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posterior urethral valve, pulmonary

A Faure, A Bouty, G Caruana, L Williams, T Burgess, M N Wong, P A James, M O'Brien, A Walker, J F Bertram, Y Heloury
INTRODUCTION: Posterior urethral valves (PUV) are among the most common urological causes of chronic kidney disease (CKD) in childhood. Recently, genomic imbalances have been cited as potential risk factors for altered kidney function and have been associated with CKD. The phenotypic effects of a copy number variant (CNV) in boys with PUV are unknown. Here, it was hypothesised that the progression to early renal failure in PUV patients may be influenced by genetic aberrations. OBJECTIVE: To assess the relationship between CNVs and renal outcomes...
August 2016: Journal of Pediatric Urology
José María Martínez, Narcis Masoller, Roland Devlieger, Esther Passchyn, Olga Gómez, Joan Rodo, Jan A Deprest, Eduard Gratacós
OBJECTIVE: To report the results of fetal cystoscopic laser ablation of posterior urethral valves (PUV) in a consecutive series in two referral centers. METHODS: Twenty pregnant women with a presumptive isolated PUV were treated with fetal cystoscopy under local anesthesia. Identification and fulguration of the PUV by one or several firing-contacts with diode laser were attempted. Perinatal and long-term outcomes were prospectively recorded. RESULTS: The median gestational age at procedure was 18...
2015: Fetal Diagnosis and Therapy
Rogelio Cruz-Martínez, Eduard Gratacos
At present, the fetus is already considered a "patient" and as such, can develop diseases with fatal outcome in which the only therapeutic option can be fetal surgery. Currently, fetal surgery is limited almost exclusively to endoscopic surgery. Different techniques have gained clinical acceptance for improving the prognosis of various lethal fetal pathologies. Laser therapy for twin to twin transfusion syndrome and cord occlusion in monochorionic twins with selective intrauterine growth restriction are the procedures of choice for the management of monochorionic twins complications, and are associated with survival rates of up to 80-90% for at least one fetus...
May 2014: Ginecología y Obstetricia de México
J Bildau, C Enzensberger, J Degenhardt, A Kawecki, A Tenzer, T Kohl, R Stressig, J Ritgen, B Utsch, R Axt-Fliedner
The aetiology of urinary tract obstructions (LUTO) is heterogeneous. The most common entities are isolated posterior urethral valves or urethral atresia in male foetuses. In female foetuses LUTO is frequently a part of complex malformations. The natural history of LUTO is characterised by high morbidity and mortality due to the development of severe pulmonary hypoplasia caused by oligo- or anhydramnios affecting the cannalicular phase (16-24 weeks of gestation) of pulmonary development. The degree of renal damage is variable and ranges from mild renal impairment in infancy to end-stage renal insufficiency, necessitating dialysis and transplantation...
February 2014: Zeitschrift Für Geburtshilfe und Neonatologie
Joseph R Siebert, Kenneth J Smith, Liza L Cox, Ian A Glass, Timothy C Cox
Prenatal obstruction of the lower urinary tract may result in megacystis, with subsequent development of hydroureter, hydronephrosis, and renal damage. Oligo- or anhydramnios, pulmonary hypoplasia, and prune belly syndrome are lethal consequences. Causes and mechanisms responsible for obstruction remain unclear but might be clarified by anatomic study at autopsy. To this end, we employed 2 methods of tomographic imaging-optical projection tomography and contrast-enhanced microCT scanning-to elucidate the anatomy of the intact urinary bladder and urethra in 10 male fetuses with lower urinary tract obstruction...
November 2013: Pediatric and Developmental Pathology
Henrietta U Okafor, Sebastian O Ekenze, Samuel N Uwaezuoke
AIM: This study aims to evaluate posterior urethral valve (PUV) in a developing country with focus on the profile of the disease, determinants of outcome and the challenges of management. METHODS: Analysis of data collected prospectively from 31 patients managed for PUV at the University of Nigeria Teaching Hospital, Enugu from January 2008 to December 2009. RESULTS: The median age at presentation was 2.5 years (range 2 weeks-15 years). Only two (6...
February 2013: Journal of Paediatrics and Child Health
Aurelia Masca, L L Indrei, Klara Brânzaniuc
Posterior urethral valves represent a congenital barrier at the level of the posterior urethra, which opposes miction. They are located near the prostatic urethra, originating at the verumontanum level, affecting male patients. The ureters are inconstantly dilated; vesicoureteral reflux is met in 2/3 of the cases. The reflux can be secondary to the sub-bladder barrier, but it can also be considered primitive, determined by the intra bladder ectopy of the ureter. The presence of the reflux is interpreted as a severe associated factor...
July 2011: Revista Medico-chirurgicală̆ a Societă̆ţ̜ii de Medici ş̧i Naturaliş̧ti Din Iaş̧i
R Katie Morris, M D Kilby
Congenital lower urinary tract obstruction (LUTO) is a heterogeneous group of pathologies, the most common being posterior urethral valves (PUV) or urethral atresia. The bladder neck obstruction in utero leads to a spectrum of disease including mild oligohydramnios with normal renal function to a picture of severe oligohydramnios associated with chronic obstructive macro/microcystic renal parenchymal disease leading to chronic renal impairment. These anomalies may be isolated or complex; the latter being associated with other structural or chromosomal abnormalities...
September 2011: Early Human Development
Joseph R Siebert, Martin P R Walker
BACKGROUND: We report the familial recurrence of urethral stenosis/atresia in two sibling fetuses with bladder outlet obstruction, severe oligohydramnios, and pulmonary hypoplasia. Urethral obstruction in the fetus, when severe, results in a dilated urinary bladder (megacystis) and associated urinary anomalies (hydroureter, hydronephrosis, renal dysplasia). Distention of the fetal abdomen, the result of megacystis or urinary ascites, leads to stretching and eventually hypoplasia or even absence of abdominal muscles...
August 2009: Birth Defects Research. Part A, Clinical and Molecular Teratology
Matthew S Clifton, Michael R Harrison, Robert Ball, Hanmin Lee
Fetal urinary tract obstruction with oligohydramnios produces pulmonary hypoplasia and renal dysplasia. Decompression of the obstructed urinary tract may restore amniotic fluid and allow lung growth, but transabdominal catheter shunt decompression is often inadequate and does not allow for cycling of the bladder, while open procedures cause significant maternal morbidity. Disruption of the anatomic obstruction, usually posterior urethral valves in a male fetus, would be ideal but has proven technically difficult...
2008: Fetal Diagnosis and Therapy
David Lissauer, Rachel K Morris, Mark D Kilby
Fetal lower urinary tract obstruction affects 2.2 per 10,000 births. It is a consequence of a range of pathological processes, most commonly posterior urethral valves (64%) or urethral atresia (39%). It is a condition of high mortality and morbidity associated with progressive renal dysfunction and oligohydramnios, and hence fetal pulmonary hypoplasia. Accurate detection is possible via ultrasound, but the underlying pathology is often unknown. In future, magnetic resonance imaging (MRI) may be increasingly used alongside ultrasound in the diagnosis and assessment of fetuses with lower urinary tract obstruction...
December 2007: Seminars in Fetal & Neonatal Medicine
Jean-Marc Biard, Mark P Johnson, Michael C Carr, R Douglas Wilson, Holly L Hedrick, Christina Pavlock, N Scott Adzick
OBJECTIVE: Limited information is available about long-term outcomes in children treated prenatally for lower urinary tract obstruction. Our aim was to evaluate outcomes in children treated in utero with vesicoamniotic shunts. METHODS: Clinical outcomes in 20 pregnancies with a singleton male fetus, oligo/anhydramnios, and lower urinary tract obstruction were studied using chart review and phone and written clinical questionnaire for parents, pediatricians, and urologists...
September 2005: Obstetrics and Gynecology
Tina Vanderheyden, Sailesh Kumar, Nicholas M Fisk
Renal function in utero deals chiefly with urine production rather than the excretion of metabolites, which are cleared by the placenta. Fetal renal impairment (FRI) in bilateral renal disease thus presents as oligohydramnios or anhydramnios; this can lead to lung hypoplasia and early neonatal death. As in the adult, FRI can be divided into prerenal, renal and postrenal causes. Causes of prerenal FRI include intrauterine growth restriction, unbalanced intertwin transfusion in monochorionic twins and maternal drug ingestion...
August 2003: Seminars in Neonatology: SN
B E Finley, T L Bennett, J Burlbaw, L Levitch
The management of bladder outlet obstruction in the fetus remains a debated issue. Recent data suggest the use of cystocentesis for the possible relief of the bladder neck obstruction or for the opening of posterior urethral valves. This case report adds further support for the use of cystocentesis and observation prior to the placement of an indwelling bladder shunt. The case further demonstrates that relief of the megacystis may not prevent prune belly syndrome, bladder dysfunction or renal damage. Finally, the case demonstrates that severe oligohydramnios in the second trimester need not invariably result in pulmonary hypoplasia...
July 1, 1993: Ultrasound in Obstetrics & Gynecology
M J Kemper, T J Neuhaus, K Timmermann, B Hueneke, G Laube, E Harps, D E Mueller-Wiefel
Urinary tract anomalies (UTA) including polycystic kidney disease nowadays can be detected antenatally by ultrasound. The concomitant presence of oligohydramnios has been regarded as a severe risk factor for renal dysfunction and pulmonary hypoplasia, although clinical data after birth are scarce. We report the postnatal course and long-term follow-up of 10 infants with oligohydramnios due to congenital UTA from two pediatric nephrology centers. The underlying final diagnoses were autosomal-recessive polycystic kidney disease (ARPKD, n = 2), familial tubular dysgenesis (n = 2) and bilateral renal hypoplasia (n = 6) including 3 children with posterior urethral valves...
December 2001: Clinical Nephrology
K M Tabsh, N L Theroux
BACKGROUND: Fetuses diagnosed with posterior urethral valve syndrome risk development of significant pulmonary and renal complications. Historically, medical options included expectant management, percutaneous shunting, fetal surgery, and pregnancy termination. CASE: A 34-year-old primigravida presented at 26.5 weeks' gestation with severe fetal hydronephrosis. In lieu of the aforementioned options, the patient elected to undergo serial amnioinfusion and fetal bladder aspirations...
October 1997: Obstetrics and Gynecology
G McLorie, W Farhat, A Khoury, D Geary, G Ryan
PURPOSE: Prenatal detection of obstructive uropathy is used widely and vesicoamniotic shunting is the accepted procedure in well-defined cases. We present outcomes of vesicoamniotic shunting in a consecutive series from a coordinated prenatal-postnatal treatment program. MATERIALS AND METHODS: From 1989 to 1998 bladder obstruction was identified in 89 fetuses at a multidisciplinary prenatal high risk clinic. Prenatal intervention was considered in cases of bilateral hydronephrosis associated with evidence of bladder outlet obstruction and oligohydramnios...
September 2001: Journal of Urology
B D Coley, J A Rusin, D R Boue
BACKGROUND: We have observed many infants with lenticulostriate vasculopathy (LSV) on neurosonograms who do not have classic histories of prenatal infection, trisomy, or prenatal drug exposure. OBJECTIVE: To investigate the underlying clinical conditions in patients with LSV. MATERIALS AND METHODS: Sixty-three cases of LSV were identified among approximately 2,400 neurosonograms performed over 42 months. All neurosonograms were reviewed. Medical records were reviewed for information regarding prenatal infection, chromosomal abnormality, respiratory and cardiac disease, and other pertinent diagnoses...
December 2000: Pediatric Radiology
R S Sutherland
Severe oligohydramnios and renal dysplasia were detected in one of diamniotic, monochorionic twins at 19 weeks' gestation. At birth (37 weeks), the affected twin had only minimal extrarenal Potter's features and mild pulmonary hypoplasia, despite severe renal dysplasia due to posterior urethral valves. The effects of virtual absence of amniotic fluid during the latter half of gestation from bilateral renal dysplasia were ameliorated by the presence of a normal co-twin and its normal amniotic fluid levels, even though the affected twin did not share the same amniotic fluid...
December 1999: Urology
R L Chevalier, S Klahr
Therapy for obstructive uropathy is largely determined by whether the obstruction involves one or both kidneys, and by the age of the patient. In the infant and child, obstructive uropathy is almost always due to a congenital malformation of the ureter, bladder, or urethra. Ultrasonographic prenatal diagnosis has permitted early detection and even fetal intervention for posterior urethral valves, although this form of treatment must be considered experimental at present. More important to the affected infant than optimal renal development is the prevention of pulmonary hypoplasia, which is a consequence of fetal oliguria and oligohydramnios...
November 1998: Seminars in Nephrology
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