The modern staged repair of bladder exstrophy in the female: a contemporary series

ObjectiveMany changes have occurred in the treatment of bladder exstrophy over the last few years and several repairs are now offered, but there is a lack of long-term follow-up data. The purpose of this study was to evaluate long-term outcomes in a select group of female patients in whom modern staged repair was undertaken.Patients and methodsFrom an institutionally approved database 41 patients were identified. All had undergone primary bladder exstrophy closure in 1988–2005, at a mean age of 2 months (range 4 h to 3 months), with or without an osteotomy by a single surgeon, and all were followed up for a minimum of 5 years. Twelve patients underwent osteotomy at the time of primary closure. Eight had a classic transverse innominate and vertical iliac osteotomy, and four a transverse innominate only. Mean age at the time of bladder neck repair (BNR) was 4.2 years (39–65 months). Mean measured bladder capacity under gravity cystograms at the time of repair was 109 cc (80–179 cc).ResultsThirty patients (74%) were continent day and night, and voiding per urethra without augmentation or intermittent catheterization. Social continence, defined as dry for more than 3 h during the day but damp at night, was found in a further four cases (10%). Seven patients are completely incontinent with dry intervals of less than 1 h day and night. The mean time to daytime continence was 12 months (4–16 months) and to night-time continence was 19 months (10–28 months). Patients with a mean capacity greater than 100 cc had better outcomes. Six of the 30 patients achieved dryness after primary closure only, and all six had transverse innominate and vertical iliac osteotomy at the time of primary closure.ConclusionsFemale classic exstrophy patients with a good template who develop adequate capacity after a successful primary closure can achieve acceptable continence without bladder augmentation and intermittent catheterization. A select group will develop continence with closure alone without the need for bladder neck repair.     

Long-term follow up (37–69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: Uro-nephrological outcome

ObjectiveTo describe the urological and nephrological long-term outcome of patients born with classical bladder exstrophy treated with bilateral ureterosigmoidostomies in early childhood.Patients and methodOut of 42 patients born with bladder exstrophy in Switzerland between 1937 and 1968, 25 participated in this study; seven had died, seven were lost to follow up and three refused consent. Assessment included chart review, clinical examination, and assessment of renal function and morphology.ResultsAfter a follow-up period of 37–69 years ((mean 50 years), 13 of the 25 participants (52%) had their ureterosigmoidostomy still in place. All others had different forms of urinary diversions. Fifteen (60%) patients had normal renal function or mild chronic kidney disease as assessed by estimated glomerular filtration rate. Three patients were on renal replacement therapy. MRI (n = 16) showed 10 morphologically normal kidneys. One patient suffered from adenocarcinoma of the colon, five had benign colonic polyps, one urethral papillary carcinoma and 18 no evidence of tumor.ConclusionThe majority of our patients have normal or mildly impaired renal function and a well functioning ureterosigmoidostomy. This is remarkable, given the fact that ureterosigmoidostomies are considered to be refluxing high-pressure reservoirs at risk of renal injury and malignancy.     

Modern staged repair of bladder exstrophy: a contemporary series

ObjectiveMany changes have occurred in the treatment of bladder exstrophy over the last few years and many repairs are now offered. The purpose of this study was to evaluate long-term outcomes in a select group of patients in whom modern staged repair (MSRE) was undertaken.Patients and methodsFrom an institutionally approved database were extracted 189 patients who had undergone primary closure between 1988 and 2004. The records of 131 patients (95 males) who underwent MSRE with a modified Cantwell-Ransley repair by a single surgeon in 1988–2004 were reviewed with a minimum 5-year follow up.ResultsSixty-seven patients with a mean age of 2 months (range 6 h to 4 months) underwent primary closure, and 18 underwent osteotomy at the same time. Mean age at epispadias repair was 18 months (8–24). Mean age at bladder neck reconstruction (BNR) was 4.8 years (40–60 months) with a mean capacity of 98 cc (75–185). Analysis of bladder capacity prior to BNR revealed that patients with a mean capacity greater than 85 cc median had better outcomes. Seventy percent (n = 47) are continent day and night and voiding per urethra without augmentation or intermittent catheterization. Social continence defined as dry for more than 3 h during the day was found in 10% (n = 7). Six patients required continent diversion after failed BNR. Seven patients are completely incontinent. The mean time to daytime continence was 14 months (4–23) and the mean time to night-time continence was 23 months (11–34). No correlation was found between age at BNR and continence.ConclusionsPatients with a good bladder template who develop sufficient bladder capacity after successful primary closure and epispadias repair can achieve acceptable continence without bladder augmentation and intermittent catheterization.     

Does bladder augmentation stabilize serum creatinine in urethral valve disease? A series of 19 cases

ObjectiveThis study evaluates the results of bladder augmentation (BA) in 19 boys with posterior urethral valves, especially as regards its efficacy in stabilizing serum creatinine.Patients and methodsIn the period 1995–2005, 188 patients with urethral valves were surgically managed. Nineteen of these had undergone BA as a part of their surgical management after initial endoscopic valve ablation in 15 and diversion in four boys. The mean serum creatinine at the time of BA was 2.11 mg/dl.ResultsBA stabilized the serum creatinine in 14 but failed to do so in five boys. A serum creatinine level of more than 2 mg/dl at the time of BA was associated with a significantly worse rate of success. BA as part of an undiversion procedure in three boys was unsuccessful.ConclusionIn an economic milieu where renal transplantation is not available for the majority of deserving children, careful selection is required before BA is considered as a surgical solution for the valve bladder. BA, when otherwise indicated, has been beneficial in children with pre-augmentation creatinine up to 2 mg/dl.     

Single-stage repair of bladder exstrophy in older children and children with failed previous repair

AimEvaluation of cosmetic and functional outcome of single-stage exstrophy–epispadias complex repair in older children and those with previously failed repair.Materials and methodsThis study comprised 15 children (12 boys and 3 girls) with classic bladder exstrophy and a mean age at repair of 8.6 months (range 2–24 months). Eight children had a previously failed repair. All children underwent complete primary repair using the single-stage Mitchell technique. Half of the boys had complete penile disassembly, while in the others a modified Cantwell–Ransley technique for epispadias repair was used. Anterior iliac osteotomy was performed and hip spica used for immobilization in all children.ResultsOne child had urethral stricture treated by endoscopic visual urethrotomy. Three children had penopubic fistulae that closed spontaneously. No bladder dehiscence or prolapse was encountered. Vesicoureteral reflux was present in 20 renal units but ureteral reimplantation was not performed. Average bladder capacity after closure was 134 cm³ (range 110–160 cm³) with only two partially continent and six incontinent children. Mean follow-up period is 2 years (range 1–3 years).ConclusionsSingle-stage repair was performed in children with previously failed repair and those presenting at an older age with satisfactory results. Acceptable bladder and genital anatomy and function were achieved together with preservation of renal function. The impact of this technique on continence is not encouraging, but needs to be determined in a longer follow-up period.     

Urodynamic changes in patients with anterior urethral valves: before and after endoscopic valve ablation

PurposeTo retrospectively review a series of children with anterior urethral valves (AUV), with emphasis on patterns of urodynamic change and long-term outcome of endoscopic treatment.Patients and methodsWe reviewed the medical records of eight patients who had undergone thorough radiological and urodynamic exams before and after treatment. The diagnosis of AUV was based on radiological imaging and confirmed by urethrocystoscopy. The valves were ablated through either transurethral fulguration or resection. The upper urinary tracts were studied by renal scan and ultrasonography before and after the procedure. Bladder function was assessed urodynamically 3 months after surgery. Uroflowmetry was performed as soon as the children were toilet trained.ResultsEndoscopic ablation of AUV was successful in all cases and no surgical complications occurred. The initial symptoms resolved in all boys. VUR disappeared in two out of three patients, and five children had bladder trabeculation that was resolved after surgery. The final outcome was successful in seven patients (88%). The major urodynamic dysfunction was bladder hypercontractility that resolved following valve ablation. The mean maximum voiding detrusor pressure (P_detmax) decreased from 213.2 ± 17.9 cmH₂O to 80.7 ± 9.9 cmH₂O, 6 months after treatment (P < 0.001). None of the patients had low-compliant bladder, detrusor instability or myogenic failure. The voiding pattern in all toilet-trained patients was staccato and of an interrupted shape prior to surgery, but changed to a normal bell-shaped voiding pattern following valve ablation.ConclusionAUV should be considered in the differential diagnosis of patients presenting with infravesical obstruction. We recommend endoscopic valve ablation as the treatment of choice.     

Perioperative anesthetic and analgesic management of newborn bladder exstrophy repair

ObjectiveReconstruction of bladder exstrophy in newborn infants requires immobilization, sedation and pain management to prevent distracting forces from compromising the repair. We present a 6-year review of our experience.Subjects and methodsWe reviewed the perioperative management of newborn infants undergoing reconstruction between November 1999 and October 2006. Data are presented as means ± SD.ResultsTwenty-three newborn infants underwent surgery under a combined epidural and general anesthetic technique. Tunneled caudal epidural catheters were inserted in all patients and intermittently injected with 0.25% bupivacaine with 1:200,000 epinephrine. Postoperatively, a continuous infusion of 0.1% lidocaine, 0.8–1 mg/kg/h was administered for 15 ± 8 (range 4–30) days. Children were sedated with diazepam for 20 ± 13 (range 2–40) days. Central venous catheters were maintained for 20 ± 9 (range 1–34) days for fluids, drug administration and blood sampling. No patient experienced bladder prolapse or wound dehiscence.ConclusionPerioperative management with tunneled epidural and central venous catheters in newborn infants with bladder exstrophy facilitates immobilization, analgesia and sedation, resulting in an excellent cosmetic repair with no case of bladder prolapse or wound dehiscence.     

Button vesicostomy: a continent urinary stoma

ObjectiveA modified technique of vesicostomy is described using a gastrostomy button, which could be used as a continent urinary stoma in children with incomplete voiding.Patients and methodsFrom 1998 to 2005, 21 children aged between 4 days and 16 years underwent insertion of button vesicostomy to permit bladder drainage. They had incomplete bladder emptying and clean intermittent urethral catheterization (CIC) could not be established. In six of 23 procedures, the button was placed through a classical vesicostomy (3) or via a suprapubic catheter tract (3). In 17, a standardized technique of button vesicostomy stoma formation was used. The median follow up was 2.5 years (0.75–8 years).ResultsPatients were selected on the basis of clinical need. Idiopathic hypotonic bladder was the most common indication (9), followed by anorectal malformation (5), neuropathic bladder and posterior urethral valves (2 each), traumatic rupture of urethra (1), visceral myopathy (1) and posterior urethritis (1). Granuloma formation around vesicostomy button was observed in five patients. Local infection was observed in three patients and urinary tract infection in four. No peri-button leakage occurred in the standardized button stomas but was seen in all three of the buttons placed in classical vesicostomies, and transient leakage occurred in one of the three patients with a button placed via a suprapubic catheter tract. The median duration of use of vesicostomy button was 11 months (2–30 months). In eight patients, bladder function improved and intermittent drainage was no longer required. Three patients are still using the button, four progressed to Mitrofanoff, four started CIC per urethra, and two reverted to continuous drainage.ConclusionsButton vesicostomy is a useful addition to the options available for a catheterizable continent urinary stoma in children in the short or medium term. The risk of major complications was low although minor complications were common, and the technique was well accepted by patients and parents.     

Bladder augmentation and artificial sphincter implantation: urodynamic behavior and effects on continence

ObjectiveTo quantify changes in bladder capacity, pressure and compliance after isolated bladder augmentation or augmentation associated with implantation of an artificial sphincter, and to compare the various types of augmentation.Patients and methodsPreoperative and postoperative urodynamic studies were performed in a group of 38 patients (18 males and 20 females; age range 2–19 years), who underwent a type of bladder augmentation.ResultsThe bladder improved in capacity in all patients (mean values: initial 137 ml, final 336 ml, individual increase 229 ml; 434%) except two, in which the augmentation was done with ureter. The mean pressure improved (initial 32 cm of H₂O, final 14, decrease per patient 18 cm of H₂O; 49%). The curve of compliance, progressively increasing typical of hyperreflexia and poor compliance, present in 70% of the cases preoperatively, improved in 78% cases postoperatively, although there were several different patterns. Urodynamic behavior was analyzed with regard to the tissue used for augmentation (ileum, ureter or sigmoid colon). In the sigmoid colon group, there were no significant differences in the urodynamic behavior of the bladder neo-reservoir in relation to the configuration used.ConclusionWith bladder augmentation comes an increase in bladder capacity, a reduction in pressure, and an improvement in compliance and continence. The level of change in capacity, pressure and compliance varies with the tissue used and the length and caliber of the insert. When the procedure is carried out using sigmoid colon tissue, there are no noteworthy differences among the various possible configurations.     

Urodynamic investigation of valve bladder syndrome in children

ObjectiveTo investigate urodynamic manifestations and their relationship with the postoperative experience of children with valve bladder syndrome (VBS).MethodsIncluded were 16 children (mean age 3.2 ± 1.8 years) with VBS, who were divided into two groups. The urodynamic study was performed less than 1 year in group 1 (seven boys, aged 1–1.9 years) and more than 1 year in group 2 (nine boys, aged 2.9–6.5 years) after urethral valve fulguration; at the time of operation patients were less than 2 years old. Standards of the International Children's Continence Society were respected, and results were compared between the two groups.ResultsCompared to group 1, group 2 showed a significant decrease in maximum detrusor voiding pressure (Pdet.void.max) and bladder compliance (BC), and an increase in post-voiding residual (PVR) and maximum bladder capacity (MBC) (p < 0.05), but the difference in detrusor instability was not significant (p > 0.05), Pdet.void.max and PVR were 56.2 ± 14.1 cmH₂O and 96.6 ± 52.4 ml, respectively, in group 2, and there were more intermittent detrusor contractions during voiding in this group.ConclusionPatients with VBS frequently present with multiple bladder dysfunctions that can be diagnosed accurately using urodynamics. Even after urethral valve fulguration Pdet.void.max and BC were inclined to decrease, while PVR and MBC increased with the growth of the children.     

Outcome of complete primary bladder exstrophy repair: Single-center experience

ObjectiveReconstruction of bladder exstrophy remains a challenge. We evaluated our experience with complete primary repair in classic bladder exstrophy.MethodsA retrospective data review was conducted of bladder exstrophy patients presenting at our institution between May 2000 and September 2007. Fifty-one patients (21 females and 30 males) with classic bladder exstrophy were included. Age of presentation ranged from 24 h to 14 months. Mean follow up was 3 years (1 month–7 years). Patients were evaluated for continence, upper tract dilatation and cosmetic result.ResultsEight patients (15.6%) had failed closures and six (11.7%) had fistulae. Evaluation of continence excluded 16 patients not followed up at our center. Thirty-seven percent were continent on clean intermittent catheterization after the age of 5 years. Patients became dry only after augmentation cystoplasty. Upper tract changes were mild during our study with all patients having normal serum creatinine.ConclusionPatients may require more than one procedure for reconstruction. In our series, augmentation was required to achieve acceptable dryness. Early promising results with dry intervals in young patients did not translate to continence in older patients.     

Influence of type of conduit and site of implantation on the outcome of continent catheterizable channels

ObjectiveContinent catheterizable channels (CCC) using the Mitrofanoff principle are essential for pediatric urinary tract reconstruction. There is controversy over the influence of type of CCC (appendix vs. Yang–Monti) and site of implantation (augmentation vs. native bladder) on outcome.Patients and methodsA retrospective record review was conducted of all patients undergoing CCC since 1999, excluding patients who underwent seromuscular colocystoplasty. We analyzed the type of channel, site of implantation, complications requiring re-operation, and the revision rate according to type of CCC, type of stoma, site of implantation (bladder vs. augmentation) and segment used for augmentation (ileum vs. sigmoid colon).ResultsThere were 41 patients with a mean age of 11.2 years and a mean follow-up of 33.3 months. Of these, 33 CCC were constructed with appendix and eight with a Yang–Monti technique (4 ileal, 4 sigmoid); 31 patients also had an enterocystoplasty (19 sigmoid, 9 ileal and 3 others). Overall revision rate was 27%; revision was required in 8/33 (24%) appendiceal and 3/8 (38%) Yang–Monti CCC (P = 0.7). Revisions were required in 4/21 CCC implanted in the native bladder and 7/20 implanted in augmented bladder (P = 0.3). The majority of revisions were at skin level.ConclusionsAlthough there was no statistical difference in revision rate according to type of CCC, type of stoma or site of implantation, complications appeared to be more common in patients requiring a more complex reconstruction.     

Pneumovesicoscopic diverticulectomy in children and adolescents: Is open surgery still indicated?

ObjectivesSurgical treatment of a congenital bladder diverticulum is indicated in symptomatic children. Diverticulectomy can be performed by an open or a laparoscopic approach. We report our recent experience in using the pneumovesicoscopic approach for accomplishing vesical diverticulectomy.MethodsWe operated on three boys with a mean age of 11.6 years (10–14 years) during August 2006 to February 2007. In all children, a ureteric catheter was introduced first by cystoscopy followed by intravesical CO₂ insufflation at a pressure of 12–15 mmHg. Three trocars were inserted under visual control in the bladder. Diverticulectomy was performed. The defect was closed by interrupted sutures. Bladder drainage was achieved using a urethral catheter for 2 days.ResultsThe mean operative time was 133.3 min (100–180 min). Oral intake began after a mean of 5.3 h (4–6 h). Minimal blood loss was encountered. Non-steroidal analgesics were used only during the 1st day postoperatively with no need for morphia. All patients were discharged on the 2nd day postoperatively after removal of the urethral catheter and tube drain. The mean follow-up period was 5 months (3–6 months).ConclusionPneumovesicoscopic diverticulectomy is a feasible procedure. It does not require a long learning curve, and is associated with shorter hospital stay and rapid recovery with good cosmetic aspect. Pneumovesicoscopy has the potential to be used in the treatment of other conditions such as vesicoureteral reflux, and may replace open surgery.     

Impact of epispadias repair on bladder growth in boys with classic bladder exstrophy

ObjectiveGrowth of the bladder in children with bladder exstrophy is primarily responsible for later ability to void continently. Improvement in bladder capacity has been noted in some boys following epispadias repair. Does the timing of epispadias repair influence the ability of the bladder to grow?MethodsData were collected regarding bladder volume measurements, obtained under anesthesia using a standard technique, during yearly follow-up of boys with classic bladder exstrophy. Volume prior to epispadias repair was compared to the next volume measure following repair. Timing of epispadias repair was compared to changes in bladder capacity in 30 boys. Monthly increases in bladder capacity were calculated in boys repaired at <12 (4), 13–24 (12) and 25–48 (14) months.ResultsPatients who had surgery prior to 12 months of age had the highest rate of monthly increase in bladder capacity (2.40 cc/month). Monthly growth rates were 1.91 cc/month for patients repaired at 13–24 months and 1.18 cc/month for those repaired at 25–48 months.ConclusionsEpispadias repair does lead to early increase in bladder capacity in boys with classic bladder exstrophy. The monthly increases in bladder capacity are greater in boys <12 months. Improvement in bladder volume is less likely when epispadias is repaired after age 29 months.     

Are urodynamic studies really needed during bladder augmentation follow-up?

ObjectiveWe assessed clinical and urodynamic outcomes, over a minimum 10-year follow-up period, of neuropathic bladder patients treated with a bladder augmentation (BA) to determine if periodic urodynamic studies are needed.Material and methodsThirty-two patients with poorly compliant bladders underwent BA at a mean age of 11 years (2.5–18). Mean follow-up was 12 years (10–14.5) and mean patient age at the end of the study was 22 years (12.2–33). During follow-up all patients were controlled at regular intervals with urinary tract imaging, serum electrolyte and creatinine levels, cystoscopy and urodynamic studies. Preoperative, 1-year post-BA and latest urodynamic studies results were compared.ResultsUrodynamic studies at 1-year post-BA showed a significant increase in bladder capacity and a decrease in end-filling detrusor pressure compared with preoperative values (396 vs 106 ml; 10 vs 50 cm H₂O, P < 0.0001). The increase in bladder capacity was more significant at the end of the study than after 1 year (507.8 vs 396 ml, P < 0.002). Thirteen patients had phasic contractions after 1 year and 11 at the end (not significant, NS), and these contractions were more frequent with colon than with ileum (NS). At the end of follow-up, phasic contraction pressure had decreased while trigger volume had increased (35 vs 28 cm H₂O; 247 vs 353 ml, NS). All patients are dry and have normal renal function, except one who had mild renal insufficiency before BA.ConclusionBA improves bladder capacity and pressure, and these changes are maintained over time (although phasic contractions do not disappear). Repeated urodynamic studies are only necessary when upper urinary tract dilatation or incontinence does not improve.     

Laparoscopic inguinal herniotomy in bladder exstrophy: A new solution to an old problem?

ObjectiveLaparoscopy is now the established management for infants with inguinal hernia at our institution. Open inguinal herniotomy in children with bladder exstrophy can be a difficult operation and is associated with a high rate of recurrence (15%). Here, we report our initial experience with laparoscopic repair of inguinal hernia in three children with bladder exstrophy.Materials and methodsThree boys with repaired bladder exstrophy presented with inguinal hernia during follow up. The clinical presentation was bilateral in one and findings at surgery were bilateral open internal rings in two. Under general anaesthesia, pneumoperitoneum of 10 mmHg was raised with a Hasson port at the umbilicus. Two further instruments were inserted in the right and left flanks without ports. Two 4/0 Prolene™ purse string sutures were placed to securely close the internal ring. The umbilicus was closed with an absorbable suture, and the skin incisions were closed with Indermil Glue™.ResultsAll infants made an uneventful recovery, being discharged within 24 h. There were no complications. There has been no sign of recurrence during a mean follow up of 16 months.ConclusionLaparoscopic inguinal herniotomy is a technically straightforward and effective way to treat inguinal hernias in children with bladder exstrophy.     

Comparison of traditional enterocystoplasty and seromuscular colocystoplasty lined with urothelium

ObjectiveTo evaluate long-term outcomes between various methods of augmentation cystoplasty.MethodsA retrospective analysis was performed of patients undergoing seromuscular colocystoplasty lined with urothelium (SCLU, n = 26), and their outcomes compared to a similar population of patients in the same institution who had received traditional forms of bladder augmentation (colocystoplasty and ileocystoplasty, n = 32). Measurements included efficacy of the procedure in increasing bladder capacity and achieving urinary continence, and the need of subsequent surgery for complications.ResultsThere was no statistically significant difference in achieved bladder capacity, subjective urinary continence and the rates of subsequent surgery for stones, vesicoureteral reflux, augment failure, bladder neck continence and catheterizable channel. None of the patients in the SCLU group had spontaneous perforation or small bowel obstruction.ConclusionPatients with SCLU are at decreased risk for bowel obstruction and spontaneous perforation, but are not devoid of other long-term complications including bladder stones, vesicoureteral reflux and augment failure. Most of the risks and benefits of augmentation cystoplasty performed using ileum, colon, or SCLU appear similar.     

Augmentation ureterocystoplasty in boys with valve bladder syndrome

ObjectiveChildren with valve bladder syndrome represent the worst end of the posterior urethral valve spectrum. When conservative measures fail to control recurrent infections, prevent deterioration of the upper tract (in the form of increasing hydronephrosis and or worsening of kidney function) and improve incontinence, augmentation cystoplasty is considered. In most of these boys, renal insufficiency precludes the use of intestine for augmenting the bladder. Our aim was to evaluate the efficacy and safety of ureterocystoplasty in managing children with valve bladder syndrome.Patients and methodsEight boys (mean age 5 years) with valve bladder syndrome were included in this study. All boys had successful valve ablation at the time of presentation. When conservative treatment failed, ureterocystoplasty was scheduled. The entire ureter was folded and used in four boys after nephrectomy for a non-functioning kidney. The lower dilated ureter was used to augment the bladder, and transureteroureterostomy in two and re-implantation of the remaining ureter in two were performed. Radiological and urodynamic investigation was performed preoperatively and postoperatively at 3, 6 and 12 months. Improvement of hydroureteronephrosis was judged by ultrasound.ResultsBladder capacity (as measured during cystometry at 30 cm H₂O) and compliance were significantly improved in all children following the procedure (P < 0.001), and reached or exceeded the normal calculated capacity for age-matched boys. Hydroureteronephrosis improved in six boys (75%). The procedure avoids almost all the complications of enterocystoplasty. Clean intermittent self-catheterization was performed in all cases routinely after surgery, weaning off as judged by the voiding pattern of the child.ConclusionUreterocystoplasty is an ideal option for augmenting the hypocompliant bladder in boys with valve bladder syndrome. The entire ureter or the dilated lower part can be used. This is a solution for boys with impaired renal function when enterocystoplasty cannot be performed.     

Severe bladder dysfunction revealed prenatally or during infancy

ObjectiveAlthough thought to be an acquired condition, non-neurogenic neurogenic bladder may sometimes be a congenital dysfunction, revealed before toilet training. We report our experience with the condition diagnosed prenatally or during early infancy.Patients and methodWe retrospectively reviewed cases of severe bladder dysfunction with upper-tract impairment, without neurological or obstructive pathology, in children diagnosed before toilet training: five with prenatal diagnosis of severe hydro-ureteronephrosis (group 1) and six with signs of bladder dysfunction during infancy (group 2).ResultsFollow up of group 1 showed decompensation toward severe bladder dysfunction, diagnosed after either toilet training or ureteral reimplantation (n = 3). After a median follow up of 14 years (0.5–20), four were on clean intermittent catheterization with bladder augmentation and one required sphincteric re-education with good result. Two of the five had chronic renal failure. In group 2, six children (two females) presented at median age of 20 months (2–30) with indirect signs of bladder dysfunction, including vesicoureteral reflux (n = 4) and/or hydro-ureteronephrosis (n = 4). After a median follow up of 11 years (5–20), three were on clean intermittent catheterization (two Mitrofanoff channels), and three underwent bladder augmentation. Three children had chronic renal failure of whom one underwent renal transplant.ConclusionThese cases of severe bladder dysfunction were initially misdiagnosed. In both groups, follow up revealed severe dilatation of the upper tract and secondary renal impairment. Antenatal diagnosis of bilateral pyeloureteral dilatation may be the first sign of early bladder dysfunction.     

Risk factors for progression to end-stage renal disease in children with posterior urethral valves

ObjectiveTo identify the variables which affect long-term renal outcome in children with posterior urethral valves (PUV).Materials and methodsRetrospective analysis of 260 children with PUV who underwent ablation of valves in 1992–2008 at our tertiary care center. The following risk factors for progression to end-stage renal disease (ESRD) were analyzed: nadir serum creatinine greater than 1.0 mg/dl, bilateral grade 3 or higher VUR at diagnosis, recurrent febrile UTIs, and severe bladder dysfunction. Patients were divided into two groups: those who developed ESRD (group 1) and those who did not (group 2).ResultsForty (17.62%) patients had nadir serum creatinine >1 mg/dl. At time of initial presentation, high-grade VUR was seen in 63.1% and 33.5% of groups 1 and 2, respectively (P = 0.002). Overall, 77 (34%) of the boys developed breakthrough urinary tract infections: 37.03% and 33.5% in groups 1 and 2, respectively (P = 1). Fifty-nine (26%) patients were found to have severe bladder dysfunction: 77.8% and 19% in groups 1 and 2, respectively (P < 0.0001). Twenty-seven (11.89%) patients progressed to ESRD, at mean age of 11.21 years (5–16). On univariate analysis, the risk-predicting variables were: nadir serum creatinine value greater than 1 mg/dl (P < 0.0001), bilateral high-grade VUR (P = 0.002) and severe bladder dysfunction (P < 0.0001). On multivariate logistic regression analysis, nadir serum creatinine greater than 1 mg/dl (OR 23.79; CI 8.20–69.05) and severe bladder dysfunction (OR 5.67; CI 1.90–16.93) were found to be independent risk factors predictive of ultimate progression to ESRD.ConclusionsNadir serum creatinine and bladder dysfunction are the main factors affecting long-term renal outcome in cases of PUV. Early identification and treatment of bladder dysfunction may thus be beneficial.