Pediatr Surg Int (2011) 27:10271035 DOI 10.

1007/s00383-011-2946-9

REVIEW ARTICLE

Posterior urethral valves: long-term outcome

Paolo Caione Simona Gerocarni Nappo

Accepted: 27 June 2011 / Published online: 12 July 2011 Springer-Verlag 2011

Abstract Posterior urethral valves represent the most common cause of bladder outlet obstruction in infancy that impairs renal and bladder function. Long-term outcome of patients with previous PUV is evaluated. Patients over 18 years of age, treated from 1982 to 1995 before the age of 3 years were considered. Previous surgery, renal function, bladder activity, urinary incontinence, and fertility/sexual activity were evaluated. Clinical interview, creatinine clearance, uroowmetry with ultrasound post-void urine residue, and self-administered questionnaire were recorded. Out of 45 identied records, 24 patients (53.3%) accepted to be enrolled (age 1834 years, mean 23 years). The mean follow-up was 19.5 years (1630 years). Out of the 21 excluded patients, 20 did not reply to the clinical interview and 1 died at age of 6 years. All the 24 patients had early endoscopic section of PUV; nine also received transient ureterocutaneostomy or vesicostomy. Ureteroneocystostomy was performed in ve patients and ureterocystoplasty with unilateral nephrectomy in two. At follow-up chronic renal failure was detected in 13 patients (54.1%) and 9 (37.5%) had arterial hypertension. End-stage renal disease developed in ve patients (20.8%): three had successful renal transplantation and two were in dialysis. Lower urinary tract symptoms were present in seven patients (29.1%). No signicant fertility decit and sexual dysfunction were observed in 23 patients, while 1 patient was azoospermic. No paternity was reported so far. Long-term outcome of

patients with previously treated PUV is mandatory. Kidney, bladder, and sexual functions should be monitored till adulthood to verify any modied behaviour. Keywords Posterior uretral valves Adolescent urology Obstructive uropathy Long-term follow-up ESRD

Introduction Posterior urethral valves (PUV) is the main cause of bladder outow obstruction in male newborns and infants and is also the most common obstructive uropathy leading to chronic renal failure (CRF) and end-stage renal disease (ESRD) in childhood [1, 2]. The incidence of PUV is estimated as 1:5,0001:8,000 male live births [35], but it is probably underestimated, due to foetal and perinatal demise for the more severe forms, and conversely to a delayed diagnosis in the milder forms. Mortality rate in PUV patients has signicantly decreased in the past four decades, from almost 40% in the 1960s [6] to less than 5% in 1985 [7] and is even lower nowadays, due to prenatal diagnosis, improvement of respiratory support and resuscitation at birth, and adequate management of ESRD [8]. In spite of these improvements in early management, long-term morbidity related to PUV treated in neonatal and infant age still represents a heavy burden for these patients. Even the advent of prenatal diagnosis and the possibility of in utero intervention have not signicantly improved the prognosis of renal function in boys with PUV [1, 9]. The development of ESRD and the need of dialysis-renal transplant programme were shown to be related to signicant bladder dysfunction before and after adulthood [10, 11]. Furthermore, the

P. Caione (&) S. G. Nappo Division of Pediatric Urology, Department of Nephrology-Urology, ` Childrens Hospital and Research Institute, Bambino Gesu Piazza S. Onofrio, 4, 00165 Rome, Italy e-mail: paolo.caione@opbg.net

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severe obstruction of bladder outlet results in a distorted, elongated and dilated posterior urethra with hypertrophic bladder neck, and these structural and functional changes, despite early endoscopic valve resection, can interfere not only with day-time and night-time urinary continence, but also with sexual and reproductive functions of this cohort of young adults [12, 13]. The long-term outcome of a group of young adult patients who were treated for PUV early after birth in our Institution is presented. The aim of this study was to evaluate the health status of valve patients after puberty.

newborns or infants. Prenatal diagnosis showing bilateral hydronephrosis and/or foetal megacystis was detected in 8 out of the 45 patients of the present series (17.7%) (Table 1). Follow-up was from 16 to 30 years (mean followup = 19.5 years). Renal function and reux Among the 24 patients, 5 developed ESRD (20.8%): 3 of them (12.5%) received successful renal transplantation that was found well functioning (mean creatinine 1.21 mg%) at the time of the survey, and 2 were haemodialysis. A total of 13 patients (54.1%) had CRF and need limited dietary restriction and nephrological support. 9 patients (37.5%) presented systemic arterial hypertension, requiring drug control (Table 1) and two of them had secondary nephrectomy of the poorly functioning kidney in order to achieve hypertension control. VUR was present in 15 patients (62.5%): six unilateral, associated with poor renal function and nine (37.5%) bilateral. The development of CRF was signicantly higher (p \ 0.05) in the group of patients with bilateral group (Table 2). Previous surgery on urinary tract Seven patients had transient suprapubic catheter or vesicostomy as rst surgical approach followed by delayed endoscopic section of valves and two had ureterocutaneostomy performed in the rst year of life, (Fig. 1). In all of them, PUV were treated by trans-urethral endoscopic section at birth or within the rst 4 months of life. An endoscopic second look was performed in 19 out of 24 patients (79.1%). Ureteroneocystostomy was performed in ve patients (20.8%), three for ureterovesical junction
Table 1 Long-term outcomes on PUV (follow-up: 1630 years mean follow-up: 19.5 years): demographic data and renal function Patients Total records 45 8 1 20 24 1834 23 13 5 3 2 9 54.1 20.8 12.5 8.3 37.5 % 100.0 17.7 2.2 44.4 53.4

Materials and methods We retrospectively reviewed the charts of boys with PUV consecutively treated in our department between 1982 and 1995 years, with less than 3 years at their rst presentation. The patients were investigated for previous surgery, renal function, lower urinary tract symptoms and urinary incontinence, sexual activity and fertility. Survival, ESRF, need of dialysis, renal transplantation, chronic renal failure (CRF) and arterial hypertension were considered for nephrological outcome. CRF was dened as creatinine clearance \65 ml/min. Urological outcome was assessed on previous or permanent urinary diversion, vesico-ureteral reux (VUR), recurrent urinary tract infections (UTIs) in the past 5 years, abnormal bladder urodynamics, day-time wetting at age [5 years, uroowmetry with post-void residual urine, 24 h urine output and self-administered selected questions from International Prostate Symptom Score (IPSS). Andrological problems regarding sexual activity and fertility were checked by interview by selfadministered International Index of Erectile Function (IIEF) questionnaire. For statistical analysis, Students t test for quantitative numeric data comparison and Chisquare test for matching two discrete variables were adopted. A p value of less than 0.05 was considered as signicant.

Results Patient recruitment and survival A total of 45 records were identied as eligible for the study. Twenty patients were lost to follow-up or refused to be enrolled and one patient died at age 6 years due to complications of severe arterial hypertension and ESRD. So, 24 patients (53.3%) with a mean age of 123 years (range 1834 years) represent the study group. All presented with classic PUV (type I or III of the Youngs classication [14]) and were treated in our Department as

Prenatal diagnosis Death Patients missed at follow-up Patients in the study Age (years) Mean age at follow-up (years) CRF ESRD Renal transplant Dialysis Systemic arterial hypertension

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Pediatr Surg Int (2011) 27:10271035 Table 2 VUR and CRF in PUV patients VUR PTS Total Unilateral Bilateral 15 6 9 % 62.5 25.0 37.5 CRF PTS 9 1 8 % 60.0 16.6 88.9 0.05 p

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obstruction and two for breakthrough urinary infections with severe reux (Table 3). Two patients had ureterocystoplasty during laparoscopic nephrectomy. Urinary tract and bladder outcomes At the end of follow-up, no patient had urinary stoma. Lower urinary tract symptoms (LUTs) were observed in 7

out of 24 patients (29.1%). Detrusor overactivity and reduced compliance were detected by pressure-ow study in ve patients (20.8%), and they received oxybutinine or other anticholinergic drug therapy (Fig. 2). High post-void urine residue (mean 8%) with increased bladder capacity (350 and 380 ml, respectively) and hypocontractility was recognized in two patients (8.3%), as consequence of bladder myogenic failure. Four patients out of the seven with LUTs were emptying their bladder by a double micturition program with voiding diary, and two of them were on intermittent clean catheterization (24 times/day) in prescholar age (Table 4). Fertility and sexual outcomes One patient with evolutive CRF and a history of previous episodes of epididimitis was azoospermic. No signicant semen abnormality was detected in the other 23 patients (95.8%). Erections were referred as normal in 21 patients

Fig. 1 Severe PUV with CRF at age of 2 months. a, b US: large ureter and dysplastic kidney with hyperechoic parenchyma. c Voiding cystogram: classic PUV, with bladder pseudodiverticula and enlarged

posterior urethra. d DMSA scan: poorly functioning left kidney, enlarged right kidney. The baby underwent transient unilateral ureterocutaneostomy and endoscopic bladder section

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1030 Table 3 Previous surgery in PUV study group Patients Transiente primary vesicostomy or suprapubic catheter Previous ureterocutaneostomy Endoscopic valve ablation Second look (endoscopic) Ureteroneocystostomy Nephrectomy Ureterocystoplasty 7 2 24 19 5 4 2 % 29.1 8.3 100.0 79.1 20.8 16.6 8.3 Dysfunctional voiding Detrusor overactivity

Pediatr Surg Int (2011) 27:10271035 Table 4 Bladder dysfunction and andrological outcomes Patients 7 5 2 2 23 1 21 9 % 29.1 20.8 8.3 8.3 95.8 4.2 87.5 12.5

High urine residue (hypocontractilities) Clean intermittent catheterization Normal semen Azoospermia and epididimitis Normal sexual activity Limited or absent sexual activity

consequence. The spectrum of severity of PUV varies from mild do lethal forms, according to the degree of obstruction [3]. Antenatal diagnosis and post-natal management The widespread diffusion of antenatal ultrasonography over the past 30 years has allowed to suspect PUV in utero in high percentage of cases [15]. In this study group, prenatal US was positive for hydronephrosis and/or large foetal bladder in eight cases (17.7%) out of 45 records (Table 1). Among 34 newborns and infants observed during the 20062009 years at our department, 85% had bilateral hydronephrosis detected prenatally, with classic prenatal features, as oligohydramnios, fetal ascites, or key-hole sign of bladder and bladder neck with poorly emptying bladder and thickened walls were detected in 35% of them (Fig. 5), and the difference between the two groups of pregnancies observed at two decades of distance was signicant (p \ 0.05). The very sick newborn or infant, admitted in Neonatal Intensive Care Unit with severe abdominal distension and septic symptomatology, is less and less frequent nowadays. Post-natal management of PUV needs adequate neonatal and infant care with nephrological support, in order to correct metabolic acidosis and electrolyte imbalance if present, to prevent or treat urinary tract infections and septicaemia that might contribute to further damage of the congenitally obstructed renal parenchyma [16]. Improved management of those severely sick neonates and infants has resulted in a better long-term outcome of children born with PUV in the past two decades [17]. Surgical management of PUV Surgical treatment of PUV is still a challenging procedure in very small infants and newborns, and it may inuence the long-term outcome. Temporary vesical drainage can be achieved by passing a ne feeding tube (5 or 6 F gauge) transurethrally or by a percutaneous suprapubic cystostomic

Fig. 2 Same case of Fig. 1: voiding cystogram showing good bladder decompression at 15 years from valve ablation and low ureterostomy closure with ureteroneocystomy

(87.5%) and with some troubles in 3 (12.5%). Fifteen patients (62.5%) experienced regular intercourses, whereas sexual activity was limited or absent in nine patients with renal insufciency. CRF and ESRD were affecting significantly sexual activity, if compared with patients with normal or adequate renal function (p \ 0.05). Slow ejaculation was referred by two patients. No paternity was reported in the study group (Table 4).

Discussion The most severe obstructive uropathy in paediatric age is represented by PUV, with signicant long-life

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tube. Surgical urinary diversion, either as primary treatment or following transurethral section of valves (vesicostomy or upper tract diversion), should in our opinion be limited to few indications, such as prematurity and small body size babies, severe urinary infections or septicaemia, and highgrade renal insufciency. The presence of a previous urinary diversion in infancy and childhood has been associated with a worse long-term outcome [15]. Anyway, bladder cycling should be ever preserved to maintain or improve bladder wall compliance and capacity. Blockson cutaneous vesicostomy was proposed by Duckett [18] in 1974 as an alternative to primary ablation in neonatal age. The use of high urinary diversion is very controversial, as no controlled studies have been conducted on long-term efcacy: in Krueger and Associates [19] series, infants treated with high-loop cutaneous ureterostomy ultimately had better outcome with regard to renal function and growth than the group managed by primary transurethral ablation of the valves. Conversely, Reinberg et al. [20] demonstrated that mild to severe renal failure developed in 79% of patients who were treated initially by high diversion versus 47% with primary ablation. More recently, further studies documented that urinary diversion does not offer signicant advantage on renal and bladder function at long-term follow-up, if compared with primary valve ablation [2123]. In case of bilateral high-grade reux, low unilateral ureterostomy can be performed on the less damaged kidney, leaving the contralateral side undiverted: the unilateral ureterostomy will allow adequate bilateral drainage and proper bladder cycling will be preserved [8, 24] (Fig. 2). Valve ablation techniques and outcomes Primary transurethral retrograde ablation is the preferred treatment for PUV and was performed in all our patients. The development of smaller endoscopic equipments, as well as improved optic systems, has allowed transurethral endoscopic incision or fulguration of posterior urethral obstruction in virtually all but the most premature patients. In most cases, primary ablation is sufcient to decompress the bladder and the upper urinary tracts. The technique consists not in resection of the valve leaets, but in a full section at 5, 7 and 12 h of the urethral face [8]. Special attention is devoted in order to avoid any injury to the urethral corpus spongiosum, to prevent post-operative urethral stricture or lesion to the external urethral sphincter that could cause the onset of late urinary incontinence [11, 2527]. It is our habit to perform a second endoscopic look at 3 months from primary valve ablation in order to detect any residual urethral obstruction: in this group it was performed in 79.1% of cases (Table 3). Other techniques of valve obstruction removal, as blind transurethral ablation using Fogarty balloon catheter, Mohans Valvotome [15], Whitaker-Sherwood diathermic

hook, or using antegrade access by percutaneous cisto-urethroscopy were not used in our patients. Urethral stricture Urethral stricture is a possible and feared complication after PUV ablation, which can be responsible of a signicant morbidity, as consequence of mechanical trauma on posterior urethra wall. The stricture generally appears late at follow-up, when the lesion on the urethral wall is deeper and the corpus spongiosum could result injured, and is reported to vary from 25 to 0% [2527]. In our series, no posterior urethra secondary stricture was experienced. This iatrogenic complication on long-term follow-up must be avoided, utilizing small endoscopic and delicate transurethral manoeuvres [26, 27] and avoiding to have a dry urethra soon after PUV resection. Pathophysiology of posterior urethral valves PUV are responsible of congenital bladder outlet obstruction that develops very early in foetal life. The high back pressure, prenatally determined, involves a series of sequential secondary pathological changes on the urethra, the bladder, the urinary upper tract and the kidneys. On the lower tract, the prostatic urethra is enlarged and elongated, and the posterior aspect of bladder neck is pronounced (Fig. 3). Into the bladder, wall trabeculations with detrusor hypertrophy and increased collagen component are the main pathological features [68]. On upper tract, ureters are frequently dilated and tortuous, with or without vesicoureteral reuxes. Signicant uretero-vesical junction obstruction is almost uncommon, although reuxes are present from 26 to 72% of PUV [2, 7]. Kidney abnormalities vary from persistent hydronephrosis to renal parenchyma dysplasia. Unilateral renal dysplasia with a non functioning kidney could require nephroureterectomy at long-term follow-up, if responsible of arterial systemic hypertension or signicant UTIs. It could be associated with high-grade ipsilateral VUR with a contralateral well functioning kidney (VURD syndrome) [22] (Figs. 4, 5). Bilateral renal dysplasia is associated with progressive renal function impairment and usually leads to ESRD that requires dialysis and renal transplantation in paediatric or adolescent age [2, 9]. It should be considered that the anatomical and functional changes of the lower and upper urinary tract may persist, although the primary urethral obstruction could be fully solved [9]. Vesico-ureteral reux outcome in PUV Reuxes are almost frequent in patients with PUV, up to 72% [28]. A recent review from Finland reported bilateral

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Fig. 5 Positive prenatal features in two groups of patients with PUV. Years 19821995: 8 patients on 24. Years 20062009: 29 patients on 34 (p \ 0.05)

Fig. 3 Voiding cistourethrogram in a boy with PUV previously resected in infancy: mild dilatation of bladder neck and posterior urethra is still present

VUR in 37% and unilateral in 27% of patients with valves, with a total of 64% [29]. In our group of patients, VUR was present in 62.5% of the cases (15 patients), bilateral in 37.5% (Table 2). Bilateral reux has higher risk of developing CRF or ESRD along time: in our study group, patients with bilateral VUR presented renal insufciency

more frequently than those with unilateral VUR: the incidence of CRF or ESRD was four times higher in bilateral reuxes (29 vs. 8%). The difference was signicant (p \ 0.05) (Table 2). Reux resolution is expected to present high rate with a long follow-up after valve ablation, especially if bladder dysfunction is normalized. In a recent series of Heikkila and Co authors [29], VUR in valve patients had spontaneous resolution in 62% of cases and was related to antireux surgery in 21%, whereas nephroureterectomy was required in 17% of boys. Ureteral reimplantation should be avoided shortly after valve ablation and long-term indications remain still undened [8]. Our recent experience supports a less aggressive surgical approach than in the past.

Fig. 4 Severe right VUR with paraureteral diverticulum and dysplastic kidney, with contralateral normal functioning kidney, as in VURD Syndrome [22] (a VCUG, b Scheme, c US)

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Dysfunctional voiding and urinary incontinence A large number of children and young boys with previous PUV present different degrees of LUTs. Symptoms of bladder overactivity such as urgency, urge incontinence and nocturnal enuresis after the toilet-trained age are often observed [11, 30]. Incontinence was initially considered as secondary to external sphincter incompetence, depending from primary maldevelopment and dilatation of sphincteric urethra, rather than iatrogenic injury during endoscopic manipulation. More recently, urodynamic studies showed signicant lower urinary tract dysfunction, which was persisting after complete urethral obstruction removal [31]. LUTs have been reported to occur in 1338% of valve patients [30]. Daytime wetting is the most common problem. Urodynamic evaluation identied four different patterns of bladder dysfunction in boys with previous PUV: detrusor overactivity, reduced compliance, high voiding pressure and myogenic failure. Bladder overactivity and low compliance are considered as consequence of detrusor hypertrophy and hyperplasia during the foetal age. The risk of progressive upper tract deterioration is signicant if high bladder pressures are present and it should be treated by early aggressive anticholinergic therapy even in infants and young children and continued till necessary [8, 11]. High voiding pressures after valve ablation can be secondary to persisting urethral stenosis or bladder neck obstruction. Endoscopic incision of bladder neck was performed to solve bladder outlet obstruction: incontinence and retrograde ejaculation resulted in some cases at longterm follow-up. The use of alpha-blockers in patients with outow functional obstruction has been offered with encouraging results [31]. In our series, ve patients received alpha-blocker therapy till puberty. Valve bladder syndrome was dened by Mitchell as a condition with progressive upper tract dilatation despite adequate valve ablation, combined with a reduced sensation of bladder fullness and elevated intravesical pressure at low lling volume [32]. This evolutive condition seems as acquired, due to a combination of reduced bladder compliance, reduced bladder sensitivity, increased postvoid residual and polyuria [8, 11, 30]. Along time, bladder contractility and compliance can decrease, leading to partial bladder emptying with increasing residual and inefcient drainage of the progressively dilated upper tract [1, 30, 32]. Polyuria, described in as many as 75% of boys with previous PUV, is related to a reduced tubular ability to concentrate urine, determining increase of urinary output and overload on the urinary tract [33, 34]. Bladder dysfunction in PUV patients is an evolving condition: a initially low capacity, hyperactive and low-

compliant bladder can progressively decompensate, becoming over distended and over compliant with hypocontractility and hyposensitivity. This situation is dened as myogenic failure, with weak bladder contractions and poorly emptying urinary tract [31, 35]. A decompensated bladder with myogenic failure requires serial urodynamic studies with pressure-ow analysis and evaluation of postvoid urine residual. Appropriate urotherapy or even intermittent catheterization could be necessary if a decompensated bladder with myogenic failure is diagnosed [15]. Recently, overnight indwelling catheter, transurethral or by continent appendicostomy, has been suggested to decompress upper tract, preserve renal function and achieve dryness [24]. Fertility and andrological outcomes The posterior urethra can remain elongated and dilated in PUV patients, despite successful valve ablation. Bladder neck and sphincteric lesion may lead to retrograde ejaculation. Scarring of the posterior urethra secondary to valve ablation, reux into the seminal vesicles and the ejaculatory ducts, and cryptorchidism, which has been referred as occurring on 16% of PUV patients [36], are further factors accounting for a possible reduced fertility. Woodhouse et al. [12] reported high incidence of slow ejaculation, imputed to the persistence of a dilated posterior urethra. No evidence of retrograde ejaculation was found. Erections were found normal. More recently, a series of European studies presented at the 22nd ESPU Annual Meeting [3739] demonstrated that ejaculatory and erectile dysfunction is uncommon in the long-term follow-up, and no retrograde ejaculation was observed. Pyospermia has been reported in semen of a subpopulation of young men with a history of PUV and severe lower urinary tract symptoms [40], but it seems to be not frequent. In our study group, we observed 1 azoospermia in a patient with severe CRF, but no other signicant andrological abnormalities.

Conclusions Congenital obstruction of male urethra is a spectrum of lesions, and classic PUV are the commonest. Valves still represents a severe disease in infancy and childhood: mortality rate has signicantly decreased in the last 30 years, from 50% to less than 10% of patients, due to signicant improvements on prenatal diagnosis, intensive care in newborn and infant age and on management of ESRD [41]. The long-term prognosis of PUV presenting intermediate severity appeared to be improved by prenatal diagnosis [42], and surgical complications of valve ablation

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Pediatr Surg Int (2011) 27:10271035 15. Sudarsanan B, Nasir AA, Puzhankara R, Kedari PM, Unnithan GR, Damisetti KR (2009) Posterior urethral valves: a single center experience over 7 years. Pediatr Surg Int 25(3):283287 16. Glassberg KI, Horowitz M (2002) Urethral valve and other anomalies of the male urethra. In: Belmon B, King LR, Kramer SA (eds) Clinical pediatric urology. Dunitz, London, pp 899945 17. Sarhan O, El-Dahshan K, Sarhan M (2010) Prognostic value of serum creatinine levels in children with posterior urethral valves treated by primary valve ablation. J Pediatr Urol 6(1):1114 18. Duckett JW Jr (1974) Cutaneous vesicostomy in childhood. The blocksom technique. Urol Clin North Am 1(3):485495 19. Krueger RP, Hardy BE, Churchill BM (1980) Growth in boys and posterior urethral valves primary valve resection versus upper tract diversion. Urol Clin North Am 7(2):265272 20. einberg Y, de Castano I, Gonzalez R (1992) Inuence of initial therapy on progression of renal failure and body growth in children with posterior urethral valves. J Urol 148(2 Pt 2): 532533 21. Walker RD, Padron M (1990) The management of posterior urethral valves by initial vesicostomy and delayed valve ablation. J Urol 144(5):12121214 22. Smith GH, Canning DA, Schulman SL, Snyder HM 3rd, Duckett JW (1996) The long-term outcome of posterior urethral valves treated with primary valve ablation and observation. J Urol 155(5):17301734 gli D, 23. Farhat W, McLorie G, Capolicchio G, Khoury A, Ba Merguerian PA (2000) Outcomes of primary valve ablation versus urinary tract diversion in patients with posterior urethral valves. Urology 56(4):653657 24. Ransley PG. (2009) Personal communication 25. Lal R, Bhatnagar V, Mitra DK (1998) Urethral strictures after fulguration of posterior urethral valves. J Pediatr Surg 33(3): 518519 26. Bruce J, Stannard V, Small PG, Mayell MJ, Kapila L (1987) The operative management of posterior urethral valves. J Pediatr Surg 22(12):10811086 27. Nijman RJ, Scholtmeijer RJ (1991) Complications of transurethral electro-incision of posterior urethral valves. Br J Urol 67(3):324326 28. Dinneen MD, Duffy PG (1996) Posterior urethral valves. Br J Urol 78(2):275281 J, Rintala R, Taskinen S (2009) Vesicoureteral reux in 29. Heikkila conjunction with posterior urethral valves. J Urol 182(4): 15551560 30. Koff SA, Mutabagani KH, Jayanthi VR (2002) The valve bladder syndrome: pathophysiology and treatment with nocturnal bladder emptying. J Urol 167(1):291297 31. Wen JG, Li Y, Wang QW (2007) Urodynamic investigation of valve bladder syndrome in children. J Pediatr Urol 3(2):118121 32. Mitchell ME (1982) Persistent ureteral dilatation following valve resection. Dial Pediatr Urol 5:811 33. Dinneen MD, Duffy PG, Barratt TM, Ransley PG (1995) Persistent polyuria after posterior urethral valves. Br J Urol 75(2): 236240 34. Kajbafzadeh AM, Payabvash S, Karimian G (2007) The effects of bladder neck incision on urodynamic abnormalities of children with posterior urethral valves. J Urol 178(5):21422147 (discussion 21472149) 35. De Gennaro M, Capitanucci ML, Silveri M, Morini FA, Mosiello G (2001) Detrusor hypocontractility evolution in boys with posterior urethral valves detected by pressure ow analysis. J Urol 165(6 Pt 2):22482252 36. De Gennaro M, Capitanucci ML, Mosiello G, Caione P, Silveri M (2000) The changing urodynamic pattern from infancy to adolescence in boys with posterior urethral valves. BJU Int 85(9):11041108

have been signicantly reduced by the introduction of small-calibre paediatric operative scopes and delicate endoscopic manoeuvres [43]. Nevertheless, long-term morbidity related to PUV still represents a heavy burden for these patients. In particular, progression to CRF and ESRD, development of arterial hypertension, evolution of LUTS and bladder dysfunction, and fertility are all issues that need to be addressed since infancy and require adequate follow-up into puberty and adulthood. Although prognosis has been improved in the past two decades, patients born with PUV are committed to life-long nephrological and urological follow-up, from early after valve ablation till adolescence and adulthood.
Conict of interest No competing nancial interests exist.

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