fetal bladder outlet obstruction

Amniotic fluid (AF) is an integral part of the fetal environment and is essential for fetal growth and development. Pathways of AF recirculation include the fetal lungs, swallowing, absorption through the fetal gastrointestinal tract, excretion through fetal urine production, and movement. In addition to being a marker for fetal health, adequate AF is necessary for fetal lung development, growth, and movement. The role of diagnostic imaging is to provide a detailed fetal survey, placental evaluation, and clinical correlation with maternal conditions to help identify causes of AF abnormalities and thereby enable specific therapy...

Prenatal therapy for LUTO (Lower Urinary Tract Obstruction) is debated due to mixed results regarding postnatal renal function following fetal cystoscopy or vesicoamniotic shunting. Current literature is, however, limited by the inability to determine the cause of the obstruction using plain sonography and the lack of selection criteria for fetuses who may benefit from prenatal therapy. Fetal cystoscopy may serve as a diagnostic tool and would offer a more "physiologic" treatment for bladder outlet obstruction...

Fetal megacystis, or an enlarged fetal bladder, is most often attributed to embryological defects, occurring early in gestation. Recent investigations have demonstrated that the underlying etiology of megacystis may be more myriad than originally thought. We present the third reported patient with megacystis due to an ACTA2 Arg179 substitution variant causing Multisystemic Smooth Muscle Dysfunction Syndrome. We also provide a description of pediatric evaluation and follow up. The growing number of cases in which this ACTA2 variant has been identified in fetal megacystis suggests that molecular sequencing is an appropriate consideration, particularly prenatally, when other features of Multisystemic Smooth Muscle Dysfunction Syndrome cannot be detected...

BACKGROUND: Severe congenital lower urinary tract obstruction (cLUTO) is associated with poor postnatal outcomes, including chronic and end stage kidney disease, and high mortality. Studies of the impact of fetal intervention through vesicoamniotic shunting are marred by a device malfunction rate of up to 60%. In this study, we delineate the postnatal course and infant kidney function following definitive urinary diversion in utero . MATERIALS AND METHODS: This is a retrospective, single-center cohort study of 16 male infants who survived the fetal intervention to birth, from 2010 to 2014 at a single center...

We present a case of antenatally detected fetal megacystis caused by an obstructing posterior urethral polyp. Antenatal and postnatal ultrasounds showed bladder wall thickening and bilateral hydroureteronephrosis, most marked antenatally. A working diagnosis of posterior urethral valves was therefore made. However, further postnatal assessment with a micturating cystourethrogram (MCUG) combined with a retrograde urethrogram identified a pedunculated urethral polyp as the cause. The addition of a retrograde urethrogram as an adjunct to the MCUG in the diagnosis of posterior urethral polyp has not previously been reported, and in this case provided diagnostic confidence of this rare condition, allowing for definitive surgical planning...

Introduction The advent of novel fetal interventions has increased interest in fetal intervention for previously "lethal" anomalies such as bilateral renal agenesis or other congenital anomalies of the kidney and urinary tract (CAKUT) associated with in utero renal failure. While there have been rare reports of successful births following intervention in these cases, there is a paucity of data regarding the risks, benefits, and outcomes of intervention. To address this gap, this study presents our experience with fetal intervention for anatomic or functional renal agenesis...

Fetal and neonatal interventions (e.g., amnioinfusions, amniotic shunting, and infant dialysis) have increased survival of infants with severe Congenital Anomalies of the Kidney and Urinary Tract (CAKUT), however, outcomes vary dramatically. Our aim was to perform Whole Exome Sequencing (WES) in a unique severe CAKUT population with the goal to identify new variants that will enhance prediction of postnatal outcomes. We performed trio WES on five infants with severe CAKUT (undergoing fetal interventions and/or those who initiated renal replacement therapy (RRT) within 1 month of life) and their parents as well as three singletons...

The presence of fetal megacystis in a renal ultrasound may suggest a mechanical or functional bladder outlet obstruction, an uncommon condition with a poor outcome. OBJECTIVE: To determine prog nostic factors in fetuses with prenatal diagnosis of megacystis. PATIENTS AND METHOD: Retrospective study carried out between 2003 and 2018 in the Orient Perinatal Reference Center (CERPO), Uni versity of Chile. Prenatal and postnatal data were analyzed, as well as etiology, pulmonary hypoplasia, medical and surgical treatment, mortality, renal function, and need for renal replacement therapy...

The presence of fetal megacystis in a renal ultrasound may suggest a mechanical or functional bladder outlet obstruction, an uncommon condition with a poor outcome. OBJECTIVE: To determine prog nostic factors in fetuses with prenatal diagnosis of megacystis. PATIENTS AND METHOD: Retrospective study carried out between 2003 and 2018 in the Orient Perinatal Reference Center (CERPO), Uni versity of Chile. Prenatal and postnatal data were analyzed, as well as etiology, pulmonary hypoplasia, medical and surgical treatment, mortality, renal function, and need for renal replacement therapy...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Ehlers-Danlos syndrome (EDS) is one of the commonest inheritable connective tissue disorders (CTD) affecting one in 5000 people globally. The incidence of bladder diverticula (BD) is reported to be 1.7% seen more commonly in children without any bladder outlet obstruction. BD are associated with congenital syndromes, namely, EDS. We report a case of huge BD in pregnancy that became symptomatic as the pregnancy progressed to term with urinary retention, recurrent urinary infections and fetal malpresentation...

Posterior urethral valve (PUV) is the most common congenital cause of bladder outflow obstruction in male neonates. We report a preterm neonate with PUV who presented as nonimmune fetal hydrops with intestinal obstruction in the antenatal period. The mother of our patient is a 33-year-old woman who started her prenatal care at our hospital at 30 weeks' gestation. Her sonogram done at 32 weeks in our hospital revealed fetal hydrops. It showed polyhydramnios, mild pyelectasis of right kidney, normal left kidney, and fetal ascites...

We reviewed data from a cohort of fetuses with ureterocele diagnosed and operated prenatally in four fetal therapy centers. Inclusion criteria were (1) ureterocele confirmed on detailed fetal ultrasound examination, (2) absence of additional fetal malformations, and (3) fetal intervention to decompress the ureterocele with local institutional review boards' approval. Data on sonographic follow-up, obstetrical, neonatal outcome, and postnatal evaluation were collected. Ten cases of prenatally treated ureterocele are described...

INTRODUCTION: The Society for Fetal Urology panel section at the 2016 Fall Congress featured a multidisciplinary discussion on appropriate patient selection, the conservative versus surgical management, and postnatal renal outcome of fetuses with lower urinary tract obstruction (LUTO). SELECTION CRITERIA FOR INTERVENTION: Rodrigo Ruano shared his experience of prenatal intervention, presenting the outcome of 111 fetuses with severe LUTO treated with vesicoamniotic shunting (VAS) (n = 16), cystoscopy (n = 34) or no intervention (n = 61) in a non-randomized series...

No abstract text is available yet for this article.

Nearly all older men will experience lower urinary tract symptoms associated with benign prostatic hyperplasia (BPH), the etiology of which is not well understood. We have generated Stk11 CKO mice by conditional deletion of the liver kinase B1 (LKB1) tumor suppressor gene, Stk11 (serine threonine kinase 11) , in the fetal Müllerian duct mesenchyme (MDM), the caudal remnant of which is thought to be assimilated by the urogenital sinus primordial mesenchyme in males during fetal development. We show that MDM cells contribute to the postnatal stromal cells at the dorsal aspect of the prostatic urethra by lineage tracing...

Prenatal ultrasound detection of fetal ureterocele with bilateral hydroureteronephrosis, obstruction of the bladder outlet and progressive amniotic fluid reduction have been associated with a significant risk of end-stage chronic renal disease after birth. Fetal cystoscopic laser incision of the ureterocele, using standard 3.5-mm fetoscopic access to the amniotic cavity and the fetal bladder with the aim of relieving the bladder outflow obstruction to preserve renal function, has been reported previously in a case with a favorable outcome...

Fetal bladder outlet obstruction (BOO), most commonly caused by posterior urethral valves (PUV), remains a challenging and multi-faceted condition. Evolving techniques, and refinement in ultrasound, optics and instrumentation, have increased our rate of prenatal diagnosis, and enabled valve ablation not only in smaller newborns, but also in fetuses. Long-term outcome studies have raised our awareness of the silent damage caused by bladder dysfunction and polyuria and encouraged their proactive management. In spite of our best efforts, the proportion of boys with PUV who progress to chronic and end-stage renal disease (ESRD) has not changed in the last 25 years...

OBJECTIVES: Our objective is to report long-term outcome after fetal cystoscopy for lower urinary tract obstruction (LUTO), as well as to investigate the accuracy of fetal cystoscopy in diagnosing the cause of bladder outlet obstruction. METHODS: This is a retrospective cohort study of all fetuses who underwent cystoscopy for prenatal diagnosis of LUTO in three tertiary referral centers. Fetal diagnostic cystoscopy was performed to determine prenatally the cause of LUTO and to ablate the posterior urethral valves (PUV)...