 1 hydronephrosis in 32 patients (48%) and SFU grade 2 hydronephrosis in 34 (52%). After a mean follow-up period of 32 months (range 12 to 60), 37 patients (56%) had complete resolution of hydronephrosis while the remaining 29 were stable (44%). None of our patients developed UTIs during follow-up and none required surgical intervention. CONCLUSIONS: Prenatally detected, isolated unilateral low-grade hydronephrosis usually have a favorable prognosis. All cases in our cohort showed either stability or resolution of hydronephrosis without any harmful consequences. Based on our findings on medium-term in this category of patients, long-term follow-up is not warranted.

Variable Resource Utilization in the Prenatal and Postnatal Management of Isolated Hydronephrosis.

OBJECTIVE: To characterize contemporary resource utilization and medical outcomes for infants with antenatal hydronephrosis and their mothers from a national claims database. We hypothesize that management of isolated hydronephrosis (IHN) varies widely, with decreased imaging following the 2010 Society for Fetal Urology Consensus Statement. MATERIALS AND METHODS: Using MarketScan claims from 2007 to 2013, we identified infants 0-12 months of age with hydronephrosis and linked mothers. Those with urologic diagnoses more specific than hydronephrosis, additional urologic comorbidities, or postnatal surgeries were excluded. Resource utilization including prenatal and postnatal imaging, laboratory studies, hospital admissions, and medical outcomes within the first year was captured. Demographics, maternal characteristics, utilization measures, and outcomes were compared across imaging intensity groups based on number of postnatal ultrasounds received using bivariate analysis. RESULTS: Among 801,919 mother-child pairs, 8610 infants (1.1%) had hydronephrosis or a related diagnosis. A total of 5876 (68.2%) met inclusion criteria for IHN. Patients underwent a mean 5.3 ± 3.5 prenatal and 2.1 ± 1.3 postnatal ultrasounds before age 1. Imaging practices were unchanged following the Society for Fetal Urology consensus statement. CONCLUSION: Antenatal hydronephrosis prevalence in an insured population is consistent with published ranges. Prenatal imaging in IHN is variable and potentially excessive. Future study into the efficacy of evidence-based pathways in reducing excess utilization is warranted.

[Laparoscopic ureterovesical repair for megaureter treatment]. / Plastia ureterovesical laparoscópica para el tratamiento del megauréter.

BACKGROUND: An alternative treatment for obstructive and refluxing obstructive megaureter with ureterovesical junction maintenance through laparoscopy is proposed. MATERIAL AND METHODS: The series consists of 8 cases, all of them studied because of prenatal hydronephrosis or febrile urinary tract infection. Seven were diagnosed with obstructive megaureter and one with obstructive refluxing megaureter. The procedure consisted in incising the stenotic portion of the ureter longitudinally and maintaining its posterior wall attached to the bladder, the anterior wall of the ureter was anastomosed transversally to the bladder mucosa in order to liberate the obstruction. RESULTS: Two of the 8 cases were women and 6 were male. They were aged between 5 months and 11 years (average age of 2.9 years). The surgical time varied between 90 and 120min, with a 48h hospital stay. The permanence of the vesical catheter and the double J stent was of 48h and 6 weeks, respectively. A female patient developed febrile urinary tract infection one week after the surgical procedure. The rest of the patients remained asymptomatic, with normal urinalysis and quarterly urine culture results. Six months after the procedure, the ultrasound showed improvement of the ureteral diameter and of the pyelocaliceal ectasia. The average follow-up was of 2 years. DISCUSSION: The handling of stenosis with longitudinal incision of the ureter and transverse anastomosis to the bladder mucosa, seems promising. The laparoscopic approach allows to identify clearly the longitude of the stenotic segment and join with relative precision such opening with the one of the vesical mucosa.

Maternal Urinary Carbohydrate Antigen 19-9 as a Novel Biomarker for Evaluating Fetal Hydronephrosis: A Pilot Study.

OBJECTIVE: To evaluate maternal urinary CA19-9 as a potential marker to diagnose severe antenatal hydronephrosis (ANH) during pregnancy and to compare the values with those in normal pregnancies as controls. PATIENTS AND METHODS: A total of 20 women in their third pregnancy trimester were enrolled. An anteroposterior pelvic diameter (APD) of ≥15 was considered as severe ANH. Case group consisted of 10 women with a diagnosis of severe ANH. Ten women with similar age, gestational age, fetal sex, normal ultrasonography, and no history of any congenital anomalies were chosen as controls. Urine samples were collected and maternal urinary CA19-9 was measured. The levels in case and control groups were compared using Mann-Whitney U test. RESULTS: Each group consisted of nine mothers with male fetuses and one with female fetus. The APD in the ANH group ranged from 17 to 40 mm. Five of 10 children in the ANH group also had contralateral APD of ≥4 mm (bilateral ANH). The mean age and gestational age of pregnant women in the two groups were comparable. The mean maternal CA19-9 was significantly higher in the ANH group compared with the controls (mean: 134.5 U/mL vs 22.2 U/mL, P < .001). CONCLUSION: To our best knowledge, this is the first time that maternal urinary CA19-9 has been used as a marker for ANH. Based on these pilot data, CA19-9 levels are significantly higher in the urine of pregnant women carrying fetuses with severe ANH, and it may have the potential to serve as a noninvasive and useful biomarker to diagnose ANH.

The Impact of Gestational Age at Delivery on Urologic Outcomes for the Fetus with Hydronephrosis.

OBJECTIVE: Compare short-term urologic outcomes with delivery timing in fetuses with severe hydronephrosis. METHODS: An ultrasound database was queried for severe hydronephrosis. Cases were categorized into late preterm/early term (36 0/7 - 38 6/7 weeks) and full term (39 0/7 weeks or greater) groups. Baseline characteristics were compared using standard statistical methods. Spearman's correlation analysis was performed for grade and severity of hydronephrosis on first postnatal ultrasound with gestational age at delivery. RESULTS: Of 589 cases, 79 (33 late preterm/early term, 46 full term) met criteria. Baseline characteristics were similar between groups. Spearman's correlation coefficients (rs) indicated that increased postnatal Society for Fetal Urology grade, rs= -0.26 (95% CI [-.48, -.002]), and severity of hydronephrosis, rs= -0.39 (95% CI [-.59, -.14]), both correlated with earlier delivery. CONCLUSION: Late preterm/early term delivery resulted in worse short-term postnatal renal outcomes. Unless otherwise indicated, delivery for fetal hydronephrosis should be deferred until 39 weeks.

Uroepithelial thickening improves detection of vesicoureteral reflux in infants with prenatal hydronephrosis.

INTRODUCTION: Postnatal evaluation of prenatal hydronephrosis (PNH) often includes a voiding cystourethrogram (VCUG) for VUR assessment. Despite limited supporting data, VCUG is currently recommended if postnatal renal and bladder ultrasound (RBUS) reveals moderate/severe hydronephrosis (HN) or hydroureter (HU). Recent studies have shown VUR is more accurately diagnosed by using certain sonographic findings as criteria for obtaining VCUG. Uroepithelial thickening (UET) of the renal pelvis is a finding associated with high-grade vesicoureteral reflux (HGVUR); however, the clinical significance of UET with PNH has not been studied. OBJECTIVE: We sought to determine if the presence of UET implies increased risk for VUR, and to investigate whether UET can improve the test characteristics of RBUS for VUR. STUDY DESIGN: We retrospectively analyzed postnatal RBUS and VCUG findings in infants ≤30 days undergoing evaluation for "prenatal hydronephrosis" over an 11-year period. We used logistic regression to identify factors associated with VUR. Test characteristics of RBUS for HGVUR were compared based on the presence of UET and two criteria sets to define abnormal RBUS. Criteria set 1 consisted of HN SFU grade 3-4 and/or HU; criteria set 2 was defined by the presence of two of following: UET, HU, duplication, and/or renal dysmorphia. RESULTS: Of 135 patients, 39 (29%) had VUR, of whom 16 (41%) had HGVUR. UET was significantly associated with VUR (p < 0.001), and the sensitivity for HGVUR based on UET alone was 94%. On multivariable analysis, UET, HU, duplication, and renal dysmorphia remained significant independent predictors of HGVUR. Compared to criteria 1, using criteria 2 resulted in 43 fewer VCUGs, and significant improvement in sensitivity and specificity for HGVUR (Table). DISCUSSION: Consistent with previous studies, HN alone on postnatal RBUS has little value in predicting the presence or severity of VUR. This study is the largest known series to evaluate UET in the setting of PNH, and our results demonstrate that UET, as well as HU, duplication and renal dysmorphia, are independent sonographic findings predicting HGVUR. Using our proposed criteria, the probability of HGVUR is fourfold more than the prevalence described in the literature, and importantly, when compared to the criteria recommended by the SFU and AUA, would have resulted in 53% fewer VCUGs while missing zero cases of HGVUR. CONCLUSION: In infants with PNH, the sonographic findings of UET, HU, duplication and renal dysmorphia independently indicate greater risk of HGVUR, and the sensitivity and specificity of RBUS for HGVUR is markedly improved when at least two of the four are present.

Urinary tract infections in children with prenatal hydronephrosis: A risk assessment from the Society for Fetal Urology Hydronephrosis Registry.

INTRODUCTION: Risk factors for urinary tract infection (UTI) in children with prenatal hydronephrosis (PNH) are not clearly defined. Our study aim was to describe incidence and identify factors associated with UTI among a cohort of children diagnosed with PNH. MATERIAL AND METHODS: Patients with confirmed PNH from four medical centers were prospectively enrolled in the Society for Fetal Urology (SFU) hydronephrosis registry between 9/2008 and 10/2015. Exclusion criteria included enrollment because of UTI, associated congenital anomalies, and less than 1-month follow-up. Univariate analysis was performed using Fisher's Exact test or Mann-Whitney U. Probability for UTI was determined by Kaplan-Meier curve. RESULTS: Median follow-up was 12 (IQR 4-20) months in 213 patients prenatally diagnosed with hydronephrosis. The majority of the cohort was male (72%), Caucasian (77%), and 26% had high grade (SFU 3 or 4) hydronephrosis. Circumcision was performed in 116/147 (79%) with known status, 19% had vesicoureteral reflux (VUR), and 11% had ureteral dilatation. UTI developed in 8% (n = 18), 89% during their first year of life. Univariate analysis found UTI developed more frequently in females (p < 0.001), uncircumcised males (p < 0.01), and the presence of parenchymal renal cyst (p < 0.05). Logistic regression found renal cyst to no longer be significant, but female gender a significant risk factor for development of UTI (p < 0.001). Regression analysis stratified by gender found neither hydronephrosis grade nor parenchymal renal cyst to be significant risk factors for UTI development among females. However, hydronephrosis grade and circumcision status were significant risk factors for development of UTI among males (p < 0.05 and p < 0.01, respectively). CONCLUSION: Identification of factors associated with UTI in patients with PNH is still progressing; however, several observational studies have identified groups that may be at increased risk of UTI. Use of prophylactic antibiotics (PA), degree of kidney dilation, gender, and circumcision status all have been reported to have some degree of impact on UTI. A previous study identified risk factors for UTI as female gender, uncircumcised status, hydroureteronephrosis, and VUR, and reported that prophylaxis provided a protective effect on prevention of UTI. Our data mirror those in some respect, identifying an association of UTI with female gender and, among males, uncircumcised status, and high grade hydronephrosis. However, we were unable to demonstrate an association between UTI and the use of PA, presence of VUR, dilated ureter, or renal duplication in this observational registry.

[Contribution of ultrasound signs for the prenatal diagnosis of posterior urethral valves: Experience of 3years at the maternity of the Bicêtre Hospital]. / Apport des signes d'appel échographiques dans le diagnostic anténatal des valves de l'urètre postérieur : expérience de 3ans à la maternité de l'hôpital Bicêtre.

OBJECTIVES: Posterior urethral valves (PUV) are the most common cause of renal impairment in boys during early childhood. The aim of this study was to evaluate the value of ultrasound (US) criteria currently used to diagnose PUV. MATERIALS AND METHODS: From 2009 to 2012, 31 patients were referred to the Bicêtre Hospital after detection of fetal bilateral hydronephrosis in male fetus. The ultrasound criteria were bladder dilation, thick-walled bladder, urethral dilation ("keyhole sign"), and amniotic fluid volume. Patients were divided in two groups: suspected or not to have PUV. RESULTS: US diagnosis of PUV was done in 18 fetuses and confirmed in 14 new borns, one of them without prenatal diagnosis. Sensitivity and specificity of US scan were 92.8 and 66.7%. The likelihood ratio (LHR) was 4.8 for a thick-walled bladder, 4.2 for oligohydramnios, 3.6 for the "keyhole sign", 2.4 for bladder dilation and 1.6 for ureteral dilation. The first four signs were combined in four fetuses, all of them with PUV. CONCLUSION: US scan is a very sensitive exam for the diagnosis of PUV but with a low specificity. A thick-walled bladder seems to have a better diagnostic performance than the "keyhole sign".

Nephric duct insertion requires EphA4/EphA7 signaling from the pericloacal mesenchyme.

The vesico-ureteric junction (VUJ) forms through a complex developmental program that connects the primordium of the upper urinary tract [the nephric duct (ND)] with that of the lower urinary tract (the cloaca). The signals that orchestrate the various tissue interactions in this program are poorly understood. Here, we show that two members of the EphA subfamily of receptor tyrosine kinases, EphA4 and EphA7, are specifically expressed in the mesenchyme surrounding the caudal ND and the cloaca, and that Epha4(-/-);Epha7(+/-) and Epha4(-/-);Epha7(-/-) (DKO) mice display distal ureter malformations including ureterocele, blind and ectopically ending ureters with associated hydroureter, megaureter and hydronephrosis. We trace these defects to a late or absent fusion of the ND with the cloaca. In DKO embryos, the ND extends normally and approaches the cloaca but the tip subsequently looses its integrity. Expression of Gata3 and Lhx1 and their downstream target Ret is severely reduced in the caudal ND. Conditional deletion of ephrin B2 from the ND largely phenocopies these changes, suggesting that EphA4/EphA7 from the pericloacal mesenchyme signal via ephrin B2 to mediate ND insertion. Disturbed activity of this signaling module may entail defects of the VUJ, which are frequent in the spectrum of congenital anomalies of the kidney and the urinary tract (CAKUT) in human newborns.

Vesicoureteral reflux in pediatric age: where are we today?

Although the diagnosis of vesicoureteral reflux and of reflux nephropathy is a well-established and shared procedure, its treatment nowadays is still very controversial. New developments on the knowledge of pathophysiology of renal damage associated to reflux opened the way towards a different diagnostic work-up and different therapeutic approaches. Recently, the "top-down" diagnostic approach has gained wider interest, versus the "down-top" protocol. The attention has recently focused on the renal parenchyma damage and less interest has been given to the presence and the radiological degree of vesicoureteral reflux. The review criteria were based on an in-depth search of references conducted on PubMed, using the terms "vesicoureteral reflux", "children", "incidence", "etiology", "diagnosis", "treatment" and "outcomes". The selection of the papers cited in this review was influenced by the content and the relevance to the points focused in the article.Conservative approaches include no treatment option with watchful waiting, long-term antibiotic prophylaxis and bladder rehabilitation. The operative treatment consists of endoscopic, open, laparoscopic and robotic procedures to stop the refluxing ureter.No final consensus has been achieved in literature yet, and further studies are necessary in order to better define the subset of children at risk of developing progression of renal damage.This review aims to clarify the diagnostic management and the urological-nephrological treatment of reflux in pediatric age, on the basis of a review of the best-published evidence.

Hydronephrosis: prenatal and postnatal evaluation and management.

Antenatal hydronephrosis (ANH) is one of the most frequently detected abnormalities found on routine prenatal ultrasounds, affecting 1% to 4.5% of all pregnancies. Despite its prevalence, there continues to be uncertainty regarding the clinical impact after birth. Prognosis depends on the severity of the dilation. Expectant prenatal management is the rule with fetal intervention rarely needed in a few select cases. Ureteropelvic junction obstruction and vesicoureteral reflux are the most common postnatal diagnoses. A renal and bladder ultrasound is essential in the follow-up of patients with ANH and helps dictate further investigation with voiding cystourethrography and/or diuretic renography.

Congenital anomalies of the urinary tract.

The upper urinary tract forms as a consequence of the reciprocal inductive signals between the metanephric mesenchyme and ureteric bud. A clue to the timing of events leading to an abnormality of the upper urinary tract can be the presence also of associated anomalies of internal genitalia since separation of these systems occurs at about the 10th week of gestation. Prenatal sonography has facilitated the detection of urological abnormalities presenting with hydronephrosis. Hydronephrosis suggests obstruction, but by itself cannot be equated with it. Instead, further radiographic imaging is required to delineate anatomy and function. Now, moreover, non-surgical management of CAKUT should be considered whenever possible. Despite the widespread use of prenatal screening sonography that usually identifies the majority of congenital anomalies of the urinary tract, many children still present with febrile urinary tract infection (UTI). Regardless of the etiology for the presentation, the goal of management is preservation of renal function through mitigation of the risk for recurrent UTI and/or obstruction. In the past many children underwent surgical repair aimed at normalization of the appearance of the urinary tract. Today, management has evolved such that in most cases surgical reconstruction is performed only after a period of observation - with or without urinary prophylaxis. The opinions presented in this section are not espoused by all pediatric urologists but represent instead the practice that has evolved at Children's National Medical Center (Washington DC) based significantly on information obtained by nuclear renography, in addition to sonography and contrast cystography.

[Comparative analysis of interuterine therapy of unilateral and bilateral fetal obstructive uropathy]. / Analiza pórownawcza wyników terapii wewnatrzmacicznej obustronnej i jednostronnej uropatii zaporowej.

OBJECTIVE: The goal of the work was a comparative analysis of intrauterine therapy among pregnant women with diagnosed fetal uropathy and unilateral hydronephrosis. METHODS: The study was conducted on a group of 98 pregnant women hospitalized at the Department of Gynecology Fertility and Fetal Therapy Polish Mother's Memorial Hospital between 2006-2012. The study group included 77 cases of fetal obstructive uropathy (79%) and 21 cases of unilateral hydronephrosis (21%). Both groups have been analyzed in terms of prognosis. Follow-up has been conducted and results have been statistically analyzed. RESULTS: In 11% of cases of obstructive uropathy therapy early complications were observed. The remaining 89% of the patients delivered at a later date, of which 54% after 34 weeks of pregnancy The average weight of a newborn was 2645g and Apgar score - 7.45. 73% of newborns were discharged home in good condition, with preserved diuresis. CONCLUSIONS: 1. The use of intrauterine therapy has a positive effect on fetal condition and newborn postnatal state. 2. Unilateral hydronephrosis group was characterized by a significantly longer duration of pregnancy higher birth weight, Apgar score, and greater survival of newborns. 3. In the group of obstructive uropathy survival improvement was achieved and dieresis was preserved Intrauterine interventions were associated with a low risk of complications.

Antenatal mild hydronephrosis with subsequent polyp of the upper ureter in a child presenting with recurrent Dietl's crisis.

We report a 7-year-old boy who had antenatally diagnosed and postnatally confirmed asymptomatic right congenital hydronephrosis secondary to ureteropelvic junction obstruction with the anteroposterior diameter ranging from 7 to 15 mm on serial ultrasound scans till the age of 6 years. He then presented with recurrent attacks of Dietl's crisis almost every month in the seventh year of his life and eluded diagnosis for over a year at various national hospitals, and ended up with an international referral to us. Investigations of the acute attacks clinched the diagnosis of ureteral polyp causing intermittent crisis and he underwent segmental resection and reconstruction in the form of dismembered Anderson-Hyne pyeloplasty with good recovery. Our case revealed that prenatally detected hydronephrosis may worsen after spontaneous postnatal improvement and a polyp acting as a flip valve may produce intermittent hydronephrosis and symptoms later in life. The child should undergo urgent investigations during acute symptoms.

Urinary tract infection and vesicoureteral reflux in children with mild antenatal hydronephrosis.

OBJECTIVE: The postnatal management of mild antenatal hydronephrosis (ANH) remains controversial. The purpose of this study was to evaluate the incidence of UTI and VUR in children with mild ANH in order to determine the necessity of antibiotic prophylaxis (ABP) and VCUG. METHOD: The data of 1511 patients with various grades of ANH who were referred to Department of Urology, Boston Children's Hospital between January 1998 and January 2010 were reviewed and 760 patients who had mild ANH were identified. The inclusion criteria were: 1) A confirmed report of ANH or actual prenatal ultrasound (US) images. 2) Postnatal evaluation and management conducted at the hospital. 3) Persistent mild hydronephrosis on the first US done between two weeks and three months of age. 4) No other US findings such as ureteral dilatation, duplication anomalies or bladder abnormalities. 5) At least one three-month follow up. Univariate statistical analysis was performed using a Student's t test. RESULTS: Of the 760 patients who were identified, 608 (80%) were males, and 225 (30%) had bilateral mild hydronephrosis. Of these, 475 patients (63%) underwent an initial screening VCUG. VUR was identified in 13 patients (1.7%) with grades varying from 1 to 5. At follow up, hydronephrosis resolved in 67% of the renal units and worsened in 3.3%. Among the 692 patients with available follow-up data, 23 (3.3%) had a documented UTI. Twelve of these children had an initial screening VCUG that was negative for VUR. Of these 12 patients, seven underwent a subsequent RNC with none having VUR; five of the 12 patients did not undergo a repeat evaluation for VUR (four had a UTI after the screening VCUG and one had an afebrile UTI). Eleven of the 23 children with mild ANH did not have an initial screening VCUG, and all underwent a subsequent VCUG/RNC. Only two children were then found to have VUR Grade 4-5. CONCLUSION: The incidence of UTI and VUR in children with mild ANH is low. Consequently, routine VCUG screening for VUR and the use of long-term ABP is not necessary for all patients with asymptomatic mild AHN. Evaluation for VUR in children with mild ANH should be reserved for those who subsequently present with a UTI.

Peroxiredoxin I protein, a potential biomarker of hydronephrosis in fetal mice exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin.

OBJECTIVE: In previous studies, we established an animal model of human congenital hydronephrosis with exposure of developing mice to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD), but the etiopathogenesis is not entirely clear. The present study was to identify the changes that may be involved in the etiology at the protein level. METHODS: C57BL/6J mice fetuses were treated with TCDD. Comparative proteomic analysis was adopted to identify the proteins associated with hydronephrosis induced by TCDD. RESULTS: Two-dimensional electrophoresis display revealed that 19 protein spots were differentially expressed in the upper urinary tract tissues in fetal mice after exposure to TCDD. Matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF-MS) identified 12 up-regulated proteins: peroxiredoxin I (Prx I), cadherin 6, gamma-actin, radixin, desmin, type II transforming growth factor-beta receptor, chromogranin B, serum albumin precursor, transferrin, hypothetical protein LOC70984, lipk protein, and zinc finger protein 336. Histochemical staining indicated that Prx I protein was positively expressed in the ureteric epithelium in the treated group, and not in the control group, which is consistent with MALDI-TOF-MS. CONCLUSION: Prx I protein may be a potential biomarker or responsive protein of hydronephrosis in fetal mice induced by TCDD.