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.     

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.     

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.     

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.     

Indiana pouch in children: A 15-year experience

ObjectiveWe report our experience with the Indiana pouch (continent urinary reservoir) in 12 consecutive children over the last 15 years and report their follow-up.Material and methodsTwelve consecutive children, who underwent the continent urinary reservoir procedure in the form of an Indiana pouch, were prospectively enrolled in the study. All consecutive children who were referred to our service with multiple failed surgeries for exstrophy–epispadias repair, cloacal exstrophy, genitourinary rhabdomyosarcoma with residual disease in the trigonal area not amenable to partial cystectomy, and neuropathic bladder with nephrogenic metaplasia were included over the period 1997–2012. All these children were offered the same form of bladder substitution (Indiana pouch) as described by the Indiana group many years ago. Postoperative care was on a fixed protocol, and follow-up details recorded over the years. They were followed up for dry interval with clean intermittent catheterisation, social acceptance, and early and late complications.ResultsOut of these 12 patients (7 males and 5 females), eight patients had exstrophy–epispadias with multiple failed operations carried out elsewhere, cloacal exstrophy (2), residual rhabdomyosarcoma in the trigonal area with incontinence following chemotherapy (1), and neuropathic bladder with recurrent diffuse neoplastic polyposis (1). In the follow-up period of 1–15 years (median 24 months) all the patients had a dry interval of 4 h or more with clean intermittent catheterisation. One patient had wound dehiscence during the postoperative period and another required stomal revision 1 year after initial surgery.ConclusionsThe Indiana pouch is a reliable, safe, and effective form of bladder substitution. It can be reconstructed in a wide range of lower urinary tract disorders. In the vast majority of children with multiple failed surgical procedures for exstrophy–epispadias, the Indiana pouch is a safe, reliable, and reproducible procedure to provide a socially acceptable quality of life with a dry interval of 4 h.     

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.     

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.     

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.     

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.     

Long-term follow up of enteric bladder augmentations: the risk for malignancy

ObjectiveTo determine the risk of bladder cancer following enteric bladder augmentation.Materials and methodsPatients followed for care after an enteric bladder augmentation have been entered into a registry; individuals followed for a minimum of 10 years were evaluated.ResultsThe study criteria were met by 153 patients. Indications for bladder augmentation were neurogenic bladder in 97, exstrophy in 38 and posterior urethral valves in 18. There was a median follow-up interval of 27 years (range 10–53). A total of seven cases of malignancy developed. Median time to tumor development following augmentation was 32 years (range 22–52). Two patients with neurogenic bladder developed transitional cell carcinoma; both were heavy smokers (>50 pack per year history). Two patients with a history of posterior urethral valves and renal transplantation developed adenocarcinoma of the enteric augment. Three patients with bladder exstrophy developed multifocal adenocarcinoma of the augmented bladder. Two patients remain alive, 5 and 6 years following radical cystoprostatectomy; five died of cancer-specific causes.ConclusionsMalignancy following enteric bladder augmentation arose in 4.5% (7/153) of our patients and was associated with coexisting carcinogenic stimuli (prolonged tobacco/chronic immunosuppressive exposure), or alternatively with the inherent risk of malignancy existing with bladder exstrophy.     

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.     

Total endoscopic management (TEM approach) of children with non-compliant neuropathic bladder: a preliminary report

PurposeWe prospectively evaluated the efficacy and durability of a combination of intradetrusor botulinum-A toxin (BTX-A) and endoscopic treatment of vesicoureteric reflux (VUR) to manage children with myelomeningocele (MMC) and non-compliant refluxing bladders who were not responding to standard conservative therapy. We also evaluated whether this combined therapy can lower intravesical pressure, increase bladder capacity, gain social continence and protect the upper tract from recurrent urinary tract infection.Material and methodsA total of 10 patients with a mean age of 5.9 ± 3.6 years (range 2–12 years) with MMC (eight females and two males) were prospectively involved in the study. All patients were fully compliant to clean intermittent catheterization, and all were non-responders (failed to gain continence and/or poor compliance) to the maximum tolerable dose of anticholinergics and catheterization. All patients were subjected to cystoscopic intradetrusor injection of 12 U/kg (maximum 300 U) of BTX-A in an infection-free bladder. They all had VUR (16 refluxing ureters, six patients with bilateral VUR) and did not show resolution in the pretreatment voiding cystourethrogram; accordingly, submucosal injection of Deflux^® was performed either with the second BTX-A treatment (initial four patients) or with the first BTX-A treatment (the other six patients). The grade of reflux was G III, IV and V in three, seven and six ureters, respectively.ResultsThe maximum bladder capacity increased significantly from 79 ± 49 to 155 ± 57 ml (p < 0.022), and the maximum detrusor pressure decreased significantly from 55 ± 16 to 37 ± 11 cm H₂O (p < 0.001). Fifteen out of 16 (93.75%) refluxing ureters were completely resolved (one of them on second attempt), and one (6.25%) (GV reflux) remained unchanged despite of two attempts. Of six incontinent patients, five reached complete dryness between catheterizations and one showed partial improvement.ConclusionsA combination of BTX-A and endoscopic correction of VUR is a simple and effective way to overcome the increased risk of high intravesical pressure and recurrent UTI. This treatment decreases the incidence of renal damage in children on whom conservative management fails to help, in a minimally invasive way.     

Penile ischemic injury in the exstrophy/epispadias spectrum: New insights and possible mechanisms

ObjectivePartial or complete penile loss following bladder exstrophy and/or epispadias repair has been reported in the literature progressively more frequently.Patients and methodsThe authors report new cases of penile injury following bladder exstrophy and/or epispadias repair referred to their centers and not previously published. They review the literature on this subject and offer an explanation as to the likely mechanism for the penile injury and recommendations to avoid this complication.ResultsSeven new cases of partial or complete penile loss following bladder exstrophy or epispadias repair have been recently referred to the authors’ institutions. Twenty-one patients have previously been reported in the literature. Altogether, 24 cases occurred after bladder exstrophy closure: 23 after complete primary repair of exstrophy (Mitchell repair) and one after first-stage radical soft-tissue mobilization (Kelly repair). Nineteen of 24 patients did not have a pelvic osteotomy at the time of primary closure. Four cases occurred after epispadias repair: two following the second-stage radical soft-tissue mobilization (Kelly repair) and two following penile disassembly epispadias repair (Mitchell repair).ConclusionExstrophy closure combined with epispadias repair can be followed by ischemic penile injury, particularly when osteotomy is not performed. Compression of the pudendal vessels after pubic apposition and/or direct injury to the pudendal vessels play an important role in the pathogenesis of this complication.     

游离尿道板卷管尿道成形治疗尿道上裂

目的 介绍和评价游离尿道板卷管尿道成形治疗男性尿道上裂.方法 2003年1月～2006年12月用游离尿道板卷管尿道成形治疗男性尿道上裂12例,年龄为1～20岁,其中单纯阴茎体尿道上裂10例,另2例为膀胱外翻曾在本院行骨盆截骨膀胱内翻关闭后.用Ransley法纠正阴茎背曲3例.结果 平均随访20个月(3～40个月),12例患儿阴茎头呈圆锥状,阴茎外观满意.1例患儿仍存在轻度阴茎背曲,1例患儿因术后尿瘘被再次手术修补.术后所有患儿阴茎勃起功能存在.结论 游离尿道板卷管尿道成形治疗尿道上裂方法简单,可达到恢复阴茎尿道正常的位置结构,使阴茎外观和功能接近正常.     

Comparison of outcomes of tubularized incised plate hypospadias repair and circumcision: a questionnaire-based survey of parents and surgeon

AimWe administered a standardized questionnaire to parents and operating surgeon to determine their opinions regarding outcomes from tubularized incised plate (TIP) hypospadias repair.MethodsLikert scale questionnaires were used to assess six outcomes: overall penile appearance, cosmesis of meatus and skin, penile size, straightness during erection, and voided stream. These were answered by consecutive parents of boys 6 weeks following TIP hypospadias repair, as well as by the surgeon. Controls were normal boys 6 weeks after elective circumcision, with their parents and the surgeon answering the same questionnaires.ResultsThere were no significant differences in responses between parents of boys undergoing TIP repair for distal (n = 50) to proximal (n = 15) hypospadias compared to parents of normal boys undergoing elective circumcision (n = 22). Surgeon impressions of cosmetic results were slightly, but significantly, more favorable than those of parents. Surgeon scores were higher for distal versus proximal hypospadias outcomes.ConclusionsOur study provides objective evidence that parents consider TIP hypospadias repair to create a normally functioning and appearing penis. Parent and surgeon opinions of cosmetic outcomes were similar, although surgeon impressions were more favorable.     

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.     

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.     

A prospective randomized trial of the effect of a soluble adhesive on the ease of dressing removal following hypospadias repair

AimTo determine whether the use of a novel dressing removal technique resulted in shorter removal times, reduced the child's experience of pain and/or reduced parental anxiety when compared to a standard approach.MethodsThis prospective unblinded randomized controlled trial of 53 consecutive boys undergoing primary hypospadias repair was powered using data from a prior feasibility study. Children were randomized to a standard control dressing (A) or standard dressing plus Cavilon™ (B) group. Removal was by (A) soaking the child and dressing in the bath or (B) application of an adhesive remover to the dressing. The primary outcome measure was dressing removal time. Secondary measures were: child pain scores on a visual analogue scale measured by the parent and nurse, and parental anxiety using the self-evaluative state anxiety measure. Data are expressed as median (range); P < 0.05 is significant.ResultsDressing B was significantly quicker to remove than A: B 30 min (5–86 min) vs A 40 min (17–105 min), P = 0.01, Mann–Whitney. No differences in parent/nurse pain scores between the two groups were seen. There was a strong correlation between parent and nurse reporting of pain scores at the time of dressing removal (Spearman 0.79, P < 0.000). No significant differences in pre- and post-dressing removal parental anxiety scores were seen (P = 0.159, Mann–Whitney). Several parents had high anxiety levels at both times.ConclusionThe novel dressing removal approach (B) was significantly shorter than the standard one (A). There were no significant differences in child's pain or parental anxiety score between the two approaches.     

Differing urinary urea excretion among children with idiopathic hypercalciuria and/or hyperuricosuria

ObjectiveTo estimate dietary protein intake in children with idiopathic hypercalciuria (HC) and/or hyperuricosuria (HU).Patients and methodsWe compared the 24-h urinary excretion of urea, as a reflection of protein intake, in four age- and sex-matched groups, each comprising 56 consecutive children: (1) HC, (2) HU, (3) HC + HU and (4) control.ResultsUrinary urea excretion was significantly higher in HC, HU and HC + HU than in controls. HC and HU children had similar urea excretion. HC + HU children had urinary urea significantly higher than HC and HU, but urinary calcium similar to HC and urinary uric acid excretion similar to HU subjects. Urinary calcium was significantly (R² = 0.21) correlated with urea excretion in HC children only, whereas urinary uric acid was significantly (R² = 0.21) correlated with urinary urea in HU children only. No significant correlation between urinary urea and calcium or uric acid excretion was found in HC + HU patients although they had the highest urinary urea. A significant (p = 0.004) interaction between urinary urea and sodium in increasing urinary calcium excretion resulted only in the HC group.ConclusionThe association of dietary protein excess with HC and/or HU is conditioned by an individual (genetic?) predisposition and may be produced by different mechanisms.     

Management of bladder exstrophy in adulthood: Report of 5 cases

ObjectiveTo report our experience in the management of adult classic bladder exstrophy.Patients and methodsDuring 1977‒2006 we treated five adult males presenting with classic exstrophy–epispadias complex. Patient age at presentation ranged from 17 to 30 with a mean age of 23 years. Four patients had received no previous treatment and one underwent previous ureterosigmoidostomy. Work-up included evaluation of upper tract and bladder biopsy. Bladder patch condition was variable. Surgery involved bladder preservation in the three patients who underwent primary repair, including bladder closure, bladder neck reconstruction and epispadias repair; two of them also had augmentation ileocystoplasty. The remaining two patients underwent ureterosigmoidostomy, cystectomy and epispadias repair. Abdominal wall closure was by fasciocutaneous M-plasty. Osteotomy was not done in any case.ResultsIn patients with bladder preservation, one patient was continent (>3 h) and voided normally whereas the other two showed day and night continence (2–3 h) with mild stress incontinence. Patients were satisfied with functional outcome. Ultrasound and intravenous pyelography showed preservation of upper tract. Follow-up period ranged from 1 to 8 years.ConclusionPatients with bladder exstrophy presenting in adulthood should not be denied the opportunity of primary reconstruction with bladder preservation in the absence of significant histological changes in the bladder mucosa.