P. López Pereira

Posterior urethral valves: prognostic factors

To determine which variables besides bladder dysfunction can help to predict the outcome of renal function in boys with posterior urethral valves (PUV).

Bladder dysfunction as a prognostic factor in patients with posterior urethral valves

Objective To investigate the extent to which different types of bladder dysfunction can affect long‐term renal function in boys with posterior urethral valves (PUV).

SHOULD WE TREAT VESICOURETERAL REFLUX IN PATIENTS WHO SIMULTANEOUSLY UNDERGO BLADDER AUGMENTATION FOR NEUROPATHIC BLADDER

PURPOSE If high pressure is responsible for vesicoureteral reflux in neurogenic bladders, eliminating the high pressure should resolve reflux in noncompliant bladders. Nevertheless, the need for simultaneous ureteral reimplantation and bladder augmentation remains controversial in patients with a noncompliant bladder and vesicoureteral reflux. MATERIALS AND METHODS Bladder augmentation was performed in 8 boys and 8 girls with a noncompliant bladder and vesicoureteral reflux at a mean age of 10 years (range 2 to 17) because they had not responded satisfactorily to clean intermittent catheterization and anticholinergic therapy alone. No effort had been made to correct reflux surgically in these patients. Before bladder augmentation reflux was grade II to III in 4 ureters (3 patients) and IV to V in 18 (13). The bladder was augmented with intestine in 14 patients and with ureter in 2. Mean followup was 5.2 years (range 2.8 to 7.5). RESULTS After bladder augmentation bladder compliance improved in all patients. Of the 18 ureters with high grade reflux 2 were used for bladder augmentation, and reflux resolved in 13, was downgraded in 1 and persisted in 2. Of the 4 ureters with low grade reflux, reflux disappeared in 2 and was down graded in 2. The rate of high and low grade vesicoureteral reflux resolution or improvement was 87.5% and 100%, respectively. At the end of the study only 3 patients had persistent reflux, which was downgraded in 2. No urinary tract infections developed in any patient and none was receiving chemoprophylaxis 6 months postoperatively. CONCLUSIONS Our experience indicates that antireflux procedures are not routinely needed in patients with a noncompliant bladder and associated vesicoureteral reflux who undergo bladder augmentation.

Initial and long-term management of posterior urethral valves.

Posterior urethral valves are the most common cause of congenital obstructive uropathy, resulting in renal failure in childhood. Nowadays, in most cases, diagnosis is suggested by antenatal ultrasound. However, antenatal intervention has not resulted in a significantly improved outcome. Endoscopic valve ablation is the initial treatment in most of these neonates, but others procedures, like vesicostomy or ureterostomy, can also be justified in some particular cases in order to improve renal function prognosis. Different factors like bladder dysfunction, VUR, polyuria and proteinuria, can be responsible for the slow and progressive deterioration in renal function that some of these patients show over the years. By treating them all, we may prevent or delay the onset of end stage renal disease.

DOES NEONATAL PYELOURETEROSTOMY WORSEN BLADDER FUNCTION IN CHILDREN WITH POSTERIOR URETHRAL VALVES

PURPOSE We determine whether long-term bladder dysfunction is more frequent in children with posterior urethral valves who undergo early supravesical urinary diversion (pyeloureterostomy) than in those who undergo valve ablation. MATERIALS AND METHODS Urodynamic studies were performed in 59 boys with severe posterior urethral valves who were divided into 2 groups based on initial treatment of valve ablation (30) and bilateral pyeloureterostomy (29). RESULTS Of the 59 boys 25 (42%) had a normal bladder, including 11 with an over distended bladder and 34 (58%) had bladder dysfunction, including instability in 22, poor compliance in 9 and myogenic failure in 3. Of the 30 boys initially treated with valve ablation 14 (46.6%) had a normal bladder, including 7 with an over distended bladder and 16 (53.4%) had bladder dysfunction, including instability in 10, poor compliance in 4 and myogenic failure in 2. Of the 29 boys initially treated with temporary bilateral pyeloureterostomy 11 (38%) had normal bladders, 4 with an over distended bladder and 18 (62%) had bladder dysfunction, including instability in 12, poor compliance in 5 and myogenic failure in 1. There were no statistically significant differences in the number or type of bladder dysfunction between the diversion and valve ablation groups. CONCLUSIONS Therefore, temporary pyeloureterostomy did not affect bladder function adversely in the long term.

Enterocystoplasty in children with neuropathic bladders: long-term follow-up.

OBJECTIVE This study assesses clinical outcome, after at least 8 years, of augmentation done before or at puberty in neuropathic bladders. PATIENTS AND METHODS A total of 29 children with neuropathic bladders who did not respond satisfactorily to clean intermittent catheterisation and anti-cholinergic therapy underwent enterocystoplasty at a mean age of 11.8 years (range 3-18). Twenty-one children (72.4%) had vesicoureteral reflux (VUR) and/or ureterohydronephrosis and 22 (75.8%) had dimercapto-succinic acid scars, but all had normal renal function. All patients were followed at regular intervals with urinary tract imaging, serum electrolytes, creatinine, urodynamic evaluation and 24-h urine collection. Urine cytology, cystoscopy and biopsy were performed at the end of follow-up. RESULTS Mean follow-up was 11 years (range 8-14.5) and mean age at the end of follow-up was 22.2 years (range 13.2-31). Urodynamic studies showed a significant improvement in bladder compliance in all patients. Upper urinary tract dilatation disappeared in all, VUR in 13/17 (76.4%), and no new renal scarring occurred in any patient. At the end of follow-up, renal function was normal in all according to serum creatinine, but cystatin C levels were normal in 27 and elevated in two. Significant proteinuria and low concentrations of renin and aldosterone were present in 80% and 82%, respectively. Only one patient had urinary tract infection, three had bladder stones, and in another a catheterisable channel was made. All patients were dry with normal urine cytology and cystoscopy, and no malignant lesions have been found in the biopsy specimens. CONCLUSION Enterocystoplasty has preserved renal function and resolved VUR and/or hydronephrosis in most patients. The future implications of proteinuria and the low serum levels of renin and aldosterone, as well as the best indicator for measuring renal function, have yet to be determined. Close, life-long follow-up, including cystoscopy, is necessary to prevent complications.

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

OBJECTIVE Although 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 METHODS Between 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. RESULTS Mean 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/m(2) in the AC group and 88.17 ± 28.20 ml/min/m(2) 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). CONCLUSIONS Bladder 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.

Renal Transplantation in Augmented Bladders

Not many years ago, children with congenital abnormalities of the lower urinary tract or with bladder dysfunction were denied renal transplantation because they were considered very high-risk recipients. However, in the past few decades, we learned that in children with poorly compliant, low-capacity bladders, augmentation cystoplasty (AC) can create a compliant, low-pressure reservoir that helps preserve the kidney graft. Although the incidence of symptomatic urinary tract infection (UTI) may be greater in pediatric transplant recipients with an AC than in those without, UTI is related more to noncompliance with clean intermittent catheterization or vesicoureteral reflux to the native kidney or graft than to the AC itself, and usually does not lead to impairment of graft function. Today, children with a bladder reconstruction may undergo transplantation with the same outcome (graft survival and function) as those with normal bladders, although there is some possibility of malignant transformation in the intestinal segment used to augment the bladder in these patients.

Bilateral laparoscopic nephrectomy with simultaneous peritoneal dialysis catheter implantation.

Abstract:   Bilateral nephrectomy prior to transplantation is indicated in some patients with end‐stage renal disease. The indications for bilateral nephrectomy include persistent heavy proteinuria, refractory hypertension, and urinary tract infections. We report an eight‐month‐old baby with male pseudohermaphroditism and renal failure secondary to diffuse mesangial sclerosis. While awaiting renal transplantation, dialysis became necessary and the child presented standard drug therapy‐resistant hypertension. A bilateral nephrectomy was performed simultaneously to peritoneal dialysis catheter implantation by using laparoscopy. At the present time, the patient is doing well with ambulatory dialysis and all antihypertensive medication has been discontinued. We recommend this technique in children who require bilateral nephrectomy and peritoneal dialyisis. Not only is it somewhat less aggressive than traditional open surgery, but it also reduces post‐operative pain, allows earlier initiation of peritoneal dialysis, and improves cosmetic appearance.

Double renal transplantation--a strategy with donors under 3 years old.

OBJECTIVE To evaluate the transplantation of both kidneys from donors under 3 years old to older pediatric recipients, in order to provide adequate renal mass and improve graft survival. PATIENTS AND METHODS Seven patients received a double kidney transplant from a donor under 3 years old (mean age 2.7 years). The primary renal disease was obstructive uropathy in two patients, and hemolytic uraemic syndrome, tuberous sclerosis, nephronoptisis, reflux nephropathy and nephrotic syndrome (one patient each). Recipients were 14-18 years old and had a mean height of 165.2 cm. Five were in dialysis and it was the second transplantation for two. Both kidneys were sequentially placed in the same iliac fossa; the first was placed the most cranially with the second placed caudal to the first. The surgical technique was similar to that used to place a single graft. Immunosuppression induction employed monoclonal antibodies followed by a triple therapeutic regime (mofetyl micofenolate/steroids/tacrolimus). RESULTS Diuresis was started immediately with all the grafts and there was no thrombosis in any patient. One patient lost both grafts due to recurrence of her primary disease. Another patient developed lymphocele 1 year post transplant and required laparoscopic drainage. The grafts in six patients are doing well after a follow up of 1 to 7 years. CONCLUSION Double transplant of both kidneys from a donor under 3 years old can provide sufficient renal mass for adequate renal function and does not present a higher risk of complications.