A third of patients with PUV will develop end-stage renal disease (ESRD) during childhood or young adulthood despite active treatment [8, 9] progressing to transplant in 15.8 % of children [10]. Warshaw et al. in their study of 22 patients found that more than half of patients developed renal failure either in infancy or in adolescence [11]. They observed that renal dysplasia plays a predominant role in the early onset of renal failure. Lal et al. in their study of 84 patients treated for PUV and followed up for 1–21 years found that 60.5 % of them had maintained normal serum creatinine values through adolescence and adulthood, and in particular 14.3 % of them had developed chronic renal failure (CRF) between the ages of 12 and 18 years [9]. Very few long-term studies exist to present true prevalence of need for renal replacement therapy or transplant in middle and later adulthood owing to the lack of consistency of databases beyond pediatric life.

Hyperfiltration injury due to the increased physiological demands during puberty is postulated to be one of the causes of renal function deterioration, and proteinuria is a very good indicator of impending CRF [12]. Jnakinen et al. have shown that there is a negative impact on Quality of Life (QoL) particularly in children with renal insufficiency due to PUV [13].

There is data to suggest that children with PUV have a twofold to threefold risk of developing lower urinary tract symptoms (LUTS) as adults [14]. Mitchell coined the term “valve bladder” in 1982 to describe the phenomenon of obstruction to the intramural ureter in PUV patients [15]. The three components of the valve bladder syndrome include dysfunctional renal units, dilated refluxing ureters, and the chronically distended bladder. Bladder dysfunction with progression through childhood and into adulthood has been found to be contributory to the worsening of renal function [8, 14, 16].

Urodynamic assessment in children treated for PUV have found bladder dysfunction in up to 75 % of the patients. Three predominant patterns found were: detrusor overactivity, poor compliance, and myogenic failure [17–19]. It is generally postulated that an increase in bladder capacity with age is responsible for the transition from detrusor overactivity to myogenic failure after puberty [19, 20]. Detrusor overactivity, poor compliance, and polyuria secondary to CRF are responsible for episodes of incontinence, which causes sleep disturbances as well negatively impacts on social functioning [13].

Anticholinergics play an important role in managing the symptoms of detrusor overactivity, especially in conjunction with bladder augmentation in patients with poorly compliant bladder. Anticholinergics may improve both bladder compliance and sensation. The Mitrofanoff continent catheterizable channel may be considered for these patients because of the relative ease of clean intermittent self catheterisation (CISC) in comparison to the urethral route, thereby improving patient compliance [21, 22]. Intradetrusor injections of botulinum toxin A may be considered a useful treatment modality to temporarily bridge the gap between anticholinergics and bladder augmentation.

Intestinal segments are generally used for bladder augmentation with the associated risks of hyperchloremic metabolic acidosis, bacteriuria, and malignancy. The risk of malignancy is said to be between 1.2 and 2 % [23, 24] after a 10-year period from operation to diagnosis, and hence the ureter may be considered as a substrate for augmenting the bladder in selected patients needing nephroureterectomy due to persistent infection secondary to unilateral nonfunctioning kidneys with chronic dilated ureters [25].

Detrusor myomectomy may reduce the intravesical pressure but does not increase bladder capacity and durability may be limited, both of which limit usefulness of this treatment [26]. Use of a gastric segment (gastrocystoplasty) is not recommended due to haematuria–dysuria syndrome [27].

In cases of detrusor failure, the management options include timed voiding, double voiding, and CISC to ensure the bladder is kept empty and avoid bladder stones and lower urinary tract infections, CISC can however be very painful in these patients since they often have a redundant posterior urethra which makes catheterization technically difficult. Hence, a Mitrofanoff may be preferred [26].

Risk factors for decreased sexual function and fertility in PUV patients include CRF [28, 29], abnormal prostatic urethra [30], crypto-orchidism [31, 32], leukospermia [33], and recurrent epididymo-orchitis [34–36].

Decreased libido and sexual activity are among the adverse effects of CRF [37] with ED reported in 70 % of men with chronic kidney disease [38]. This is an important risk factor as a third of patients will progress to ESRD [8, 9].

Surgery for crypto-orchidism is seen in up to 16 % of patients and the resultant reduced testicular size and testosterone levels can cause reduced sexual function [39]. However only men with bilateral crypto-orchidism have been shown to have reduced fertility than men with unilateral crypto-orchidism [40].

Recurrent epididymo-orchitis with or without secondary leukospermia is thought to be a result of reflux to dilated ejaculatory ducts due to the increased pressure in the posterior urethra [34, 35, 41, 42].

There is conflicting evidence in the literature regarding fertility in PUV patients. Some studies have shown decreased fertility [43–45] while the largest study to date has shown no difference in fertility and ED between PUV patients and the general population [46].

The ability to follow the progress of young patients as they develop is paramount in attempting to understand the long-term outcomes of treatments. The only way to achieve this is to establish good quality longitudinal care for patients as they leave pediatric practice and enter adult healthcare. Young males with treated PUV need regular follow-up with measurement of urea and electrolytes, blood pressure and USS to assess the upper tracts. Periodic urodynamic monitoring, particularly when new voiding problems (retention, UTI, stones) appear or imaging and serum Cr suggest worsening renal function, is necessary.

Health education forms an integral part of Transitional care. As young patients move on into adolescence, there is a high risk of noncompliance with treatments and health education should be included to ensure compliance with e.g. medications (anticholinergics, antibiotics), voiding strategies, intermittent self catheterization/bladder washouts, and attendance at follow-up clinics. Loss of renal function with progression through adolescence is well demonstrated and not solely explained by noncompliance of teenagers. The ItalKid study authors suggested that the possible mechanisms for this finding are: (1) imbalance between glomerular capacity and increased body mass, or (2) an effect related to the changing sex hormone environment with progression [47]. In addition, adolescence and young adulthood also represent a time of sexual growth and experimentation, and for young men with PUV, erectile dysfunction, ejaculatory issues, and fertility are of paramount concern. Timely and appropriate transfer to a suitable adult urologist becomes paramount as patients grow up to enable uninterrupted lifelong follow-up, sometimes in conjunction with other medical professionals e.g. the nephrologist. The medical consequences of a patient with such needs being lost in follow-up are potentially serious and often difficult to rectify when considerable time has elapsed since the patient was last seen.