经内镜电灼术治疗小儿后尿道瓣膜症

目的 探讨内镜电灼术治疗小儿后尿道瓣膜的临床效果.方法 对45例经内镜电灼术治疗后的小儿后尿道瓣膜进行回顾性分析.本组病例均为男性,年龄2周～15岁,中位年龄3岁.婴幼儿用6/7.5F输尿管镜或9F膀胱尿道镜和Bugbee针状电极,大龄儿用12F或13F膀胱镜和钩状电极,在尿道的5点、7点和12点处电灼后尿道瓣膜.术后长期随访.结果 根据Young分型,本组患儿中I型44例,Ⅲ型1例,术中顺利,术后排尿通畅度改善(尿线明显增粗,最大尿流率均在16.50 ml/s以上),血肌酐水平恢复正常,尿白细胞消失,肾积水程度均不同程度减轻(35例肾积水患儿中12例轻度肾积水消失,16例中度肾积水转为轻度肾积水,7例重度肾积水转为中度肾积水),膀胱输尿管反流程度较术前均有明显改善(29例膀胱输尿管反流的患儿中16例患儿反流消失,6例反流程度由术前Ⅱ度转为Ⅰ度,4例患儿由术前Ⅳ度转为Ⅱ度,3例患儿由术前V度转为Ⅲ度).结论 内镜下电灼术治疗后尿道瓣膜症是一种可行的方法,疗效确切,术后并发症少,预后好.针状电极可明显减少尿道狭窄的发生率.

Abstract：

Objective The aim of this study was to assess the effectiveness of endoscopic ablation of posterior urethral valves. Methods We retrospectively reviewed a database of 45 patients with PUV treated by endoscopic valve ablation. In this study, all patients were diagnosed at the age of 3 years (range: 2 weeks to 15 years). 6/7. 5F ureteroscopy and 9F cystoscopy with needle electrode were used in infants,whereas 12F or 13F cystoscopy and hook electrode were used in older children.The valves were ablated mainly at the 5,7 and 12 o'clock positions. The patients were followed up after surgery. Results Based on Young's classification, there were 44 type Ⅰ and 1 type Ⅲ PUV in this study. Urine line became thicker, the maximum flow rate can be achieved more than 16. 50 ml/s. Serum creatinine returned to normal and white blood cells in urine disappeared. The degree of dilatation of hydronephrosis relieved (among 35 hydronephrosis patients, 12 cases disappeared, 16 mild hydronephrosis to moderate hydronephrosis and 7 severe hydronephrosis to moderate hydronephrosis). Compared to preoperation.vesicoureteral reflux had a significantly improved (among 29 cases, 16 reflux disappeared,6 grade Ⅱ to grade I ,4 grade Ⅳ to Ⅱ and 3 grade Ⅴ to grade Ⅲ ). Conclusions Endoscopic ablation in treating pediatric posterior urethral valves seems to be safe and is associated with low rate of postoperative complications. Needle electrode can reduce the rate of urethral stricture.     

Timing of posterior urethral valve diagnosis and its impact on clinical outcome

Objective

Our goals were to assess long-term outcome for patients with posterior urethral valves (PUV) and determine the impact of the timing of diagnosis.

Patients and methods

We reviewed the hospital records for patients with PUV treated in 1994–2008. Only those patients diagnosed, treated surgically, and followed clinically at our center were included.

Results

There were 52 patients with PUV. Thirty-nine were diagnosed by 1 year of age (early diagnosis cohort), while 13 were diagnosed after 1 year (late diagnosis cohort). Mean follow up after valve operation was 7.2 years (range 15 months–14 years). Chronic renal failure rates were not statistically different between the early and late diagnosis groups after surgical intervention: 48% (14/29) vs 25% (3/12), P = 0.30. Among the early diagnosis cases, 10% (3/29) eventually required renal transplant, while no child in the late diagnosis group has developed end-stage renal disease to date (P = 0.55).

Conclusions

Gestational age at diagnosis is an important predictor of postnatal renal outcome. Our results suggest that diagnosis after 1 year of age is associated with a lower risk of developing renal insufficiency on long-term follow up.     

Role of vesicostomy in the management of posterior urethral valve in Sub-Saharan Africa

ObjectiveTo review the role of vesicostomy in the management of posterior urethral valve (PUV), in neonates and infants, given the limitations for endoscopic treatment in this setting.MethodsA review of 35 patients who presented with posterior urethral valve over a 10-year period. Demographic and clinical information were prospectively recorded on a structured pro forma, and the data extracted analysed using SPSS 11.0.ResultsThe 35 boys were aged 3 days to 10 years (median 3 weeks). Twenty-three (65.7%) had a vesicostomy (age range 3 days–3 years, median 3 weeks). The mode of presentation was poor urinary stream 15 (65.2%), urinary retention 4 (17.4%), and renal failure 6 (26.1%). Main findings were palpable bladder 23 (100%), hydronephrosis 4 (17.4%). Abdominal ultrasound confirmed hydronephrosis and thickened bladder wall, and voiding/expressive cystourethrogram confirmed dilated posterior urethra and vesicoureteric reflux in all 23 patients. Complications following vesicostomy were stoma stenosis 1 (4.3%), bladder mucosal prolapse 1 (4.3%), perivesicostomy abscess 1 (4.3%); there was no mortality. Following vesicostomy, 10 (43.5%) patients had excision of the valves and vesicostomy closure at age 2–8 years (median 4 years). They are well, with normal renal ultrasonographic findings, bladder capacity range 115–280 ml, and normal urea, serum electrolytes, creatinine, at 3 years of follow up. Thirteen (56.5%) are still awaiting valvotomy but have remained well and with normal ultrasonographic renal findings.ConclusionVesicostomy is a useful temporising mode of urinary diversion in neonates and infants with posterior urethral valve (in the absence of unobstructed upper tracts) when facilities for endoscopic valve ablation are not readily available.     

Does bladder augmentation negatively affect renal transplant outcome in posterior urethral valve patients?

ObjectiveAlthough renal transplant (RT) is a safe and effective treatment for end-stage renal disease, the outcome of RT has been mixed for posterior urethral valve (PUV) patients. In addition, some PUV patients need an augmentation cystoplasty (AC), which may negatively affect their RT outcome. The aim of this study is to compare RT outcome between PUV children with and without AC.Materials and methodsBetween 1985 and 2012 a total of 309 children received 369 RTs at our institution. Among these patients, 36 were had classified as having PUV. Of these, 12 underwent an AC before RT (AC group) and 24 did not (controls). Data, including age at transplant, allograft source, urological complications, urinary tract infection (UTI) incidence, the presence of vesicoureteral reflux (VUR), and patient and graft survival, were compared between groups.ResultsMean age at RT and mean follow-up were 7.6 versus 7.9 years and 8.9 versus 7.9 years in the AC group and in the control group, respectively (not significant [NS]). Allografts were from living donors in 50% of the AC group and in 41.6% of the controls (NS). The rate of UTI was 0.02 UTI/patient/year and 0.004 UTI/patient/year in the AC and control group, respectively (p = 0.001). Of the nine patients with UTI in the augmented group, five (55.5%) had VUR, while 5/8 (62.5%) patients in the control group with UTI had VUR. All patients with VUR in either group had UTIs previously. Of the five AC patients with more than three UTIs, two (40%) were non-compliant with clean intermittent catheterization (CIC), and UTI incidence was not associated with either a Mitrofanoff conduit or the urethra being used for CIC. Graft function at the end of study was 87.8 ± 40.5 ml/min/m² in the AC group and 88.17 ± 28.20 ml/min/m² in the control group (NS). The 10-year graft survival rate was 100% in AC group and 84.8% in controls. Two patients in the AC group lost their grafts (mean follow-up 13.3 ± 0.8 years) and five in the control group (mean follow-up 7.1 ± 4.7 years).ConclusionsBladder augmentation does not negatively affect renal outcome in PUV patients undergoing transplantation. However, recurrent UTIs are more frequent in transplanted PUV patients with an AC than in those without AC, and they are generally related to non-compliance with CIC or the presence of VUR but, mostly, they will not result in impaired graft function.     

Posterior urethral valve

Background  

Posterior urethral valve (PUV) is a significant cause of morbidity, mortality and ongoing renal damage in children. It accounts for end-stage renal disease in a proportion of children. This article aims at highlighting the current trend in the management of boys with posterior urethral valve.     

Anterior urethral valve

The case of an 11-year-old boy with an anterior urethral valve is reported. A weak urinary stream and urinary dribbling were his main symptoms. Clinical evaluation consisted of renal ultrasonography, an intravenous urogram, a voiding cystourethrogram, and panendoscopy. Transurethral excision of the valve achieved a satisfactory result.     

Megalourethra with posterior urethral valves

A case of scaphoid megalourethra associated with the posterior urethral valves was seen in a 4-year-old male. The embryology of the anomaly and its management are discussed with a review of the literature. Accepted: 30 November 1998     

Anterior urethral valve associated with posterior urethral valves

The association of anterior urethral valve (AUV) with posterior urethral valve (PUV) is rare. A 7-month-old infant was presented at a district hospital with episodes of acute pyelonephritis. He was treated medically and a voiding cystourethrogram (VCUG) confirmed bilateral vesico-urethral reflux. The presence of concomitant AUV and PUV was not recognized. He underwent several surgical procedures, which failed. He had reflux recurrence following two antireflux procedures. He had urinary retention after each operation, which was managed by vesicostomy and perineal urethrostomy. At the age of 3.5 years, he was referred to our paediatric urology clinic. Noticing the AUV and PUV in the past VCUG, the valves were fulgurated. Urodynamic study before and 3 months after valve ablation showed a high voiding pressure. VCUG 6 months following ablation showed no reflux, but several uroflowmetric studies showed a staccato and interrupted pattern. Empirical treatment with an alpha-blocker was started. One year after treatment, a repeat VCUG showed no reflux. Uroflowmetry and urodynamic studies returned to normal. The perineal urethrostomy was closed. The child was asymptomatic after 9 months of follow up.     

Low imperforate anus associated with seminal vesicle cyst,posterior urethral valve,and lung hypoplasia

This report describes the association of a low imperforate anus with a seminal vesicle cyst, posterior urethral valve, and lung hypoplasia complicated by necrotizing enterocolitis and unilateral vesicoureteral reflux. The embryological basis, diagnosis, and management of these anomalies is reviewed.     

Urinary continence following posterior urethral valves treatment

This retrospective study discusses the incidence of symptomatic voiding dysfunction, the etiological factors and the changes with age and with increasing duration of follow up in 67 boys treated for posterior urethral valves and followed up for a period ranging from 1–21 years. Twenty three of the 67 patients (34.3%) had symptomatic voiding dysfunction; the commonest symptom being nocturnal enuresis with diurnal urgency and frequency. Data in this study suggests a definite decrease in the incidence of urinary incontinence as the patient grows older or as the duration of follow up after valve fulguration increases. A Tc-99m DRCG based uroflowmetry was available in 31 of the 67 patients at the time of this review and evidence of detrusor dysfunction was identified in 9 of the 31 patients on the basis of uroflowmetry. Of significance was the detection of detrusor dysfunction on uroflowmetry in 3 patients with a history of normal urinary control and urinary stream with persistent post treatment hydroureteronephrosis eventually culminating into renal failure. Imipramine therapy was given to 10 of the 17 patients with symptoms of urinary frequency, urgency and nocturnal enuresis with a significant symptomatic improvement in 9 of these patients. Urinary incontinence, detrusor dysfunction and its effect on the upper tracts and renal function is a significant determinant of the long term outcome of the boys treated for posterior urethral valves and this paper emphasises on the need to carefully evaluate and treat this aspect of all valve patients.     

Posterior urethral valves after infancy–urodynamic consequences

This study describes a subset of patients with posterior urethral valves (PUV) who presented late in childhood. The objective was to identify factors that lead to back-pressure effects on the upper tracts, which persist in spite of adequate valve ablation in some patients, and seek factors that may preserve the upper tracts despite untreated obstruction in other patients. Six children with PUV diagnosed after infancy were evaluated. The pre-operative work-up included renal biochemistry, ultrasonography, voiding cystourethrography, and uroflowmetry. Detailed urodynamic studies, including uroflowmetry and slow-fill cystometry, were performed in all cases 6 months after surgery. Adequacy of valve fulguration was confirmed by urethroscopy. Three of the six patients had normal upper tracts; in these, there was marked improvement in peak urine flow rates after fulguration and bladder pressures were normal. The other three patients had bilateral hydroureteronephrosis, and two had chronic renal failure. This group had markedly decreased functional bladder capacity with loss of compliance at low bladder volumes and significant residual urine volumes in spite of adequate valve fulguration, suggesting myogenic detrusor failure. We conclude that in patients with PUV presenting beyond the age of 5 years, upper-tract deterioration may accompany high storage pressures in the bladder. In some boys with long-standing obstruction the upper tracts may escape damage; in our series this was associated with normal bladder dynamics and appeared unrelated to the severity or duration of outflow obstruction. Accepted: 13 August 1997     

Evolution of urodynamic patterns in posterior urethral valves

A retrospective, long-term urodynamic study was performed in order to follow the evolution of the urodynamic patterns in 55 unselected patients previously affected by posterior urethral valves (PUV). The mean maximum cystometric capacity (MCC) values progressively increased over time and, on long-term follow-up, were just above 2 standard deviations (SD). The mean compliance values were clearly reduced in the first urodynamic studies after valve ablation, and only after a mean of 5 years follow-up did they approach the lower limits of normal. The small compliance and capacity (SCC) group showed two trends of evolution: a more numerous subgroup tended toward progressive normalization while a second subgroup (<20%) showed mean compliance values below normal limits, with reduced (-2 SD) MCC persisting at long-term follow-up. The number of patients in this group decreased over time. In contrast, we were able to show a significant increase in patients with myogenic failure. In this group scheduled voiding using the Valsava maneuver in conjunction with a regimen of double or triple micturition was usually succesful in modifying the course, normalizing MCC, reducing residual urine, and also eliminating incontinence. Finally, initial urodynamic investigations in the fulguration and vesicostomy groups showed a much higher percentage of SCC bladders in the latter group (83.5% vs. 35%). However, at long-term examinations the urodynamic parameters were nearly identical in both groups, showing that temporary bladder defunctionalization does bot adversely affect future detrusor activity. No direct relationship between urodynamic abnormalities and renal insufficiency could be shown, however, the majority of patients with reduced glomerular filtration rates still showed urodynamic dysfunction at long-term follow-up. In the authors' opinion, serial urodynamic investigations in association with serial evaluation of the evolution of upper urinary tract and renal function are mandatory for correct PUV management and provide useful guidelines for avoiding incorrect treatment and obtaining better long-term results.     

An unique case of Y-type urethral duplication associated with posterior urethral valve

This case report is about a 4-year-old patient with a IIA2 'Y-type' duplication, with the accessory urethra arising from the anterior orthotopic urethra and exiting in perineal-scrotal position, in association with posterior urethral valves (PUV). Cystourethroscopy through the ventral urethra revealed a type III urethral valve (diaphragm) and this was fulgurated. Duplicated dorsal urethra was excised surgically. Postoperative period was uneventful. This case is unique, because it shows PUV in a child with a very rare type of 'Y-type' duplication. The presence of PUV in patients with urethral duplication is probably not an incidental finding but, to date, embryology of this rare association is not known.     

Duplication of the male urethra with posterior urethral valves

The only known case of complete duplication of the male urethra associated with posterior urethral valves is reported. The embryological implications of this association are discussed.     

Ultrasound diagnosis of the anterior urethral valve

We present a neonate with an obstructing anterior urethral valve depicted by longitudinal transpenile ultrasound. Voiding images are recommended to identify the obstructing valve tissue. Ultrasound depiction of the anterior urethral valve tissue is identical to that seen in posterior urethral valves. Received: 6 December 2000 Accepted: 12 March 2001     

Congenital scaphoid megalourethra associated with posterior urethral valves

Megalourethra is a very rare mesenchymal congenital anomaly and its association with posterior urethral valves is still rarer. We hereby present such a rare combination and discuss its etiopatho-genesis Accepted: 16 June 1998     

Posterior urethral valves: the scenario in a developing center

We have reviewed 233 patients with posterior urethral valves treated in a single center in Calcutta, India, over the last 20 years: 37 were neonates, 75 were between 1 and 12 months, 88 were between 1 and 5 years, and 33 were more than 5 years old when first seen. The clinical presentation and methods employed in diagnosis and assessment are described. Primary endoscopic valve ablation was performed in 140 patients (60%). One or other form of diversion was done in 100 (43%), 93 before and 7 either during or after valve ablation. The short- and long-term results have been studied. Eleven patients died during the initial hospitalization, 3 died subsequently, 15 are in end-stage renal disease, 17 are in poor health, and 18 have been totally lost to follow-up. The remaining 169 have been in good health for periods between 1 and 20 years. While our results of primary valve ablation in low-risk patients with responsible parents are as good as anywhere else in the world, we are concerned at our relatively high diversion rate and relatively poor long-term follow up; the methods being adopted to reduce these problems are discussed. Accepted: 8 May 2000     

Upper-tract changes after treatment of posterior urethral valves

This paper discusses the long-term sequelae in the upper urinary tract with respect to hydroureteronephrosis (HUN), vesicoureteral reflux (VUR), renal parenchymal disease, and their correlation with renal function in 84 boys with posterior urethral valves followed for 1 to 21 years. Thirty-one boys (39.3%) were adolescents or older at the time of review. The incidence of high-grade VUR (grade III or more) was 47.6% at presentation, and resolution following decompression of the lower urinary tract occurred in 38.7% of refluxing units. VUR was associated with a high incidence of chronic renal failure (CRF) (30%) on long-term follow up; however, 16% of non-refluxing patients also progressed to CRF. The incidences of renal parenchymal disease and persistent upper-tract dilatation in the non-refluxing group were 25% and 50% of renal units respectively. Gross HUN persisted in 12.3% of patients despite decompression and reconstructive surgery, with vesicoureteral junction (VUJ) obstruction being documented in 1 patient only. Moderate and mild upper-tract dilatation persisted in 31.6% and 43.9% of patients, respectively. Persistent gross HUN was associated with a very high incidence of CRF (92.3%), while 88.4% of those with persistent mild/moderate dilatation maintained normal renal function over a follow-up period ranging from 1 to 21 years. This study emphasizes the need for systematic evaluation to exclude VUJ obstruction and abnormal urodynamics as a cause of persistent HUN so that effective therapy can be instituted early to relieve back-pressure and to provide a low-pressure reservoir with effective emptying. In the absence of either of these causes, persistent ureterectasis after treatment is presumably due to secondary peristaltic failure as a consequence of ureteral fibrosis, ureteral tortuosity, or developmental dysplasia. Accepted: 6 November 1998     

Subdural empyema in a child with posterior urethral valves

An unusual case of subdural empyema is reported in a three months old infant with posterior urethral valve. The patient presented with convulsion. The diagnosis was made by CT Scanning. Repeated aspirations were carried out and patient was discharged well without any deficit. The review of literature did not reveal any case report of subdural empyema in patients with posterior urethral valve.