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Urethral Valves (urethral + valve)
Kinds of Urethral Valves Selected AbstractsAnterior urethral valve as a cause of end-stage renal diseaseINTERNATIONAL JOURNAL OF UROLOGY, Issue 3 2001Cem Aygün Abstract Although posterior urethral valves are predominant as a cause of obstructive uropathy in children, anterior urethral valves may also appear as the underlying etiologic factor in end-stage renal disease that results from obstruction. Two cases are presented of anterior urethral valve patients that were admitted with end-stage renal disease. The first case was successfully treated with diverticulectomy and urethral reconstruction in preparation for renal transplantation. The second case, however, had been on cystostomy drainage for 6 years and also had a contracted bladder. A more extensive lower urinary tract reconstruction was delayed. Children with poor stream and recurrent infections should be evaluated carefully and anterior urethral valve or diverticula should be considered in differential diagnosis of obstructive lesions. [source] Histological study of fetal kidney with urethral obstruction and vesicoureteral reflux: A consideration on the etiology of congenital reflux nephropathyINTERNATIONAL JOURNAL OF UROLOGY, Issue 10 2003KENJI SHIMADA Purpose: A recent subject of interest regarding reflux nephropathy is the presence of renal abnormalities in neonates and infants who have no history of urinary tract infections. Debates have centered on the etiology of this renal abnormality , congenital reflux nephropathy; regarding whether it is the result of abnormal ureteral budding or of back pressure effect from sterile reflux. We examined the renal pathology of fetuses with urethral obstruction and vesicoureteral reflux, and we suggest herein a possible etiology of congenital reflux nephropathy. Methods: The renal pathology of seven autopsied fetuses with vesicoureteral reflux was studied. Reflux was demonstrated at autopsy by slow injection of contrast medium into the bladder. Severe urethral obstruction, either atresia or urethral valves, was evident in six of the subjects. Results: In six subjects, abnormality of the urinary tracts was detected by prenatal ultrasonography. Of these six subjects, three revealed characteristics of prune belly syndrome. Reflux was graded as moderate in five subjects, and severe in two. In three subjects autopsied at 21 weeks gestation or earlier, the kidneys were well-developed with normal corticomedullary configuration, and nephrogenesis was retained. In three cases autopsied at over 25 weeks of gestation, the kidneys were grossly cystic, and the nephrogenic zone was completely absent. Contrast medium was observed not only in the dilated ducts and tubules, but also in the subcapsular cysts. Extravasation of the contrast medium was seen in the peritubular space. In the last subject with normal lower urinary tract, abnormal segments among normal cortical structures were observed. Conclusion: Our findings of renal pathology in fetuses with reflux are quite similar to those seen in fetal hydronephrosis. Back pressure from reflux probably damages the developing kidney leading to a degeneration of the ampullae and a reduction in the number of nephrons. Both dilatation of the collecting ducts and tubules, and extravasation of the urine may result in interstitial fibrosis. We postulate that one of the important etiologies of congenital reflux nephropathy may be the result of back pressure from sterile reflux. [source] Anterior urethral valve as a cause of end-stage renal diseaseINTERNATIONAL JOURNAL OF UROLOGY, Issue 3 2001Cem Aygün Abstract Although posterior urethral valves are predominant as a cause of obstructive uropathy in children, anterior urethral valves may also appear as the underlying etiologic factor in end-stage renal disease that results from obstruction. Two cases are presented of anterior urethral valve patients that were admitted with end-stage renal disease. The first case was successfully treated with diverticulectomy and urethral reconstruction in preparation for renal transplantation. The second case, however, had been on cystostomy drainage for 6 years and also had a contracted bladder. A more extensive lower urinary tract reconstruction was delayed. Children with poor stream and recurrent infections should be evaluated carefully and anterior urethral valve or diverticula should be considered in differential diagnosis of obstructive lesions. [source] Limited surgical interventions in children with posterior urethral valves can lead to better outcomes following renal transplantationPEDIATRIC TRANSPLANTATION, Issue 5 2002Leah Bartsch Abstract: There is currently no consensus as to the most appropriate means by which children with posterior urethral valves (PUV) are to be managed prior to transplantation. We compared (i) renal allograft survival and function in patients with PUV vs. those with non-obstructive causes of ESRD and (ii) graft outcomes in children who had limited interventions (Group 1) vs. those with more extensive urologic surgeries to decompress the urinary tract (Group 2). Twenty-six pediatric renal transplant recipients had ESRD due to PUV (Group 1, n = 16; Group 2, n = 10). The study group was compared to 23 matched controls with ESRD due to non-obstructive causes. Five yr patient and graft survival was similar in all patients with PUV (Groups 1 and 2) when compared to all other kidney recipients in the transplant program, 96.2% vs. 98.0% and 87.5% vs. 87.0%, respectively. Although calculated creatinine clearance (Ccr), was similar between the PUV group and controls for the first 4 yr, the 5 yr graft function was significantly lower in the PUV group. (53.7 ± 15.7 vs. 70.2 ± 21.0 mL/min/1.73 m2; p = 0.03). When the two PUV groups were compared, graft survival was equivalent, but graft function was significantly better at 5 yr in Group 1(60.4 ± 10.8 vs. 33.8 ± 9.3 mL/min/1.73 m2; p = 0.02). Thus, patients with PUV managed by a limited intervention approach of vesicostomy with delayed valve ablation or primary valve ablation, had better outcomes. When ESRD is virtually certain, additional pre-transplant surgeries affecting the urinary tract should be avoided. [source] Early prenatal diagnosis of concordant posterior urethral valves in male monochorionic twinsPRENATAL DIAGNOSIS, Issue 1 2006G. M. Maruotti Abstract The association between monozygotic twins and posterior urethral valves (PUV) in postnatal life has been thoroughly described. In the fetus, the prenatal recognition of PUV is feasible. However, it has been repeatedly reported in singletons but never in monochorionic twins. We describe two cases of early prenatal diagnosis of concordant PUVs in monochorionic twins. In one of the sets, the expression of the disease was different for each twin. Copyright © 2006 John Wiley & Sons, Ltd. [source] Fetal cystoscopy in the management of fetal obstructive uropathy: experience in a single European centrePRENATAL DIAGNOSIS, Issue 13 2003Alec Welsh Abstract Objective To audit diagnostic and therapeutic fetal cystoscopy for suspected posterior urethral valves (PUV). Methods In 13 fetuses, (14,28 weeks) the bladder was entered with a 1.3 mm embryo-fetoscope and intravesical findings documented. In 10 fetuses, an attempt was made to treat the obstruction by saline hydro-ablation (n = 4) and/or guide-wire passage (n = 9). Renal function was assessed post-natally at 10 to 34 months. Results The bladder wall was visualised in 12/13 cases and the bladder neck in 11; in 10 cases the upper urethra was entered, and the obstruction visualised in five. PUV were ,seen' in 4/9 confirmed cases, but also in one case of urethral atresia, while in two others the degree of resistance to guide-wire passage suggested atresia or prune belly. Therapeutic attempts were technically successful, at least initially, in 6/10 cases. Of the five cases with confirmed PUV and normal fetal urinary electrolytes, two have acceptable renal function at follow-up. Hydro-ablation in one case resulted in resolution of sonographic signs of obstruction, and ablated valves were confirmed post-natally. Conclusions Semi-rigid fetal cystoscopy allows entry into the upper urethra in most obstructive uropathies, although bladder neck angulation precludes visualisation of the site of obstruction in the majority. Guide-wire passage or hydro-ablation may allow relatively atraumatic ablation of PUV in utero without the chronic bladder decompression associated with vesico-amniotic shunting. However, current technical limitations need to be overcome, possibly by the use of flexible or angled fetoscopes, before the role of cystoscopic treatment can be formally evaluated. Copyright © 2003 John Wiley & Sons, Ltd. [source] An overview of the literature on congenital lower urinary tract obstruction and introduction to the PLUTO trial: Percutaneous shunting in lower urinary tract obstructionAUSTRALIAN AND NEW ZEALAND JOURNAL OF OBSTETRICS AND GYNAECOLOGY, Issue 1 2009R. Katie MORRIS Congenital lower urinary tract obstruction (LUTO) comprises a heterogeneous group of pathologies causing obstruction to the urethra, the most common being posterior urethral valves. Such pathology is often associated with high perinatal mortality and varying degrees of perinatal and infant morbidity. A high proportion of LUTO may be visualised during routine second trimester (and first trimester) ultrasound giving rise to the possibility of determining individual fetal prognosis and treatments such as vesico-amniotic shunting, with a view to altering pathogenesis. The aims of the percutaneous shunting in low urinary tract obstruction (PLUTO) trial are to determine the effectiveness of these treatments and accuracy of the investigations with the primary outcome measures being perinatal mortality and postnatal renal function. [source] Myogenic bladder decompensation in boys with a history of posterior urethral valves is caused by secondary bladder neck obstruction?BJU INTERNATIONAL, Issue 1 2005Philippos A. Androulakakis OBJECTIVE To investigate whether myogenic bladder decompensation in patients treated for congenital posterior urethral valves (PUV, the most serious cause of infravesical obstruction in male neonates and infants) may be secondary to bladder neck obstruction, as despite prompt ablation of PUV these patients can have dysfunctional voiding during later childhood or adolescence, the so-called ,valve bladder syndrome'. PATIENTS AND METHODS The study comprised 18 boys (mean age 14 years, range 6.2,18.5) who had had successful transurethral ablation of PUV between 1982 and 1996, and had completed a follow-up which included serial assessment of serum creatinine, completion of a standard voiding diary, ultrasonography with measurement of urine before and after voiding, a urodynamic examination with simultaneous multichannel recording of pressure, volume and flow relationships during the filling and voiding phases, coupled with video-cystoscopy at least twice. The mean (range) follow-up was 9.3 (6,17) years. RESULTS Urodynamic investigation showed myogenic failure with inadequate bladder emptying in 10 patients; five with myogenic failure also had unstable bladder contractions. On video-cystoscopy the posterior bladder neck lip appeared elevated in all patients but in those with myogenic failure it was strongly suggestive of hypertrophy, with evidence of obstruction. At the last follow-up one patient with myogenic failure who had had bladder neck incision and four others who were being treated with ,-adrenergic antagonists had a significant reduction of their postvoid residual urine. CONCLUSION Despite early valve ablation, a large proportion of boys treated for PUV have gradual detrusor decompensation, which may be caused by secondary bladder neck obstruction leading to obstructive voiding and finally detrusor failure. Surgical or pharmacological intervention to improve bladder neck obstruction may possibly avert this course, but further studies are needed to validate this hypothesis. [source] Complete unilateral leg duplication with ipsilateral renal agenesisACTA PAEDIATRICA, Issue 3 2007Christian Lilje Abstract A case of a 5-month-old infant with complete duplication of the right leg and ipsilateral renal agenesis is presented. There was also a duplicated scrotum, posterior urethral valves with hydronephrosis, abnormal pelvic bones, and a ventricular septal defect. Complete caudal duplication is exceedingly rare. Associated defects are common. Several aetiological theories have been proposed. Among them, an underlying polytopic (acro-renal) developmental field defect has been suggested. It best explains the specific findings in our patient. However, the aetiology of caudal duplication syndromes may be multifactorial. In the light of twinning theories, associated duplication of hindgut derivates should be suspected in similar cases. Further research is needed. The motor-skeletal functional outcome after leg duplication surgery is mostly favourable. Conclusion: A case of complete unilateral leg duplication with ipsilateral renal agenesis is presented. It appears to support the theory of a polytopic developmental field defect. [source] | ||||||||||