Safety and feasibility of laparoscopic appendicovesicostomy in children

IntroductionMitrofanoff appendicovesicostomy is needed for securing a conduit for clean intermittent catheterization in children with myelomeningocele, posterior urethral valves and non-neuropathic neuropathic bladder. An open technique is widely used; herein we report our initial experience with minimally invasive laparoscopic appendicovesicostomy in children.Patients and methodsDuring 2007–2011 we operated on 4 male children with a mean age of 6 years (3–9) suffering from posterior urethral valves (1), myelomeningocele (2), and non-neuropathic neuropathic bladder (1). A posterior Mitrofanoff trough was used in one child while in the remaining children we used the anterior Mitrofanoff trough.ResultsThe mean operative time was 3.5 h (3–5). The mean hospital stay was 3.7 days (2–5). The mean follow up was 12.5 months (5–30). All are continent; one child was converted to open because of failure to pass the catheter at the end of the procedure. Cosmetic aspect is perfect. No difficulty in catheterization was encountered.ConclusionLaparoscopic Mitrofanoff is a feasible, safe and effective technique associated with low morbidity.     

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.     

The use of refluxing ureter in the creation of a Mitrofanoff channel in children undergoing bladder augmentation: is a formal reimplantation necessary?

To study the fate of a refluxing ureteral stump used as a Mitrofanoff channel (MC) for clean intermittent catheterization (CIC), without reimplantation in augmented bladders. Ten boys and seven girls, 1–14 years old (mean 6.5 years) seen in period 1998–2005, underwent creation of MC using refluxing or potentially refluxing ureters. Concomitant bladder augmentation (BA) was performed for various abnormalities of lower urinary tract, including exstrophy epispadias complex, neurogenic bladder cloaca and posterior urethral valve. The uretero-vesical junction was maintained intact during creation of the MC. Follow-up period ranged from 2–72 months (mean 32.5 months). Satisfactory CIC was possible in 14 children while in two children the stoma became obstructed due to non-usage. Urinary leak via the ureteric MC was seen in only one patient who partially responded to anticholinergic therapy and a shortened CIC interval but this needed to be formally closed subsequently. A refluxing ureter can be used in the creation of a MC without formal reimplantation in the majority of children undergoing BA.     

The search for continence in bladder exstrophy: Bladder neck transection and Macedo catheterizable reservoir to augment the nativebladder

IntroductionBladder exstrophy remains one of the most challenging abnormalities in pediatric urology. We propose bladder neck transection and bladder augmentation with a catheterizeable reservoir technique to achieve continence after previous anatomic reconstruction in stages.MethodsAt the age of 5–6 years, we offer the transection of bladder neck and enterocystoplasty to achieve continence. We report on a 6-year-old boy that underwent this procedure. We perform the reservoir from ileum according to Macedo-technique that constructs a catheterizeable channel from the same bowel segment. The continence mechanism of the efferent tube is based on angulation and a serous lined tunnel created with 3.0 prolene sutures. The stoma is placed in the midline.ResultsPatient had an uneventful evolution and is continent performing CIC every 4 h with 9 months of follow up.DiscussionIn spite of continuous development of bladder exstrophy surgery, the urethral continence and voluntary micturition is still not possible in the majority of patients. We discuss with our patients honestly and offer this method as a viable alternative to achieve continence.ConclusionIn our experience, most patients accept urethral transection and suprapubic CIC when educated about results with other alternatives of bladder neck plasty.     

Vesicostomy in childhood: indications and results

The authors report their experience with 27 patients who underwent cutaneous vesicostomy (CV) diversion for either lower tract disorders or obstruction. The indications for CV included 18 cases of neurogenic bladder, 3 pseudo-prune belly syndrome, 3 bladder exstrophy, 2 vesicoureteral reflux, and 1 posterior urethral valves. A Lapides-type technique with minor changes was performed in all cases. Improvement of renal function occurred after CV in all but 3 patients (11.1%). No early surgical complications were observed. Late complications occurred in 8 patients (29.6%): 4 (14.8%) had a stomal stenosis and 4 (14.8%) urinary tract infections. The overall revision rate was 7.4%. We believe this technique is simple to perform, tubeless, easy to manage, and readly reversible. Our experience tends to confirm CV to be an easy, effective, and reversible means to treat children and infants with selected pathology of the lower urinary tract. Results and complications are discussed.     

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.     

Outcomes of renal transplantation in children with and without urinary tract abnormalities: is there any difference?

PurposeTo evaluate if children with urinary tract abnormalities, particularly bladder disorders, have different long-term outcome after renal transplantation, compared to those with non-urological cause of ESRD.Material and Methods211 children (age <19 years) were submitted to 226 renal transplantations between 1989 and 2005. They were separated in three groups: 136 children with ESRD due to non-urological cause (GROUP 1), 56 children with urological disorders but with adequate bladders (GROUP 2) and 19 children with lower urinary tract dysfunction associated or not to inadequate bladder emptying (GROUP 3). In group 3, 15 children were submitted previously to a bladder augmentation (ureterocystoplasty in 6 and enterocystoplasty in 9), one to a bladder autoaugmentation, two children to a continent urinary diversion, and one to an appendical Mitrofanoff procedure. The renal transplantation was performed in a classical, extraperitoneal access, and the ureter implanted, whenever possible, with an antireflux technique.ResultsAfter a mean follow-up of 75 months, 13 children died, 59 grafts were lost, and 15 children received a second transplant. Two patients of GROUP 3 required a complementary urological procedure to preserve renal function (one enterocystoplasty and one vesicostomy). There was a total of 12 (5.3%) major surgical complications, with an equal incidence in the three groups. The overall graft survivals at 5 years were 75% (GROUP 1), 74% (GROUP 2) and 84% (GROUP 3).ConclusionsWith an individualized treatment, children with urological abnormalities, including those with lower urinary tract disorders, have the same long-term outcome as those with non-urological cause of ESRD.     

Simultaneous pediatric kidney transplantation and ureterocystoplasty in a 20-month-old boy

Kidney transplantation (KT) in children with end-stage renal disease and an abnormal bladder poses a complex management challenge. Ureterocystoplasty (UC) has been previously reported in older children with non-compliant bladders, but the timing and technique of repair are controversial. This case reports the youngest patient, a 20-month-old boy to undergo successful single-stage UC and living-related KT. UC was performed because of a fibrotic, non-compliant bladder. A temporary vesicostomy was placed to provide adequate drainage in the presence of urethral stenosis. The patient developed a single episode of pyelonephritis within the first six months post-operatively, but there were no other urologic complications. At 13 months, the renal function is excellent with a mean glomerular filtration rate of 100 mL/min/1.73 m(2) and no clinical evidence of rejection. This case demonstrates that simultaneous UC and KT can be safely performed even in infants with non-compliant bladders and renal failure.     

Role of urethral bulking agents in epispadias–exstrophy complex patients

ObjectiveInjection of urethral bulking agents (UBA) has been used to increase bladder capacity prior to bladder neck reconstruction (BNR) or as an adjuvant therapy following BNR to improve continence. The purpose of this study was to determine the effectiveness of urethral injections in the exstrophy population.Materials and methodsA review was performed of patient characteristics, bladder capacity, and continence status of all patients with the exstrophy–epispadias complex who underwent injection of UBA between 1980 and 2008.ResultsAmong 66 patients with a median follow-up of 8 years, 41 underwent injections prior to BNR, and 25 had injections after BNR. Only 24% of patients who underwent injections prior to BNR were continent on last follow-up. Among 25 patients who underwent BNR prior to injection(s), 16 were partially continent and nine were incontinent prior to first injection. Patients who were partially continent attained social continence (dry interval greater than 3 h) at a significantly higher rate than those who were incontinent (63% vs. 13%, p = 0.047). No patient with cloacal exstrophy in either group attained urethral continence.ConclusionUBAs do not appear to have a role prior to BNR. However, they may provide benefit when given adjunctively following BNR in patients who are partially continent.     

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.     

Renal transplantation and congenital anomaly of the kidney and urinary tract

Paediatric renal transplantation in children differs from adult series because of the high incidence of abnormalities of the lower urinary tract. We report our experience concerning five children with end-stage renal disease and associated bladder dysfunction due to posterior urethral valves, and lower urinary tract abnormalities (‘valve bladder’), who underwent renal transplantation without prior correction of the uropathy. Of 151 paediatric patients (aged 34 months to 23 years) who underwent renal transplantation, 58 had lower urinary tract abnormalities, and 42 underwent surgical correction of the uropathy prior to renal transplantation. In 15 patients, the uropathy was fibrotic bladder secondary to posterior urethral valves (‘valve bladder’). After clinical and urodynamic evaluation, all 15 patients were considered as candidates for bladder augmentation. In 10 patients, the bladder augmentation was performed prior to renal transplantation. The remaining five patients presented with oligoanuria at the time of the evaluation, and the decision to consider bladder augmentation was postponed until the post-transplant period. At the time of renal transplantation, 2 of the 5 patients underwent ureterostomy, and three had a ureteral reimplant associated with a suprapubic catheter for 2 months. Periodic assessment of renal function and bladder capacity/compliance was performed, as was renal ultrasound. After 4 months, the five patients were re-evaluated for the need for bladder augmentation. At 1, 2, 3 and 4 months follow-up, the five patients had normal renal function with improvement of bladder capacity and absence of hydronephrosis. In 3 of the 5 children, bladder augmentation was judged to be no longer necessary due to the complete restoration of clinical and urodynamic parameters. Therefore, renal transplantation can be safely performed without pre-emptive reconstruction of the lower urinary tract. When possible, ureteral reimplantation is recommended, even in a very small valve bladder, since the initial indication for bladder augmentation may be modified once normal diuresis has been restored.     

Long-term renal and neurodevelopmental outcome in infants with LUTO, with and without fetal intervention

Congenital lower urinary tract obstruction (LUTO) is a heterogeneous group of pathologies, the most common being posterior urethral valves (PUV) or urethral atresia. The bladder neck obstruction in utero leads to a spectrum of disease including mild oligohydramnios with normal renal function to a picture of severe oligohydramnios associated with chronic obstructive macro/microcystic renal parenchymal disease leading to chronic renal impairment. These anomalies may be isolated or complex; the latter being associated with other structural or chromosomal abnormalities. If isolated, the congenital bladder neck obstruction may be amenable to in-utero therapy. In a significant proportion of babies affected by LUTO there is severe oligohydramnios (occurring before 20 weeks gestation) and associated with pulmonary hypoplasia, a scenario almost always associated with perinatal death. For those babies that survive the perinatal period there is a significant risk of renal impairment, often necessitating renal dialysis or transplantation in childhood. In addition, there may be other morbidities such as chronic filling anomalies of the bladder that may require treatment.     

Urologic complications of major genitourinary reconstruction in the exstrophy–epispadias complex

ObjectiveTo present the authors' experiences with urologic complications associated with various techniques used to create a continent stoma (CS), augmentation cystoplasty (AC), and neobladder in the exstrophy–epispadias complex (EEC) population.MethodsRetrospective review of medical records of patients who underwent CS with or without bladder augmentation were identified from an institutional review board-approved database of 1208 EEC patients. Surgical indications, tissue type, length of hospital stay, age, preoperative bladder capacity, prior genitourinary surgeries, postoperative urological complications, and continence status were reviewed.ResultsAmong the EEC patients reviewed, 133 underwent CS (80 male, 53 female). Mean follow-up time after initial continent stoma was 5.31 years (range: 6 months to 20 years). Appendix and tapered ileum were the primary bowel segments used for the continent channel and stoma in the EEC population. The most common stomal complications in this population were stenosis, incontinence, and prolapse. Seventy-nine percent of EEC CS patients underwent AC primarily done with sigmoid colon or ileum. Eleven patients (8%) underwent neobladder creation with either colon or a combination of colon and ileum. Bladder calculi, vesicocutaneous fistula, and pyelonephritis were the most common non-stomal complications. Stomal ischemia was significantly increased in Monti ileovesicostomy compared to Mitrofanoff appendicovesicostomy in classic bladder exstrophy patients (p = 0.036). Furthermore, pyelonephritis was more than twice as likely in colonic neobladder than all other reservoir tissue types in the same cohort (OR = 2.53, 95% CI: 1.762–3.301, p < 0.001).ConclusionsTo the best of the authors' knowledge, this is the largest study examining catheterizable stomas in the exstrophy population. While Mitrofanoff appendicovesicostomy is preferred to Monti ileovesicostomy because it is technically less challenging, it may also confer a lower rate of stomal ischemia. Furthermore, even though ileum or colon can be used in AC with equally low complication rates, practitioners must be wary of potential urologic complications that should be primarily managed by an experienced reconstructive surgeon.     

Duplicate exstrophy

A 4-year-old boy presented with a mucous membrane in the suprapubic region, a low-set umbilicus, and the typical musculoskeletal deformity seen in classic bladder exstrophy. The penis had a thin, rudimentary urethral plate on its dorsal surface. The main urethra was orthotopic and normal in size. Micturition was undisturbed and the child was continent of urine. Excision of the lesion and reconstruction of the suprapubic area with bilateral rhomboid skin flaps was curative. Pathologic examination of the removed specimen revealed transitional-cell epithelium and a tubular structure with an epithelial-cell lining resembling a deferential duct.     

The variability of urographic findings in posterior urethral valves

The full variety of secondary and associated upper tract abnormalities concurrent with obstructive valves seems not to be generally appreciated. While upper urinary dilatation and impaired renal function are features regarded as almost obligatory, there are cases at the other end of the spectrum with surprisingly slight or no abnormalities of the bladder, ureters or collecting systems. Therefore a negative IVP does not exclude posterior urethral valves in any boy with symptoms pertaining to the urinary tract. A MCU should invariably be included in the radiological work-up.     

An alternative channel for the Mitrofanoff principle based on transverse skin flaps: an extraperitoneal minimal invasive approach (the RPM technique)

ObjectiveThe Mitrofanoff principle is a well established strategy in pediatric urology, with the appendix and Yang-Monti tube being the most used channels. The search for an alternative tube with less morbidity is justified. Hence, we present a patient treated via an alternative approach in which the channel was constructed from two lower abdominal transverse skin flaps (the RPM technique).MethodsA 17-year-old patient with posterior urethral valves, hypocontractile bladder and experiencing pain on urethral clean intermittent catheterization was selected. The procedure consisted of defining two rectangular transverse skin flaps of 5 × 1 cm opposite to each other. The flaps were rotated 90° and anastomosed to create a tube. A small extraperitoneal bladder wall incision was performed and the tube was connected to the bladder. Two rectal abdomen muscle strips were crossed in the midline as a neosphincter.ResultsThe patient had an uneventful postoperative course and remains continent for intervals of 4 h. The stoma and incision have a good cosmetic aspect at 16 months follow-up.ConclusionThe RPM technique is an alternative approach for a minimal invasive strategy according to the Mitrofanoff principle. Long-term follow-up is necessary to confirm the excellent initial results.     

Pre- and postoperative urographic findings in posterior urethral valves

In a series of 65 male infants and children, all with the diagnosis of posterior urethral valves, pre- and postoperative urographic findings were reviewed. In addition changes occurring in the bladder, and the implication of vesico-ureteral reflux were assessed.--Preoperatively diagnosed impairment of kidney function and concommitant dilatation of the upper urinary tract, with some exceptions. Remained fairly unchanged at postoperative examinations In the case of marked vesico-ureteral reflux, permanent kidney function annihilation was significantly commoner than with slight or no reflux.--Although, as a rule, both the upper urinary tract and the bladder were affected, there were cases of posterior urethral valves with a normal appearing bladder. As the intravenous urography do not exclude the urethral abnormality, voiding cysto-urethrography has to be included in the primary radiological exploration of all cases with urological problems. Key words: Intravenous urography, non-functioning kidney, upper urinary tract dilatation, voiding cysto-urethrography, posterior urethral valve, bladder outflow obstruction.     

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.     

Vesico-cutaneous fistula: A simple method for continent urinary diversion

IntroductionPatients with lower urinary tract anomalies or neurogenic disorders often suffer from voiding difficulties. Clean intermittent catheterization (CIC) is effective for bladder drainage; however, this is often painful. Transurethral catheterization is also impossible in patients with urethral stricture. A Mitrofanoff conduit may solve some of these problems, but a few disadvantages have been reported, including: difficult surgical techniques and frequent operative complications. A vesicostomy is easy to perform but persistent urine leak over the abdomen and diaper rash can be annoying. A better way to achieve continent urinary diversion is indicated.MethodBetween December 01 1998 and December 31 2013, six patients underwent a vesico-cutaneous fistula for CIC. The etiologies included urethral stricture (n = 2) and neurogenic bladder (n = 4). The fistula was created at the bladder dome with only the muscle layer of the bladder sutured to the skin. A Foley catheter was left in place for at least two weeks to prevent stoma stricture. After removing the Foley catheter, regular CIC from the fistula was performed every 2 h during the daytime with a Fr. 10–12 feeding tube, depending on the patient's age. Further stenting during the night in the first six months was necessary to prevent early closure of the fistula. Patients were followed with periodic renal ultrasonography, blood tests and urinalysis in the outpatient department.ResultsFollow-up ranged from 6 months to 16 years. All patients showed improvements in hydronephrosis. Decreased UTI frequency was seen in five patients. Renal function was normal in five patients, whilst the other suffered from chronic renal failure preoperatively. Only one patient had occasional mild urine leakage from the stoma at night, which was once in two weeks. No patient experienced painful or difficult catheterization and CIC becomes easy, even by young children.ConclusionsThe vesico-cutaneous fistula is a simple, effective and tolerable method for CIC. It may be a substitute for or a transition to a Mitrofanoff conduit in some patients.     

Complications of surgical reconstruction of the exstrophy–epispadias complex

This review of the complications of all methods of modern treatment of bladder exstrophy emphasizes the complexity of reconstruction of the bladder exstrophy spectrum. The main complications of any method of primary bladder exstrophy closure are complete wound dehiscence, bladder prolapse and urethral outlet obstruction; others include bladder and renal calculi. These complications as well as methods to avoid them are discussed here. Evidence supporting the management strategy is presented where possible.