Fetal bladder morphology as a predictor of outcome in fetal lower urinary tract obstruction.

OBJECTIVE: We evaluate survival of fetuses with severe Lower Urinary Tract Obstruction (LUTO) based on bladder morphology. We hypothesize that fetuses with a "floppy" appearing bladder on initial prenatal ultrasound will have worse infant outcomes than fetuses with full/rounded bladders. METHOD: We retrospectively reviewed all cases of LUTO evaluated in our fetal center between January 2013 and December 2021. Ultrasonographic assessment, renal biochemistry, and bladder refilling contributed to a "favorable" or "unfavorable" evaluation. Bladder morphology on initial ultrasound was classified as "floppy" or "full/rounded." Vesicoamniotic shunting was offered for favorably evaluated fetuses. Baseline demographics, ultrasound parameters, prenatal evaluations of fetal renal function, and infant outcomes were collected. Fetuses diagnosed with severe LUTO were included in analysis using descriptive statistics. The primary outcome measured was survival at 6 months of life. RESULTS: 104 LUTO patients were evaluated; 24 were included in analysis. Infant survival rate at 6 months was 60% for rounded bladders and 0% for floppy bladders (p = 0.003). Bladder refill adequacy was lower in fetuses with floppy bladders compared with rounded bladders (p value < 0.00001). CONCLUSION: We propose that bladder morphology in fetuses with severe LUTO may be a prognostication factor for predicting infant outcomes and provides a valuable, noninvasive assessment tool.

The utility of gene sequencing in identifying an underlying genetic disorder in prenatally suspected lower urinary tract obstruction.

OBJECTIVE: Fetal megacystis generally presents as suspected lower urinary tract obstruction (LUTO), which is associated with severe perinatal morbidity. Genetic etiologies underlying LUTO or a LUTO-like initial presentation are poorly understood. Our objectives are to describe single gene etiologies in fetuses initially ascertained to have suspected LUTO and to elucidate genotype-phenotype correlations. METHODS: A retrospective case series of suspected fetal LUTO positive for a molecular diagnosis was collected from five centers in the Fetal Sequencing Consortium. Demographics, sonograms, genetic testing including variant classification, and delivery outcomes were abstracted. RESULTS: Seven cases of initially prenatally suspected LUTO-positive for a molecular diagnosis were identified. In no case was the final diagnosis established as urethral obstruction that is, LUTO. All variants were classified as likely pathogenic or pathogenic. Smooth muscle deficiencies involving the bladder wall and interfering with bladder emptying were identified in five cases: MYOCD (2), ACTG2 (2), and MYH11 (1). Other genitourinary and/or non-genitourinary malformations were seen in two cases involving KMT2D (1) and BBS10 (1). CONCLUSION: Our series illustrates the value of molecular diagnostics in the workup of fetuses who present with prenatally suspected LUTO but who may have a non-LUTO explanation for their prenatal ultrasound findings.

Advancement of 3D printing technology for the development of a training model in US-guided vesicoamniotic shunting for early LUTO therapy.

INTRODUCTION: Prenatal lower urinary tract obstruction (LUTO) is a rare and challenging condition with potential severe morbidity and mortality. Prenatal shunting methods, specifically vesicoamniotic shunting (VAS) and fetal cystoscopy, aim to manage this condition. However, comprehensive education and training are hindered by the rarity of LUTO. To address this gap, we present a low-cost 3D-printed ultrasound training model for VAS in LUTO fetuses. The aim of the study was to evaluate ultrasound and haptic fidelity of the model. MATERIAL AND METHODS: Ultrasound images of three LUTO fetuses at 12-14 weeks were utilized to create detailed 3D-printed models. Fusion360TM software generated stereo-lithography files, and the Formlabs Form3® printer, using Flexible 80A resin, produced the models. A simulation box mimicking uterine conditions and fetal anatomy was developed for testing. Ultrasound assessments determined model accuracy, and expert evaluations gauged fidelity for VAS placement. RESULTS: The 3D-printed model accurately replicated LUTO fetal anatomy, demonstrating structural integrity and realistic sonographic and haptic feedback during 20 punctures. Macroscopic visualization confirmed the model's durability and authenticity. DISCUSSION: This innovative 3D-printed model addresses the scarcity of LUTO cases and the lack of realistic training tools. Simulation models enhance skills, providing a controlled learning environment that bridges theoretical knowledge and clinical application, potentially improving patient outcomes. CONCLUSIONS: The 3D-printed training model for VAS in LUTO represents a significant advancement in surgical education, offering realistic anatomical simulation and tactile feedback. Future studies should assess its effectiveness in enhancing surgical skills and impacting patient outcomes in clinical practice.

Z-scores of fetal bladder size for antenatal differential diagnosis between posterior urethral valves and urethral atresia.

OBJECTIVE: To construct reference values for fetal urinary bladder distension in pregnancy and use Z-scores as a diagnostic tool to differentiate posterior urethral valves (PUV) from urethral atresia (UA). METHODS: This was a prospective cross-sectional study in healthy singleton pregnancies aimed at constructing nomograms of fetal urinary bladder diameter and volume between 15 and 35 weeks' gestation. Z-scores of longitudinal bladder diameter (LBD) were calculated and validated in a cohort of fetuses with megacystis with ascertained postnatal or postmortem diagnosis, collected from a retrospective, multicenter study. Correlations between anatomopathological findings, based on medical examination of the infant or postmortem examination, and fetal megacystis were established. The accuracy of the Z-scores was evaluated by receiver-operating-characteristics (ROC)-curve analysis. RESULTS: Nomograms of fetal urinary bladder diameter and volume were produced from three-dimensional ultrasound volumes in 225 pregnant women between 15 and 35 weeks of gestation. A total of 1238 urinary bladder measurements were obtained. Z-scores, derived from the fetal nomograms, were calculated in 106 cases with suspected lower urinary tract obstruction (LUTO), including 76 (72%) cases with PUV, 22 (21%) cases with UA, four (4%) cases with urethral stenosis and four (4%) cases with megacystis-microcolon-intestinal hypoperistalsis syndrome. Fetuses with PUV showed a significantly lower LBD Z-score compared to those with UA (3.95 vs 8.83, P < 0.01). On ROC-curve analysis, we identified 5.2 as the optimal Z-score cut-off to differentiate fetuses with PUV from the rest of the study population (area under the curve, 0.84 (95% CI, 0.748-0.936); P < 0.01; sensitivity, 74%; specificity, 86%). CONCLUSIONS: Z-scores of LBD can distinguish reliably fetuses with LUTO caused by PUV from those with other subtypes of LUTO, with an optimal cut-off of 5.2. This information should be useful for prenatal counseling and management of LUTO. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Case report laparoscopy-assisted pre-pubic urethrostomy as a palliative procedure for resection of distal urethral tumor in a female dog.

BACKGROUND: This paper presents the first described case of laparoscopy-assisted prepubic urethrostomy and laparoscopic resection of a tumor of the distal part of the urethra in a female dog as a palliative treatment. CASE PRESENTATION: An intact, 11 -year-old, mixed breed female dog, weighing 15 kg, was admitted with signs of urinary obstruction and difficulty with catheterization. Vaginal, rectal, and endoscopic examinations revealed a firm mass in the pelvic cavity at the level of the pelvic urethra. Ultrasound and computed tomography examination showed enlargement of the urethral wall (5.5 cm width and 3 cm thick), which was significantly restricting the patency of the urethra. The lesion affected only the distal part of the urethra without the presence of local or distant metastatic changes. The affected portion of the urethra was laparoscopically removed while performing pre-pubic urethrostomy with laparoscopy. The patient regained full consciousness immediately after the end of anesthesia, without signs of urinary incontinence. Histopathological examination of the removed urethra revealed an oncological margin only from the side of the bladder. In the period of 2.5 months after the procedure, the owner did not notice any symptoms that could indicate a postoperative recurrence, which was diagnosed three months after the procedure. CONCLUSIONS: Pre-pubic urethrostomy can be successfully performed with the assistance of laparoscopy. The use of minimally invasive surgery will allow, in selected cases, removal of the urethral tumor, and in inoperable cases, to perform a minimally invasive palliative pre-pubic urethrostomy.

Predictive accuracy of prenatal ultrasound findings for lower urinary tract obstruction: A systematic review and Bayesian meta-analysis.

BACKGROUND: Lower urinary tract obstruction (LUTO) is a rare but critical fetal diagnosis. Different ultrasound markers have been reported with varying sensitivity and specificity. AIMS: The objective of this systematic review and meta-analysis was to identify the diagnostic accuracy of ultrasound markers for LUTO. MATERIALS AND METHODS: We performed a systematic literature review of studies reporting on fetuses with hydronephrosis or a prenatally suspected and/or postnatally confirmed diagnosis of LUTO. Bayesian bivariate random effects meta-analytic models were fitted, and we calculated posterior means and 95% credible intervals for the pooled diagnostic odds ratio (DOR). RESULTS: A total of 36,189 studies were identified; 636 studies were available for full text review and a total of 42 studies were included in the Bayesian meta-analysis. Among the ultrasound signs assessed, megacystis (DOR 49.15, [15.28, 177.44]), bilateral hydroureteronephrosis (DOR 41.33, [13.36,164.83]), bladder thickening (DOR 13.73, [1.23, 115.20]), bilateral hydronephrosis (DOR 8.36 [3.17, 21.91]), male sex (DOR 8.08 [3.05, 22.82]), oligo- or anhydramnios (DOR 7.75 [4.23, 14.46]), and urinoma (DOR 7.47 [1.14, 33.18]) were found to be predictive of LUTO (Table 1). The predictive sensitivities and specificities however are low and wide study heterogeneity existed. DISCUSSION: Classically, LUTO is suspected in the presence of prenatally detected megacystis with a dilated posterior urethra (i.e., the keyhole sign), and bilateral hydroureteronephrosis. However, keyhole sign has been found to have modest diagnostic performance in predicting the presence of LUTO in the literature which we confirmed in our analysis. The surprisingly low specificity may be influenced by several factors, including the degree of obstruction, and the diligence of the sonographer at searching for and documenting it during the scan. As a result, providers should consider this when establishing the differential for a fetus with hydronephrosis as the presence or absence of keyhole sign does not reliably rule in or rule out LUTO. CONCLUSIONS: Megacystis, bilateral hydroureteronephrosis and bladder wall thickening are the most accurate predictors of LUTO. Given the significant consequences of a missed LUTO diagnosis, clinicians providing counselling for prenatal hydronephrosis should maintain a low threshold for considering LUTO as part of the differential diagnosis.

Evaluation of Sequential Urine Analysis when Selecting Candidates for Vesicoamniotic Shunting in Lower Urinary Tract Obstruction.

OBJECTIVE: The objective of our study was to assess the utility of sequential fetal urine analysis in severe lower urinary tract obstruction (LUTO) when selecting cases suitable for vesicoamniotic shunting. MATERIAL AND METHODS: This was a retrospective cohort study of cases of severe LUTO treated in our fetal medicine center from 1994 to 2013. Two fetal bladder samples were taken 24-48 h apart to assess renal function. A vesicoamniotic shunt was inserted in case of improvement in urinary biochemistry between the 2 samples. We assessed perinatal morbidity and mortality and renal function at 5 years. RESULTS: Among a total of 26 LUTO cases with sequential urine analysis, 5 showed normal urinary biochemistry, 13 were abnormal, and 8 improved between the 2 samples. These 8 cases underwent vesicoamniotic shunt placement, leading to the birth of 6/8 (75%) live infants, 5/6 (83%) of whom had normal renal function at 5 years. The 5 cases with normal biochemistry occasioned 2 neonatal deaths and 3 children with normal renal function at 5 years. Elective termination of pregnancy was requested by parents for the fetuses exhibiting abnormal biochemistry. CONCLUSION: An improvement in urinary biochemistry between 2 sequential fetal bladder punctures in severe LUTO could be an effective criterion in the selection of candidates for vesicoamniotic shunting. However, the benefit of a shunt in fetuses with normal amniotic fluid remains to be evaluated in clinical trials.

Fetal lower urinary tract obstruction: What should we tell the prospective parents?

Fetal lower urinary tract obstruction (LUTO), which often results in marked perinatal morbidity and mortality, is caused by a heterogeneous group of anatomical defects that lead to blockage of the urethra. The classic prenatal presentation of LUTO includes megacystis with hydronephrosis. While mild forms of the disease can be associated with favorable outcomes, more severe disease commonly leads to dysplastic changes in the fetal kidneys, and ultimately oligohydramnios, which can result in secondary pulmonary hypoplasia and renal failure at birth. The aim of this review is to provide practitioners with a general overview of the diagnosis and treatment of LUTO based on disease severity, along with some points to consider when counseling prospective parents of fetuses with this condition.

Etiology and management of early pregnancy renal anhydramnios: Is there a place for serial amnioinfusions?

Early pregnancy renal anhydramios (EPRA) comprises congenital renal disease that results in fetal anhydramnios by 22 weeks of gestation. It occurs in over 1 in 2000 pregnancies and affects 1500 families in the US annually. EPRA was historically considered universally fatal due to associated pulmonary hypoplasia and neonatal respiratory failure. There are several etiologies of fetal renal failure that result in EPRA including bilateral renal agenesis, cystic kidney disease, and lower urinary tract obstruction. Appropriate sonographic evaluation is required to arrive at the appropriate urogenital diagnosis and to identify additional anomalies that allude to a specific genetic diagnosis. Genetic evaluation variably includes karyotype, microarray, targeted gene testing, panels, or whole exome sequencing depending on presentation. Patients receiving a fetal diagnosis of EPRA should be offered management options of pregnancy termination or perinatal palliative care, with the option of serial amnioinfusion therapy offered on a research basis. Preliminary data from case reports demonstrate an association between serial amnioinfusion therapy and short-term postnatal survival of EPRA, with excellent respiratory function in the neonatal period. A multicenter trial, the renal anhydramnios fetal therapy (RAFT) trial, is underway. We sought to review the initial diagnosis ultrasound findings, genetic etiologies, and current management options for EPRA.

Prenatal diagnosis and postnatal outcome of anterior urethral anomalies.

OBJECTIVES: Anterior urethral anomalies (AUA) which present as anterior urethral valve, stenosis or atresia, are a rare cause for congenital urinary tract obstruction. We present our AUA prenatal diagnosis case series. METHODS: Fetuses presenting with prenatal findings suggestive for AUA according to postnatal reported clinical and imaging signs (urinary tract dilatation, dilated bladder, enlarged edematous fetal penis, dilatation of the fetal urethra and diverticula) were followed prospectively. RESULTS: Six fetuses were diagnosed with AUA. Diagnosis was confirmed upon examination of the neonate or the abortus. All cases presented with variable degrees of urinary tract dilatation. Four fetuses who presented with additional congenital anomalies of the kidneys and urinary tract (CAKUT) developed intra-uterine or early postnatal renal failure, while two isolated AUA cases have a normal renal outcome. CONCLUSIONS: AUA is a rare diagnosis. However, high index of suspicion and careful sonographic assessment of the male fetal urethra in cases referred for urinary tract dilatation may enable appropriate parent counseling, optimal prenatal surveillance and timed postnatal urological intervention. As in other lower urinary tract obstructions, future renal function seems to correlate with associated CAKUT, therefore close follow up throughout pregnancy and meticulous sonographic assessment is recommended.

Decreased biventricular myocardial deformation in fetuses with lower urinary tract obstruction.

BACKGROUND: To observe myocardial deformations in fetuses with isolated lower urinary tract obstruction (LUTO) and identify the correlation between myocardial deformation and the severity of obstruction. METHODS: The strain (S), strain rate in systole (SRs) and strain rate in diastole (SRd) of the left and right ventricles at the first examination were prospectively analyzed and compared between fetuses with isolated LUTO and gestational age (GA)-matched normal control fetuses. Multiple regression analyses were used to assess the obstructive factors for impaired strain and strain rate, and the independent variables included bladder volume, sum of the bilateral pelvic diameters, sum of the bilateral ureteral diameters, mean bilateral renal artery pulsatility index, and amniotic fluid index. RESULTS: Thirty-six fetuses with isolated LUTO and 36 normal controls were enrolled. Overall, decreased S, SRs and SRd of both ventricles were noted in fetuses with LUTO (p < 0.001). Moreover, S and SR were significantly negatively related to distended bladder volume (p < 0.001). CONCLUSIONS: Fetuses with LUTO demonstrated decreased left and right myocardial deformation, and this impaired cardiac dysfunction was correlated with the urinary bladder volume. Evaluating the myocardial deformation in fetal LUTO could provide information to aid in parental counselling and intervention monitoring.

Neurodevelopmental Outcome in Infants with Lower Urinary Tract Obstruction Based on Different Degrees of Severity.

OBJECTIVE: To determine postnatal neurodevelopmental (ND) outcomes for children with congenital lower urinary tract obstruction (LUTO) based on disease severity. METHODS: Twenty male infants with LUTO were classified prenatally as Stage 1 (normal amniotic fluid and renal function; n = 5), Stage 2 (signs of obstruction with preserved renal function; n = 13), and Stage 3 (signs of severe renal damage; n = 2). ND status was assessed using the Developmental Profile-3 test in 5 developmental domains (physical, adaptive behavior, social-emotional, cognitive, and communication). Each domain was considered to be delayed if standard scores were 2 or more SD below the mean. ND outcomes were compared between cases with an expected normal renal function (LUTO Stage 1) and those with impaired renal function (LUTO Stages 2 and 3). Results from cases with Stage 2 were also compared to those from Stage 3. ORs were calculated to predict risk for adverse ND outcome for each domain considering prenatal and postnatal factors. RESULTS: Gestational age (GA) at the diagnosis of LUTO was similar between both groups (Stage 1: 24.85 ± 7.87 vs. Stages 2 and 3: 21.4 ± 4.31 weeks; p = 0.24). Twelve of 15 cases with Stage 2 or 3 underwent vesicoamniotic shunt placement compared to none of Stage 1 fetuses (p < 0.01). No differences in GA at delivery were detected between the groups (37.9 ± 1.6 vs. 35.1 ± 3.6 weeks; p = 0.1). One of the infants in the Stage 2 and 3 groups received a kidney transplant during follow-up. One case (20%) from Stage 1 group required dialysis during the first 6 months of life, and 1 case from Stage 2 to 3 group required it during the first 6 months (p = 1.0), whereas 3 additional cases needed dialysis from 6 to 24 months (p = 0.6). Mean age at Developmental Profile 3 (DP-3) testing was 20.3 ± 12.3 months (Stage 1: 11.2 ± 8.6 vs. Stages 2 and 3: 23.4 ± 13.4 months; p = 0.07). Fifteen of the 20 patients (75%) had no ND delays. Of the 5 patients with ND delays, 4 received dialysis. No differences in ND outcomes between infants with LUTO Stage 1 and those with Stages 2 and 3 were detected except for a trend toward better physical development in Stage 1 (102.6 ± 11.6 vs. 80.7 ± 34.9; p = 0.05). Infants diagnosed with LUTO Stage 3 showed significantly lower adaptive scores than those diagnosed with Stage 2 (Stage 2: 101.9 ± 22.3 vs. Stage 3: 41.5 ± 30.4; p = 0.04) and a nonsignificant trend for lower results in physical (85.8 ± 33.0 vs. 47.5 ± 38.9; p = 0.1) and socio-emotional (94.7 ±17.9 vs. 73.5 ± 13.4; p = 0.1) domains. Infants who received dialysis showed 15-fold increased risk (95% CI 0.89-251) for delayed socio-emotional development (p = 0.06). Diagnosis of fetal renal failure increased the risk for delays in the adaptive domain 30-fold (95% CI 1.29-93.1; p = 0.03). Infants with abnormal renal function had 19 times (95% CI 1.95-292) increased risk for delays in the physical domain (p = 0.03). CONCLUSIONS: While most patients with LUTO do not exhibiting ND delays, our results support the importance of ND monitoring, especially in severe forms of LUTO, as increased severity of this condition may be associated with poorer ND outcomes.

Outcome of fetuses with lower urinary tract obstruction and normal amniotic fluid volume in second trimester of pregnancy.

OBJECTIVE: Congenital lower urinary tract obstruction (LUTO) is a rare condition with high perinatal mortality and morbidity when associated with severe oligohydramnios or anhydramnios in the second trimester of pregnancy. Severe pulmonary hypoplasia and end-stage renal disease are the underlying causes of poor neonatal outcome in these cases. However, little is known about the subset of fetal LUTO that is associated with a normal volume of amniotic fluid at midgestation. The objective of the current study was to describe the natural history, underlying causes, survival and postnatal renal function outcomes in pregnancies with fetal LUTO and normal amniotic fluid volume during the second trimester of pregnancy. METHODS: This was a retrospective study of all pregnancies with fetal LUTO and normal amniotic fluid volume in the second trimester that received prenatal and postnatal care at our quaternary care institution between 2013 and 2017. Data on demographic characteristics, fetal interventions, perinatal survival, need for neonatal respiratory support, postnatal renal function and need for dialysis at the age of 1 and 24 months were analyzed. RESULTS: Of the 18 fetuses that met the study criteria, 17 (94.4%) survived the perinatal period. Eleven (61.1%) pregnancies developed oligohydramnios in the third trimester, six of which were eligible for and underwent fetal intervention with vesicoamniotic shunt placement, which was performed successfully in all six cases. Two (11.1%) neonates required respiratory support owing to pulmonary hypoplasia. At the age of 2 years, 14 children had follow-up information available, two (14.3%) of whom had normal renal function, eight (57.1%) had developed some degree of chronic kidney disease (Stage 1-4) and four (28.6%) had developed end-stage renal disease (ESRD), including two who had already manifested ESRD in the neonatal period. CONCLUSIONS: Most fetuses diagnosed prenatally with LUTO that is associated with a normal volume of amniotic fluid at midgestation will have a favorable outcome in terms of perinatal survival and few will need long-term respiratory support. However, these children are still at increased risk for chronic renal disease, ESRD and need for renal replacement therapy. Larger multicenter studies are needed to characterize the prenatal factors associated with postnatal renal function, and to investigate the role of fetal intervention in the group of fetuses that present with late-onset oligohydramnios and evidence of preserved fetal renal function. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Fetal cardiac findings and hemodynamic changes associated with severe lower urinary tract obstruction in utero.

OBJECTIVES: To describe fetal echocardiographic findings associated with lower urinary tract obstruction (LUTO) and to compare anatomic and hemodynamic measurements between fetuses with LUTO and gestational age (GA)-matched controls, with an emphasis on quantitative indices of diastolic function and cardiac output. METHODS: This was a retrospective cohort study of fetuses diagnosed with severe LUTO with giant bladder, which underwent at least one fetal echocardiogram at our center between January 2005 and June 2018. Fetuses with major congenital heart disease were excluded. Control fetuses did not have any structural or functional abnormalities and were GA-matched to the LUTO fetuses based on the time of the first fetal echocardiogram. Cardiac anatomy and hemodynamic measurements were compared between fetuses with LUTO and controls. In infants with LUTO, serial fetal and postnatal echocardiographic data were assessed, when available, and clinical outcomes were reviewed. RESULTS: Twenty-six fetuses with LUTO and at least one fetal echocardiogram available were identified, one of which was excluded due to hypoplastic left heart syndrome, leaving 25 LUTO fetuses in the final cohort. The mean GA at the first fetal echocardiogram was 25.4 ± 5.1 weeks in the LUTO group and 25.3 ± 5.0 weeks in the control group. Common findings in fetuses with LUTO included cardiomegaly (40%), pericardial effusion (44%), right ventricular (RV) hypertrophy (64%) and left ventricular (LV) hypertrophy (48%). Compared with GA-matched controls, LUTO fetuses had lower ascending aorta Z-score (-0.10 ± 0.94 vs -0.93 ± 1.03; P = 0.02) and aortic isthmus Z-score (-0.14 ± 0.86 vs -1.62 ± 1.11; P < 0.001), shorter mitral valve inflow time indexed to cardiac cycle length (0.46 ± 0.04 vs 0.41 ± 0.06; P = 0.002), and worse (increased) LV myocardial performance index (0.39 ± 0.03 vs 0.44 ± 0.04; P < 0.001). In addition, the ratio of RV to LV cardiac index was higher in LUTO fetuses compared with controls (1.62 ± 0.13 vs 1.33 ± 0.11; P < 0.001). Of the 25 LUTO pregnancies, two were lost to follow-up, three underwent elective termination of pregnancy and three ended in intrauterine fetal demise. Four (16%) patients had mildly hypoplastic left-heart structures, comprising two with aortic arch hypoplasia and two with mitral and aortic stenosis. CONCLUSION: In addition to presenting with cardiomegaly, pericardial effusion and ventricular hypertrophy, fetuses with LUTO demonstrate LV diastolic dysfunction and appear to redistribute cardiac output as compared to control fetuses, which may contribute to the development of left-heart hypoplasia. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Urine biochemistry to predict long-term outcomes in fetuses with posterior urethral valves.

OBJECTIVE: Because the literature on the predictive value of fetal urinalysis is controversial in fetuses with lower urinary tract obstruction, we determined the best model of fetal urine biochemical markers correlated with long-term postnatal renal function based on glomerular filtration rate (GFR). METHOD: This retrospective study concerned 89 fetuses with lower urinary tract obstruction and their renal function after 10 years of age. We correlated fetal urine biochemical markers (total protein, ß2-microglobulin, sodium, chloride, glucose, calcium, and phosphorus) with GFR at 10 to 30 years of age in 89 patients with posterior urethral valves. We defined five stages of chronic kidney disease (CKD). RESULTS: Of the 89 patients, 18 (20%) are 20 years old or over. Postnatal renal function was good in 67.4% (GFR > 60 mL/min/1.73 m2 ) and poor in 17% (GFR < 30 mL/min/1.73 m2 ). All fetal urine markers differed between CKD stage 1 + 2 and CKD stage 4 + 5 (P < 0.001). ß2-microblobulin showed an 87% sensitivity for a 72% specificity. A combination of ß2-microglobulin and chloride gave the best results (93% sensitivity and 71% specificity) versus amniotic fluid volume (80% sensitivity and 73% specificity). CONCLUSION: Fetal urine biochemistry predicts long-term (10-30 years) postnatal renal function.

Posterior urethral valves: 10 years audit of epidemiologic, diagnostic and therapeutic aspects in Yaoundé gynaeco-obstetric and paediatric hospital.

BACKGROUND: The incidence of posterior urethral valve (PUV) is estimated at 1:5000-1:8000 males. It is the most common paediatric urologic urgency and the most common cause of male obstructive uropathy and chronic renal failure in children. The study aimed to describe the experience of Yaoundé gynaeco-obstetrics and paediatric hospital in the management of PUV. METHODS: Retrospectively, medical records were retrieved over a ten year period and all data recorded and analyzed for study objectives. Patients were called and evaluated for outcomes regarding morbidity and mortality. RESULTS: A total of 18 patients all males were managed over the ten year period, given prevalence of 13 cases/100,000 admissions and an admission rate of 2 per annum. The median age at presentation was 22 months and 13 (72.2%) participants presented late. Voiding urethrocystogram was done in all the participants where it showed dilated and elongated posterior urethral valves in 16 (88.9%) of the cases. Endoscopic valve ablation resulted in the relief of obstruction in all but 3 (16.7%) participants that had residual valves and 2 (11.2%) participants that had urethral stenosis. Type I valves were most common in 14 (78.0%) participants. The mean duration of follow up was 34.56 ± 21.47 months. Complications at final follow up were: 10 (55.6%) chronic renal failure, 2 (11.2%) end-stage renal failure. The case fatality rate was 5.6%. CONCLUSION: Many patients present late in our setting with already established complications. There is the need to counsel parents/guardians on the importance of long-term follow up after relief of obstruction.

Current State of Fetal Intervention for Lower Urinary Tract Obstruction.

PURPOSE OF THE REVIEW: In this article, we explore the origins of intervention of fetal lower urinary tract obstruction, and we specifically discuss the background and recent outcomes of vesicoamniotic shunt placement and fetal cystoscopy. The article seeks to provide a comprehensive overview of the field while bringing the reader quickly up to speed on the pertinent literature and the critical data that are available to guide decision-making regarding intervention. RECENT FINDINGS: Appropriate patient selection for fetal intervention remains challenging despite advances in prenatal imaging. Both a randomized controlled trial and multiple systematic reviews show evidence of a perinatal survival benefit following fetal intervention but rates of renal morbidity remain very high. Despite 30 years of research, fetal lower urinary tract obstruction remains a difficulty entity to treat. Intervention may lead to survival, but physicians and caregivers must remain alert for the distinct possibility of long-term renal morbidity in survivors.

Perinatal Outcomes of Fetal Lower Urinary Tract Obstruction After Vesicoamniotic Shunting Using a Double-Basket Catheter.

OBJECTIVES: To evaluate the perinatal survival and renal function of fetuses with lower urinary tract obstruction (LUTO) who underwent vesicoamniotic shunting using a double-basket catheter METHODS: We retrospectively reviewed 32 fetuses with LUTO who underwent vesicoamniotic shunting using a double-basket catheter between 1998 and 2013. RESULTS: Among the 32 fetuses examined, 5 died because of termination of pregnancy, and 2 died in utero. The median gestational age at diagnosis was 15.5 (range, 10.0-27.3) weeks, and that at initial shunting was 17.1 (range, 12.3-32.2) weeks. Shunt dislocation or occlusion occurred in 18 of 42 procedures (42.8%). The median gestational age at delivery for the 25 live births was 35.5 (range, 28-40) weeks. Postnatal diagnosis revealed posterior urethral valves in 15 fetuses, a cloacal anomaly in 7, and urethral stenosis in 3. Three neonatal deaths occurred, resulting in an overall perinatal survival rate of 68.8% (22 of 32). The rates of normal renal function were 40.6% (13 of 32) at 28 days and 40% (10 of 25) at 2 years after birth. The absence of oligohydramnios after shunting was the only prognostic factor for normal renal function at 2 years (P < .05). CONCLUSIONS: Vesicoamniotic shunting may be helpful for fetuses with LUTO.

Natural History of Fetal Lower Urinary Tract Obstruction with Normal Amniotic Fluid Volume at Initial Diagnosis.

OBJECTIVE: The aim of this study was to define the natural history of lower urinary tract obstruction (LUTO) with normal midgestational amniotic fluid volumes. MATERIALS AND METHODS: We performed a retrospective review of 32 consecutive patients with LUTO with normal midgestational amniotic fluid volume followed at 11 North American Fetal Therapy Network (NAFTNet) centers from August 2007 to May 2012. Normal amniotic fluid volume was defined as an amniotic fluid index (AFI) of ≥9 cm. RESULTS: The mean gestational age (GA) and AFI at enrollment were 23.1 ± 2.1 weeks and 15.8 ± 3.9 cm, respectively. The mean GA at delivery was 37.3 ± 2.8 weeks. The mean creatinine level at discharge was 1.2 ± 0.8 mg/dL. Perinatal survival was 97%. Twenty-five patients returned for serial postnatal assessment. Renal replacement therapy (RRT) was required in 32%. Development of oligohydramnios and/or anhydramnios, development of cortical renal cysts, posterior urethral valves, prematurity, and prolonged neonatal intensive care unit stay were associated with need for RRT (p < 0.05) by univariate analysis. By multivariate analysis, preterm delivery remained predictive of need for RRT (p = 0.004). CONCLUSION: Prenatal diagnosis of LUTO with normal midgestational amniotic fluid volumes is associated with acceptable renal function in the majority of patients. Approximately one-third of these children require RRT. Surrogate markers of disease severity appear to be predictive of need for RRT.

Fetal surgery for lower urinary tract obstruction: the importance of staging prior to intervention.

Fetal lower urinary tract obstruction (LUTO) is a heterogeneous pathology associated with a high morbidity and mortality due to pulmonary hypoplasia. Previously, when a fetus was diagnosed on ultrasound with LUTO, expectant care or termination was the only option; this has changed because of fetal surgical intervention. Vesicoamniotic shunts and cystoscopy are the current methods utilized to treat LUTO; however, it remains difficult to determine whether fetal prognosis favors intervention and long-term outcome follow-up has been limited. This review covers the history and current challenges of fetal intervention for obstructive uropathies. We also present the Ruano's LUTO staging system which integrates information from fetal urine analysis with ultrasound findings to assess whether a specific fetal intervention is recommended. Utilizing a standard staging system that is readily adopted by providers carrying out fetal surgery is crucial for determining the true impact on outcomes intervention has on fetuses diagnosed with LUTO.