The dispute about the external sphincter and the urogenital diaphragm

PURPOSE: We present a modification of bladder neck reconstruction which resulted in improved continence and voiding compared to other techniques of bladder neck repairs in patients with exstrophy and complete incontinence. MATERIALS AND METHODS: The series consisted of 10 patients with the exstrophy-epispadias complex and complete incontinence who previously had undergone multiple operations for bladder closure, bladder neck reconstruction and epispadias repair. This modification combines bladder neck lengthening and narrowing of the distal half of the urethra, and submucosal embedding of the proximal half of the neourethra in the trigonal area. All patients also underwent bladder augmentation with detubularized sigmoid colon concurrent with bladder neck reconstruction. Additionally the appendical Mitrofanoff principle was applied to 5 cases. RESULTS: Of the 10 patients who underwent bladder neck reconstruction with sigmoid cystoplasty 8 are voiding voluntarily without catheterization and are dry for longer than 4 hours day and night. Only 2 patients are partially dry with stress nocturia incontinence and in both we performed a Mitrofanoff procedure as an adjunct to catheterization and to ensure voiding and continence. CONCLUSIONS: Our modified bladder neck reconstruction provides better overall voiding and continence rates than the other bladder neck/urethral reconstruction procedures in patients with exstrophy and complete incontinence.     

Combined bladder neck, urethral and penile reconstruction in boys with the exstrophy-epispadias complex

OBJECTIVE: To describe a one-stage combined bladder neck, urethral and penile reconstruction for achieving urinary continence and creating a penis with good cosmesis and function in boys with the exstrophy-epispadias complex. PATIENTS AND METHODS: Seventy-three boys underwent the combined procedure, including 36 after classic bladder exstrophy closure and 37 with epispadias. All were completely incontinent at the time of combined reconstruction. The bladder capacity just before surgery was 70-180 mL and was greater in those with epispadias. The boys were 2.5-11 years old, with those in the exstrophy group being slightly older. RESULTS: Thirty-three boys (89%) with epispadias were completely continent during the day but 15 had episodes of nocturnal enuresis. Of boys with classic exstrophy, 27 (75%) were continent during the day but nine had occasional nocturnal enuresis. Eleven boys required intermittent catheterization, which they found easy to perform. In seven boys (10%) a urethrocutaneous fistula or urethral stricture developed. CONCLUSIONS: Combined bladder neck, urethral and penile reconstruction can be carried out as a one-stage procedure in selected patients with adequate bladder capacity. Reconstruction of the whole length of the urethra facilitates intermittent catheterization.     

The role of the pelvic floor in female urinary incontinence

This paper presents a review of the various factors believed to be involved in female urinary continence. Components within the wall of the urethra include smooth muscle, the striated muscle of the rhabdosphincter, elastic connective tissue, a subepithelial vascular component, and the urethral epithelium. Extramural factors comprise the fascial support of the bladder neck and proximal urethra, the transmission of intra-abdominal pressure to the urethra, and the periurethral muscles of the pelvic floor. Special emphasis is placed on the periurethral muscles, and the anatomy, innervation, and histochemistry of the levator ani are discussed. This account is followed by consideration of evidence that partial denervation of the levator ani may be an etiological factor in female genuine stress incontinence of urine. Finally, various non-invasive methods for the relief of genuine stress incontinence are discussed, including pelvic floor exercises and the use of intravaginal cones and electrostimulation.     

Management of epispadias

Although epispadias is considered to be the least severe defect of the exstrophy-epispadias complex, the treatment of this anomaly is far from trivial. Epispadias does not involve the body of the bladder or the hindgut but does affect the urethra and can affect the bladder neck. As a consequence, it presents with a spectrum of severity that can affect urinary continence if the epispadias anomaly is proximal enough to affect the urinary sphincter mechanism.     

Mechanism of Postoperative Urinary Continence

Stress incontinence used to be attributed mostly to urethral hypermobility, and consequently most surgical techniques focused on the region of the bladder neck and proximal urethra. This article reviews our knowledge about the mechanism of postoperative urinary continence based on anatomic, imaging and urodynamic studies. Reduction of urethral mobility, as measured by cotton swab testing or imaging studies, is not the only reason why continence surgery succeeds. Imaging techniques are of limited value for elucidating the continence mechanism because radiologic landmarks and criteria are not reproducible. Urodynamically, the increased pressure transmission after successful continence surgery is attributed to the retropubic repositioning of the urethra, its compression against the anterior vaginal wall, and improved transmission of intra-abdominal pressure during stress. The role of the ‘functional’ urethral obstruction remains to be studied. In incontinent patients with hypermobility of the bladder neck and proximal urethra continence can be achieved by surgical correction. However, stress incontinence is possible in the absence of urethral hypermobility, and standard surgical techniques can fail to restore continence in these patients.     

Role of the pelvic floor in Bladder neck opening and closure II: Vagina

The aim of the study was to examine the role of vaginal stretching during bladder neck opening and closure. The study group comprised 12 patients with GSI and 4 controls. The position of the bladder neck relative to the vagina was assessed in the resting, straining and ‘squeezing’ positions using video-radiological studies. Radio-opaque dye was instilled into the bladder, vagina, rectum and levator plate. Vascular clips applied to the midurethral, bladder neck and bladder base parts of the anterior vaginal wall assisted in determining differential movements of these parts of the vagina during bladder neck opening and closure. The suburethral vagina (hammock) was shown to stretch downwards and forwards during straining, and downwards and backwards during micturition. The bladder neck, upper part of the vagina and the rectum were stretched backwards and downwards in an identical manner during straining and micturition, apparently in response to backward contraction of the levator plate and downward angulation of its anterior lip. All organs were stretched upwards and forwards during ‘squeezing’. The findings support the hypothesis that, during stress and micturition, selective pelvic floor contractions stretch the vagina against intact pubourethral and uterosacral ligaments to assist opening and closure of the urethra and bladder neck. EDITORIAL COMMENT: The authors propose a new theory for the mechanism of micturition and continence. The new ‘Integral Theory’ describes the role of the vagina and three pelvic floor muscles, the levator plate, the anterior portion of the pubococcygeus (PCM) and the longitudinal muscles of the anus (LMA) in the opening and closing of the urethra. Unlike the ‘hammock hypothesis’, proposed by Delancey, the authors believe it is the forward movement of the vagina around its attachment to the pubourethral ligaments via contraction of the PCM that closes off the urethra, and not increased abdominal pressure transmission to the proximal urethra. Their proposed mechanism for incontinence is laxity of the vaginal hammock and decreased forward motion, and less compression of the urethra. This theory is also in contrast to Shafik’s ‘common sphincter’ concept of continence, which stresses the importance of the puborectalis and external urethral sphincter as the main mechanism for continence, with no significance given to the role of the anterior vaginal wall or its attachment and movement around the pubourethral ligament. This new integral theory is based on findings of muscle movements using cadaveric dissection, video X-ray studies, digital palpation, EMG and dynamic urethral pressure measurements. This is an interesting theory which again emphasizes the role of the pelvic floor muscles, not only in their support function but also in their role of active movements of the viscera within the bony pelvis.     

A case of female epispadias without exstrophy treated with a novel vulvoplasty method

Epispadias surgery has mainly two components: bladder and genitourethral reconstruction. Herein, a novel vulvoplasty method, an alternative for the latter component, carried out in a rare case of female epispadias was presented. In a 6-year-old girl admitted with complaint of total incontinence, epispadias without evident extrophy associated with adequate bladder capacity was determined. Firstly the vulvoplasty in which a circumferencial skin flap around the urethral meatus was rolled backwards, and secondly bladder neck reconstruction was performed. The vulvoplasty resulted in sufficient cosmetic outcome and continence up to half an hour, and after cervicoplasty total day and night continence at rest or on exertion were obtained. It was concluded that this vulvoplasty with sufficient cosmetic outcome could be considered as a first stage operation in female epispadias, because it could facilitate bladder neck reconstruction by increasing urethral resistance, and contribute to continence. This revised version was published online in August 2006 with corrections to the Cover Date.     

Periurethral muscle complex reassembly for exstrophy-epispadias repair

PURPOSE: Continence is a difficult goal in exstrophy-epispadias complex repair. It is presumed that all anatomical components involved in the exstrophy-epispadias abnormality are present but laterally and anteriorly displaced. The penile disassembly technique for epispadias restores the normal anatomical relationship of the male genital components. Its extension to complete primary bladder exstrophy closure enables deeper positioning of the bladder neck within the pelvic diaphragm. We identified the perineal striated muscular complex and present its appropriate periurethral reassembly as a main step in exstrophy-epispadias complex repair. MATERIALS AND METHODS: Bladder exstrophy and epispadias repairs were performed in 10 male and 3 female consecutive patients with the exstrophy-epispadias complex, including 1-stage reconstruction in 2 male newborns and 2 females with exstrophy, and as further surgery in a female with cloacal exstrophy and previous failed 1-stage repair, 4 males with incontinent epispadias (secondary repair in 1) and 4 males with epispadias in whom exstrophy closure had been previously done. In the males after bladder plate closure and corporeal body splitting a sagittal incision was made in the intersymphyseal tissue and extended posteriorly to the perineal body midline. The bipolar electrical stimulator was used to identify pelvic muscle components in the sagittal plane and reapproximate them along the tubularized posterior urethra to form the periurethral muscle complex. In the 3 females the urethral plate and vagina were similarly mobilized posterior through the sagittal incision of the perineal body. No patient underwent bladder neck plasty. RESULTS: At 9 months to 4 years of followup cosmesis was good in 12 patients, while 1 required secondary glanular urethroplasty. There was mild pyelectasis in 3 cases but no severe hydronephrosis and no renal function deterioration. Pyelonephritis developed in 6 patients (46%). Cystography at 1 year showed that bladder capacity was 35 to 80 and 65 to 120 cc in exstrophy and epispadias cases, respectively. There was cyclic voiding with 30 to 90-minute dry intervals in 7 patients (54%), of whom 5 had exstrophy and 2 had epispadias. Daytime voiding control with a 2 to 3-hour voiding interval was achieved in 1 female with exstrophy and 2 patients with epispadias (23%). Incontinence was present in 2 patients with previous exstrophy closure and 1 with cloacal exstrophy (23%). CONCLUSIONS: Early restoration of a physiological vesicourethral balance of coordinated activity is feasible for the progressive achievement of continence in patients with the exstrophy-epispadias complex. Sagittal splitting of the perineal tissue with identification of the muscle components as well as midline reassembly of the periurethral striated muscular complex helps to reconfigure the pelvic anatomy in a more normal fashion and allows better restoration of coordinated vesicourethral activity.     

The significance of the open bladder neck associated with pelvic fracture urethral distraction defects.

PURPOSE: As a result of pelvic fracture urethral distraction defects, urinary continence relies predominantly on intact bladder neck function. Hence, when cystoscopy and/or cystography reveals an open bladder neck before urethroplasty, the probability of postoperative urinary incontinence may be significant. Unresolved issues are the necessity, the timing and the type of bladder neck repair. We report the outcome of various therapeutic options in patients with pelvic fracture urethral distraction defects and open bladder neck. We also attempt to identify prognostic factors of incontinence before urethroplasty. MATERIALS AND METHODS: We retrospectively reviewed the records of 15 patients with a mean age of 30 years in whom an open bladder neck was identified before posterior urethroplasty between January 1981 and October 1997. RESULTS: Of the 15 patients 6 were continent and 8 were incontinent postoperatively. One patient underwent artificial urethral sphincter implantation simultaneously with pelvic fracture urethral distraction defect repair and was dry postoperatively without sphincter activation. Average bladder neck and prostatic urethral opening on the cystourethrogram before urethroplasty was significantly longer in incontinent (1.68 cm.) than in continent (0.9 cm.) patients. Of the 8 patients who were incontinent 6 underwent bladder neck reconstruction, 1 artificial urinary sphincter and 1 periurethral collagen implant. Five patients with bladder neck reconstruction are totally continent and 1 requires 1 pad daily. The patient who underwent collagen implant requires 2 pads daily and the patient who received an artificial urethral sphincter has minor urge leakage. CONCLUSIONS: Open bladder neck before urethroplasty may herald postoperative incontinence which may be predicted by radiographic and cystoscopic features. Evaluation of the risk of postoperative incontinence may be valuable, and eventually guide the necessity and timing of anti-incontinence surgery, although our preference remains to manage the pelvic fracture urethral distraction defects and bladder neck problem sequentially. Bladder neck reconstruction provides good postoperative continence rates and is our technique of choice.     

Bolsa ileocecal con utilización del apéndice como neouretra en pacientes dispuestos a sustitución vesical ortotópica: descripción de la técnica

IntroductionThe authors describe the technique of orthotopic bladder replacement with an ileocecal pouch and unaltered appendix used as an orthotopic urethral substitute. Additional procedures with regard to the bothersome voiding symptoms will be described.Material and methodsIn a small cohort of 5 patients with muscle invasive bladder cancer with tumor involvement of the bladder neck or proximal urethra (2 males/3 females) we performed the following reconstruction. A low pressure reservoir was achieved by antimesenteric longitudinal transection of terminal ileum and cecum/colon ascendens and formation of a pouch. To develop the neourethra, the appendix together with its accompanying mesentery was drawn through the pelvic floor and sutured to the bulbar urethra in males or formed as a complete neourethra in female patients respectively.ResultsThere were no intraoperative nor early postoperative unwanted sequelae. Both male patients experienced recurrent anastomotic urethral stricture, consequently a Memokath stent and artificial urinary sphincter was implanted resulting in normal voluntary micturition. All female patients remained socially continent during the follow up period, one of them performing clean intermittent catheterization (CIC).ConclusionThe technique described offers the possibility of orthotopic bladder replacement even in traditionally unsuitable, but highly motivated patients, who are requesting orthotopic bladder replacement for improved body image. It allows extension of urethral resection and provides additional continence support. However, additional measures such as urethral stenting, CIC or artificial urinary sphincter implantation may be necessary for long lasting success. Although, not being a routine method for urinary diversion this technique may be used in select patients.     

Endoscopic treatment of complete urethral obstruction using thin trocar

We used an endoscopic thin trocar to reestablish the continuity of the completely obliterated urethra in 2 patients. In 1 man the obstruction resulted from a pelvic fracture and in 1 woman from early removal of the urethral catheter after a bladder neck reconstruction. We found this technique safe and effective, and we consider it to represent an improvement over previously described methods of endoscopic treatment of the obliterated urethra.     

Pelvic fracture urethral injuries in girls

PURPOSE: Injuries to the female urethra associated with pelvic fracture are uncommon. They may vary from urethral contusion to partial or circumferential rupture. When disruption has occurred at the level of the proximal urethra, it is usually complete and often associated with vaginal laceration. We retrospectively reviewed the records of a series of girls with pelvic fracture urethral stricture and present surgical treatment to restore urethral continuity and the outcome. MATERIALS AND METHODS: Between 1984 and 1997, 8 girls 4 to 16 years old (median age 9.6) with urethral injuries associated with pelvic fracture were treated at our institutions. Immediate therapy involved suprapubic cystostomy in 4 cases, urethral catheter alignment and simultaneous suprapubic cystostomy in 3, and primary suturing of the urethra, bladder neck and vagina in 1. Delayed 1-stage anastomotic repair was performed in 1 patient with urethral avulsion at the level of the bladder neck and in 5 with a proximal urethral distraction defect, while a neourethra was constructed from the anterior vaginal wall in a 2-stage procedure in 1 with mid urethral avulsion. Concomitant vaginal rupture in 7 cases was treated at delayed urethral reconstruction in 5 and by primary repair in 2. The surgical approach was retropubic in 3 cases, vaginal-retropubic in 1 and vaginal-transpubic in 4. Associated injuries included rectal injury in 3 girls and bladder neck laceration in 4. Overall postoperative followup was 6 months to 6.3 years (median 3 years). RESULTS: Urethral obliteration developed in all patients treated with suprapubic cystostomy and simultaneous urethral realignment. The stricture-free rate for 1-stage anastomotic repair and substitution urethroplasty was 100%. In 1 girl complete urinary incontinence developed, while another has mild stress incontinence. Retrospectively the 2 incontinent girls had had an associated bladder neck injury at the initial trauma. Two recurrent vaginal strictures were treated successfully with additional transpositions of lateral labial flaps. CONCLUSIONS: This study emphasizes that combined vaginal-partial transpubic access is a reliable approach for resolving complex obliterative urethral strictures and associated urethrovaginal fistulas or severe bladder neck damage after traumatic pelvic fracture injury in female pediatric patients. Although our experience with the initial management of these injuries is limited, we advocate early cystostomy drainage and deferred surgical reconstruction when life threatening clinical conditions are present or extensive traumatized tissue in the affected area precludes immediate ideal surgical repair.     

Urethral function after cystectomy: a canine in vivo experiment

Summary To study the function of the pelvic floor and the isolated urethra after removal of the bladder, 5 male and 5 female mongrel dogs were used in an acute in vivo experiment. Urethral pressure changes secondary to unilateral stimulation of the pelvic and pudendal nerves were recorded. After baseline data of the intact system were documented, the following procedures were carried out: separation of the urethra from the bladder neck (prostate), nerve-sparing cystectomy (cystoprostatectomy), and cold-knife incision through the entire length of the proximal urethra. Pressure recordings were repeated after each step of surgery. Pudendal nerve stimulation resulted in rapid and large pressure rises in the distal urethra (reaction typical of striated muscle). This response remained unchanged after all three surgical steps. Pelvic nerve stimulation provoked pressure rises within the urethra of a pattern typical of smooth muscle. The findings persisted after separation of the urethra from the bladder neck (prostate) and after cystectomy, but were not observed after urethrotomy. Contractions secondary to pudendal nerve stimulation were inhibited by curare, which did not affect the reaction to pelvic nerve stimulation. Our experiments demonstrate that in the dog the continuity of bladder and urethra is not required for the function of urethral closure mechanisms. The contractile potency of the urethral smooth muscles remains intact after nerve-sparing cystectomy. We believe that problems with the baseline continence of surrogate bladders should mainly be ascribed to a lack of surgical caution in preserving the autonomic nerves of cystectomy. A poor response to stress conditions cannot be explained by damage to the neural pathway of the striated sphincter, as the pudendal nerve is not at risk during nerve-sparing cystectomy. In our opinion mechanical malfunction of the striated muscle components secondary to scarring at the site of the anastomosis is the main reason for stress incontinence after orthotopic bladder replacement.     

The staged approach to bladder exstrophy closure and the role of osteotomies

Since the 1970's, the staged reconstruction of bladder exstrophy has yielded consistent surgical success. The Johns Hopkins Hospital approach begins with early pelvic ring approximation with abdominal wall, bladder, and posterior urethral closure. Within the first 72 hours of life, the malleable pelvis can sometimes be approximated without osteotomies. Beyond this age, the author's prefer a combined vertical iliac and horizontal innominate osteotomy. Second, we typically perform the epispadias closure at 1 year of age. A modified Cantwell-Ransley technique is performed, usually yielding an increase in bladder capacity and very satisfactory results. In the last phase, the modified Young-Dees-Leadbetter continence procedure along with transtrigonal/cephalotrigonal ureteroneocystostomies are performed when the urethra is catheterizable, the bladder capacity is 60cc or greater, and the child will participate in a postoperative voiding program (typically 4–5 years of age). This applied approach usually results in a continent, voiding patient with pleasing external genitalia and preserved renal function.     

Success of the artificial urinary sphincter after failed surgery for incontinence

We implanted the artificial urinary sphincter in 15 incontinent patients for whom multiple urethral and bladder neck operations, including sphincter placement, had been unsuccessful. The 5 male and 10 female patients ranged from 3 to 26 years old (mean age 11 years). The etiology of incontinence was neurogenic bladder in 10 patients, epispadias in 2, exstrophy in 1, ectopic ureters in 1 and traumatic urethral disruption in 1. Of the 15 patients 13 required augmentation enterocystoplasty and clean intermittent catheterization. The initial anti-incontinence procedures were Young-Dees-Leadbetter bladder neck reconstruction in 10 patients, artificial urinary sphincter placement in 4 and bladder neck suspension in 1. Causes of failure of the primary treatment were erosion (artificial urinary sphincter), and incontinence and/or difficult catheterization (Young-Dees-Leadbetter). Followup from the last salvage operation averaged 21 months (range 6 to 37 months). A total of 58 operations was performed. Among the 4 patients in whom the artificial urinary sphincter eroded the bladder neck repeated attempts to place the cuff at the same site were unsuccessful and erosion occurred in all 4 within 1 year. Sphincter placement was more successful among the 11 patients who initially underwent Young-Dees-Leadbetter bladder neck reconstruction or bladder neck suspension; acceptable continence was attained in 8 patients (73%). We conclude that placement of the sphincter cuff around a previously eroded bladder neck probably will result in erosion. Sphincter implantation should be attempted in patients in whom bladder neck reconstruction has failed. Persistence in the treatment of these patients is essential because multiple operations often are necessary to achieve continence.     

Is there a shortening of the urethral plate in complete penile disassembly used in epispadias repair,and what is its impact on the final outcomes?

Purpose

To assess the importance of shortening of the urethral plate that occurred with complete penile disassembly technique in epispadias repair and its impact on cosmetic and functional results (on urinary incontinence).

Surgical correction of urinary incontinence

Over the last 6 years, 114 patients have undergone surgery for urinary incontinence. The majority (79%) had neurologic dysfunction of the bladder because of spinal malformation (myelodysplasia, sacral agenesis, or trauma) and the remaining were a mixed group including exstrophy/epispadias, urethral valves, pelvic fractures, etc. The patients were grouped in six categories. Those with lower urethral resistance underwent bladder neck reconstruction with Young-Dees-Leadbetter procedure (five patients) or had placement of an artificial urinary sphincter (27 patients). Those with poor bladder compliance underwent primary bladder augmentation (21 patients). Those with combined urethral problems and poor compliance had combined procedures (14 patients). Thirty-seven patients previously diverted for incontinence and undergoing undiversion were considered separately, as were ten patients without any bladder precluding preoperative assessment. Of the entire group, continence was achieved in 83 patients with the initial procedure (73%). Secondary procedures have resulted in continence in 101 patients (89%). Three patients were improved but unsatisfactory, and nine remain wet; one is unknown.     

Effects of selective autonomic and pudendal denervation on the urethral function and development of retention in female dogs

PURPOSE: This trial is an experimental approach to the possible causes of continence and voiding problems after urethra sparing radical cystectomy and orthotopic bladder substitution in women. MATERIALS AND METHODS: Between January 1996 and January 1999 we included 24 mongrel female dogs in this 4-phase study of 6 dogs each. The effects of autonomic denervation of the urethra (phase 1) and urethral transection just distal to the bladder neck (phase 2) on the urethral pressure profile were recorded. In phase 3 the effects of autonomic denervation, urethral transection and pharmacological manipulation of the denervated transected urethra on the urethral pressure profile were studied in succession. In phase 4 the effects of pudendal nerve transection and pharmacological blockade were recorded. In the 12 phases 2 and 3 dogs the transected urethra was re-anastomosed to the bladder neck. Acute experiments were repeated after 2 and 6 months, urethrocystoscopy was done and post-void residual urine was estimated. Two of the latter dogs were sacrificed 6 months after the acute experiment and the urethras were histopathologically examined. RESULTS: Autonomic denervation resulted in a 46% to 48% decrease in mean maximal pressure in the proximal urethra in phases 1 and 3 (p <0.001) with no significant effect on the distal urethra. Urethral transection in phase 2 did not affect the urethral pressure profile. Phentolamine injection after urethral denervation and transection in phase 3 produced a further reduction of 11.3% and 46.3% in mean resting pressure in the proximal and distal urethra, respectively, while succinyl choline produced a 38.1% further decrease in the distal urethra. Unilateral and bilateral pudendal denervation reduced pressure in the distal urethra significantly but not in the proximal urethra. When phentolamine was given thereafter, a further decrease of 38% and 2.4% resulted in resting pressure values in the proximal and distal urethra, respectively. The change in distal urethral pressure was marginally significant after succinyl choline injection (p = 0.05). Results were reproducible after 2 and 6 months. The proximal urethra remained patent with no post-void residual urine after autonomic denervation. There was no significant urethral fibrosis after realignment of the transected urethra in the 2 sacrificed phases 2 and 3 dogs. CONCLUSIONS: From this study we concluded that autonomic denervation reduced pressure in the proximal urethra by less than 50%. Continuity of the urethra with the bladder is not necessary for proper urethral function. After autonomic denervation the proximal urethra remained patent with no subsequent fibrosis. In addition, no post-void residual urine was noted. Bilateral pudendal denervation did not completely block activity of the distal urethra. The nonneuromuscular components had a small role in the creation of urethral closure function.     

Reconstruction of the female urethra: versatility, complexity and aptness

PURPOSE: Female urethral anomalies, whether congenital or acquired, are rare. Urethral defects are usually if not always associated with variable degrees of incontinence. In this case series we demonstrate the approach in management and surgical outcome of congenital and traumatic urethral anomalies. MATERIALS AND METHODS: The study was conducted on 13 patients with an age range of 2 to 38 years (median 20). Of these patients 4 had female epispadias, 1 had hypospadias, 3 had traumatic urethral loss and 2 had iatrogenic trauma involving the urethra. There were 2 patients with urogenital sinus syndrome and 1 patient had urethral prolapse. RESULTS: After the first stage of repair 4 patients were dry and socially satisfied, and no further intervention was needed. However, in 9 patients a second intervention was necessary to achieve continence. Notably 3 patients empty the bladder through clean intermittent catheterization. CONCLUSIONS: Female urethral defects are usually complex. Congenital causes are associated with severe incontinence. Repair of such defects is challenging, yet 1-stage reconstruction is feasible and potentially successful.     

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 79 cc, 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.