Management Of Classic Bladder Exstrophy Research Articles

Commentary to ‘One-stage combined delayed bladder closure with Kelly radical soft-tissue mobilization in bladder exstrophy: preliminary results’

The authors present data from a retrospective review of 27 bladder exstrophy patients operated in a modified technique of the radical soft tissue mobilization (RSTM). According to their concept, they combined delayed bladder closure and RSTM as a single-stage procedure. Focussing on the safety of this modified concept, the main outcome criterion was the rate of bladder dehiscence. Secondary outcome criteria were UTI, urethral stenosis urethrocutaneous fistula and parietal hernia. Important technical details are keenly described and complemented by coherent drawings and video clips. After a follow-up of 12 months, there was no case of bladder dehiscence, but a number of complications occurred such as urethral stenosis and urethrocutaneous fistula. The authors concluded that early single-stage RSTM combined with bladder closure is a feasible and attractive option in the management of classical bladder exstrophy.

Perioperative management of classic bladder exstrophy

The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall muscles, and pelvic structures. Modern surgical repairs focus on reconstruction of the bladder and its adjacent structures, with the goal of achieving urinary continence, a satisfactory cosmetic result, and a high quality of life. Complex surgery in neonates and young children, as well as a prolonged postoperative course require close collaboration between surgeons, anesthesiologists, intensivists, pediatricians, and an experienced nursing staff. This article will review the spectrum of bladder exstrophy anomalies, the surgical repair, and the perioperative interdisciplinary management.

Outcomes in bladder exstrophy: 30 year's experience in a single-centre

Purpose The management of exstrophy-epispadias has changed markedly over the past thirty years, and long-term outcomes for different approaches of closure are not known. We have evaluated the outcomes and complications seen in all patients with exstrophy-epispadias complex managed at our institution over the past thirty years. Material and methods A retrospective notes review of all patients treated for exstrophy-epispadias complex was carried out. Basic patient demographics were recorded, as were details of all operations, complications, continence and renal function. Continence rates for patients with classic bladder exstrophy were calculated. Patients were divided into three groups on the basis of their date of birth (1975-1985, 1985-1995 and 1995-2005). Results A total of 62 patients with exstrophy-epispadias complex were treated at RCH from 1975-2005. The majority of these patients were treated from 1985-2005 (53), using the Kelly soft tissue mobilisation principle. The majority of patients had classic bladder exstrophy (42). Patients typically undergo a large number of operations in the management of classic bladder exstrophy and for complications of surgery. Complications remain commonplace, but a small number of patients had no complications. Most patients have normal renal function, but with attempts to achieve continence, more patients have some degree of renal impairment (25% of patients born 1995-2005). Continence without catheterisation is uncommon in patients with classic bladder exstrophy aged under 10 (25%), but continence improves dramatically in patients aged between 10 and 20 with 53% of patients in this age range being continent without catheterisation and a further 25% continent with catheterisation. Conclusions Bladder exstrophy remains a challenging condition to treat effectively. Complications are common, and continence is uncommon in the first decade of life. Secondary operations to improve bladder capacity and reconstruct the bladder neck have allowed the majority of patients to achieve continence in adolescence and early adult life.

Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder

The surgical management of classic bladder exstrophy has evolved over time. Different techniques are used to address the challenge of reconstructing these patients. We review the long-term outcomes of bladder exstrophy treatment from the published literature with regard to urinary continence, voiding and secondary complications. Continence now can be achieved in up to 80% of children in specialist centres. Whether such success can sustained into adult life is uncertain. About 40% of adults are dry in the best hands. Up to 84% of children can void, but there is some evidence that this function is lost with time in 70%. The need for bladder augmentation is widely variable between series, reported in 0-70% of children. This reduces the ability to void spontaneously to about 50% of children. It brings with it the later risk of metabolic disturbance and stone formation. Adults with exstrophy have a 694-fold increase in the risk of bladder cancer by the age of 40 years.

Combined bladder neck reconstruction and epispadias repair for exstrophy-epispadias complex.

The achievement of satisfactory continence in the management of classic bladder exstrophy remains a surgical challenge. During the last 20 years a staged approach to the management of the exstrophy-epispadias complex has been used at many exstrophy centers to attain this goal. In select cases repairs can be combined to reduce the number of mandatory operations to achieve continence. We retrospectively review our experience with, and long-term results and complications of combined bladder neck reconstruction and epispadias repair. A total of 19 boys with classic bladder exstrophy (17) and complete male epispadias (2) underwent combined bladder neck reconstruction and epispadias repair between 1982 and 1999. Primary closure was performed elsewhere in 16 cases and osteotomy was performed at primary closure in 8 (42%). All patients have undergone modified Cantwell-Ransley epispadias repair except for 2 who underwent a Young procedure. At the time of combined bladder neck and epispadias repair mean patient age was 5.2 years (range 2.5 to 10). Mean bladder capacity was 119 ml. (range 60 to 250). Of the 19 patients 13 (69%) are completely continent, and 2 (11%) are partially continent and 1 remained incontinent. Three patients did not gain satisfactory functional bladder capacity after combined repair, and underwent bladder augmentation and a continent diversion procedures. Combined bladder neck and epispadias repair is applicable in experienced hands but careful patient selection and long-term followup are the most important issues to develop criteria to select those best to undergo this procedure.

COMBINED BLADDER NECK RECONSTRUCTION AND EPISPADIAS REPAIR FOR EXSTROPHY-EPISPADIAS COMPLEX

The achievement of satisfactory continence in the management of classic bladder exstrophy remains a surgical challenge. During the last 20 years a staged approach to the management of the exstrophy-epispadias complex has been used at many exstrophy centers to attain this goal. In select cases repairs can be combined to reduce the number of mandatory operations to achieve continence. We retrospectively review our experience with, and long-term results and complications of combined bladder neck reconstruction and epispadias repair. A total of 19 boys with classic bladder exstrophy (17) and complete male epispadias (2) underwent combined bladder neck reconstruction and epispadias repair between 1982 and 1999. Primary closure was performed elsewhere in 16 cases and osteotomy was performed at primary closure in 8 (42%). All patients have undergone modified Cantwell-Ransley epispadias repair except for 2 who underwent a Young procedure. At the time of combined bladder neck and epispadias repair mean patient age was 5.2 years (range 2.5 to 10). Mean bladder capacity was 119 ml. (range 60 to 250). Of the 19 patients 13 (69%) are completely continent, and 2 (11%) are partially continent and 1 remained incontinent. Three patients did not gain satisfactory functional bladder capacity after combined repair, and underwent bladder augmentation and a continent diversion procedures. Combined bladder neck and epispadias repair is applicable in experienced hands but careful patient selection and long-term followup are the most important issues to develop criteria to select those best to undergo this procedure.

Penile reconstruction combined with bladder closure in the management of classic bladder exstrophy: illustration of technique

Objectives. Interest has increased in combining procedures during reconstruction of bladder exstrophy in an effort to reduce the number of procedures required for reconstruction and to improve results. This study illustrates our technique of reconstruction and summarizes our current experience with the combination of epispadias repair and bladder closure during initial reconstruction or following prior failed bladder closure. Methods. Twenty-four boys with classic bladder exstrophy underwent combined bladder closure and epispadias repair. The mean patient age was 20 months, and 18 boys had a prior failed closure. Osteotomies were performed in all patients. Results. No instances of bladder prolapse or dehiscence were noted on follow-up. Urethrocutaneous fistula developed in 7 patients. Eventual bladder neck reconstruction has been performed in 11 boys (6 boys are dry day and night, 3 are dry during the day with occasional wet episodes at night, 2 have required follow-up bladder augmentation and continent diversion for persistent incontinence), and 1 boy had augmentation at the same time as bladder neck reconstruction. Twelve boys are awaiting adequate capacity for bladder neck reconstruction, and 1 is awaiting bladder augmentation and continent stoma construction. Conclusions. Epispadias closure can be safely combined with bladder closure in select patients with classic bladder exstrophy. Complication rates and cosmesis approximate that achieved with staging the two procedures. This achievement represents strict patient selection and attentive follow-up.

COMPLETE PRIMARY REPAIR OF EXSTROPHY

The surgical correction of bladder exstrophy to achieve continence with voiding remains a challenging problem for the urologist. Since 1989 we have performed complete primary repair for exstrophy based on the concept that the primary defect of bladder and cloacal exstrophy is anterior herniation. Thus, the bladder and urethra must be treated as a single unit to move them posteriorly into the pelvis. We present this technique. From 1989 to 1997, 18 patients with bladder exstrophy and 6 with cloacal exstrophy underwent complete primary repair of exstrophy. This procedure was done on day 1 of life in 18 patients. Mean followup is 44 months (range 4 months to 8 years). At a median followup of 48 months 4 boys and 4 girls have volitional voiding after complete primary repair of bladder exstrophy, 21 patients have continent intervals and 2 boys void with continent intervals after complete primary repair of cloacal exstrophy. No patient has had a loss of renal function in this series. Postoperative complications included urethrocutaneous fistula formation in 2 cases. No patient had primary closure dehiscence. The rate of urinary continence achieved with complete primary repair compares favorably to that of staged repair for exstrophy. Complete primary repair also minimizes the number of surgical procedures required to achieve urinary continence and potentiates bladder neck function that permits bladder cycling in year 1 of life. The complication rates of these techniques are significantly lower than those reported in previous series of primary closure of exstrophy.

Bladder Exstrophy: Increase in Capacity Following Epispadias Repair

The achievement of satisfactory continence in the management of classical bladder exstrophy remains a surgical challenge. This may be particularly difficult in the patient with a small bladder capacity after initial closure. The 12-year experience at our institution with bladder exstrophy is reviewed and attention is focused on the approach to those patients with a small bladder capacity. A total of 155 children with bladder exstrophy were treated. Of these 155 patients 28 boys were believed to have a bladder capacity inadequate for satisfactory bladder neck reconstruction (less than 60cc). All patients have undergone staged reconstruction with a urethroplasty for epispadias and they are available for evaluation. After a median interval of 22 months bladder capacity increased by a median 54.5cc. No child had hydronephrosis after the epispadias repair or bladder neck reconstruction. Of the 28 patients 25 have undergone bladder neck reconstruction with a median followup of 4.5 years and 88 per cent (22 of 25) demonstrate continence with a dry interval greater than 3 hours. Another 2 patients have been rendered continent following bladder augmentation. These results demonstrate that satisfactory continence is an achievable goal in the exstrophy patient with a small bladder capacity when bladder neck reconstruction is preceded by epispadias repair.

The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy: Analysis of 144 patients treated at the Johns Hopkins Hospital between 1975 and 1985: J.E. Oesterling and R.D. Jeffs. J Urol 137:258–262, (February), 1987

The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy: Analysis of 144 patients treated at the Johns Hopkins Hospital between 1975 and 1985: J.E. Oesterling and R.D. Jeffs. J Urol 137:258–262, (February), 1987

The Importance of a Successful Initial Bladder Closure in the Surgical Management of Classical Bladder Exstrophy: Analysis of 144 Patients Treated at the Johns Hopkins Hospital Between 1975 and 1985

The staged functional closure of classical bladder exstrophy has produced improved results for many urologists involved in the surgical management of this congenital anomaly. To determine which factors are most important for achieving a successful outcome (urinary continence with preservation of normal renal function) the 144 patients treated at The Johns Hopkins Hospital between 1975 and 1985 were reviewed. Of these patients 51 were managed entirely at our institution, while 93 had had the initial surgical treatment elsewhere.All patients who had undergone primary bladder closure and bladder neck reconstruction were divided into 2 groups: group 1—patients who had a successful initial bladder closure (an exstrophied bladder that is converted into a complete epispadias without wound infection, dehiscence or any degree of bladder prolapse on the first attempt) and group 2—children whose initial bladder closure was not successful. Both groups were analyzed with respect to bladder capacity at the time of bladder neck reconstruction, time required for the bladder to become sufficiently large for bladder neck reconstruction, urinary continence rate, and interval between bladder neck reconstruction and achievement of urinary continence. Patients in group 1 had the largest bladders at the time of bladder neck reconstruction (mean capacity 79cc, p equals 0.03), shortest intervals between primary closure and bladder neck reconstruction (mean 3.5 years, p equals 0.006), highest urinary continence rate (92 per cent, p equals 0.002), and the shortest interval between bladder neck reconstruction and achievement of urinary continence (mean 1.5 years, p equals 0.18).These findings suggest that a successful initial bladder closure is an important factor for obtaining a larger bladder more quickly and for achieving a high urinary continence rate in patients with classical bladder exstrophy undergoing the staged functional bladder closure.

Muscarinic Cholinergic Receptors in Bladder Exstrophy: Insights Into Surgical Management

The surgical management of classical bladder exstrophy (functional bladder closure or urinary diversion) should be influenced by the inherent detrusor function of the exstrophied bladder. Cystometrograms performed previously on individuals with successful exstrophy closures demonstrate normal bladder function. The biochemical and neurophysiological properties of the exstrophied bladder have otherwise not been investigated. In this study radioligand receptor binding techniques were used to compare the density and equilibrium dissociation constant of muscarinic cholinergic receptors in control and exstrophy bladders. The density of muscarinic cholinergic receptors in the control and exstrophy groups was 1.97 plus or minus 0.29 and 1.44 plus or minus 0.21fmol. per μg. deoxyribonucleic acid (mean plus or minus standard error of mean), respectively. The dissociation constant of the control and exstrophy groups was 0.15 plus or minus 0.02 and 0.14 plus or minus 0.02nM. (mean plus or minus standard error of mean), respectively. These data show that the muscarinic receptor density and binding affinity in control and exstrophy bladders are similar. Therefore, the neurophysiological composition of the exstrophied bladder is not grossly altered during the anomalous development.

An Operation for the Cure of Incontinence Associated with Epispadias

An Operation for the Cure of Incontinence Associated with Epispadias