Modern staged repair of bladder exstrophy: a contemporary series.

OBJECTIVE: Many 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 METHODS: From 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. RESULTS: Sixty-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 3h 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. CONCLUSIONS: Patients 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.

Reconstructive lower urinary tract surgery in incontinent adolescents with exstrophy/epispadias complex.

OBJECTIVES: To outline the management strategies applied to the adolescent patient population with exstrophy/epispadias and incontinence at our institution. These patients present a difficult management problem. At the same time they are dealing with difficult issues, including body image and sexual awareness, the added burden of ongoing incontinence causes major anxieties and lifestyle restrictions. In many, incontinence has persisted despite numerous operations. METHODS: A total of 25 (19 male and 6 female) patients who remained incontinent into adolescence or early adulthood were reviewed. Of the 25 patients, 19 had exstrophy, 4 had cloacal exstrophy, and 1 male and 1 female had epispadias. Six patients had undergone eight prior attempts at continent reconstruction, one had undergone cutaneous diversion, and one had problems after ureterosigmoidostomy. RESULTS: The mean age at continence surgery was 12.9 years. Of the 25 patients, 18 underwent bladder augmentation, with a continent stoma in 17 and an artificial sphincter in 1. An additional 5 patients underwent bladder neck transection, with a new continent stoma in 3. The ureterosigmoidostomy was converted to a Mainz II pouch. One patient underwent continent neobladder formation. Nine patients (36%) developed complications during follow-up. Three required stoma revision for stenosis and one for prolapse. Pouch stones occurred in 4 patients, and vesicocutaneous fistula developed in 1. All achieved full urinary continence. The mean follow-up was 72.4 months. CONCLUSIONS: Some children with exstrophy/epispadias reach adolescence and remain incontinent. For these patients, modern reconstructive techniques provide hope of continence. With careful preoperative assessment, exact surgical precision, and regular follow-up, a successful outcome can be expected in virtually all cases without the need for external urine collection devices.

The use of combined bladder and epispadias repair in boys with classic bladder exstrophy: outcomes, complications and consequences.

PURPOSE: Despite widespread use of modern staged reconstruction for classic bladder exstrophy, there remains a role for combined bladder closure and epispadias repair when primary closure is delayed or initial reconstruction has failed. The principle of combining bladder and urethral closure in 1 operation was first proposed more than 40 years ago, and represents a demanding technical procedure. We recount our experience to date with this approach. MATERIALS AND METHODS: A total of 38 boys underwent combined bladder and epispadias repair using pelvic osteotomies. Five cases were delayed primary closures owing to a bladder template unsuitable for newborn closure. A total of 30 cases were previous failed bladder closures, with concurrent epispadias repair in 6. The 3 remaining cases were staged closures where the epispadias repair failed, leading to bladder prolapse via the posterior urethra. Overall, there was a major bladder prolapse in 25 cases, and separation of the pubic symphysis with dehiscence of anterior abdominal wall structures and bladder in 8. RESULTS: Mean age at surgery was 26.5 months for the 33 reclosures. A total of 10 boys had development of a urethrocutaneous fistula and 4 had development of strictures. A total of 19 patients required additional procedures of the bladder neck (endoscopic), urethra or penis. A later bladder neck reconstruction was undertaken in 19 boys, of whom 12 are continent. Seven boys underwent continent diversion and 5 are considering the procedure. A total of 14 boys are awaiting adequate capacity for bladder neck reconstruction. Ureteral reimplantation was performed in 22 patients, and no patient was rendered hypospadiac. CONCLUSIONS: When combined closure is applied to failed previous exstrophy repair one can expect at best a 50% continence rate without continent diversion. Furthermore, based on the experience at this institution the majority of patients require ureteral reimplantation, and many require additional surgery to the penis or urethra. However, with the application of modern reconstructive techniques continence and a cosmetically pleasing phallus can be expected in most cases, although at the expense of multiple surgical procedures.

The place of pelvic osteotomy in the modern era of bladder exstrophy reconstruction.

BACKGROUND: The place of pelvic osteotomy in reconstructing bladder/cloacal exstrophy has been debated for some time; the experience with 'combined' osteotomy in primary and re-operative exstrophy closure at this institution is presented, with a discussion of the historical and scientific place of osteotomy in managing this condition. PATIENTS AND METHODS: Sixty-eight patients had bilateral vertical and transverse iliac osteotomy between 1992 and 2003, and with outcome data available. Of 58 patients with classic exstrophy, eight were newborns, eight were deliberately delayed primary closures, 36 were re-operative after previous failed closure and six were bladder neck reconstructions where the bladder outlet was very wide, such that bony closure was felt necessary for successful bladder neck coaptation. Of 10 patients with cloacal exstrophy, nine were primary closures and one was a re-operative closure. Data were collected relating to age at closure, complications and continence outcome. RESULTS: The mean (range) age (months) was 41 (5-179) for re-operative closures, 12.5 (3-32) for delayed primary closures, 64.1 (38-79) for bladder neck reconstruction, 51.4 (6-165) for cloacal exstrophy closure, and 15 (2-45) days for newborn exstrophy closure. There was a superficial wound infection in two patients, pin-site infection in one, loose pins in two, and two had transient femoral nerve palsy. In two patients the procedure failed and they required further re-operative closure with osteotomy. Sixteen patients are dry urethrally day and night, 12 have had and four are awaiting bladder augmentation, one has a colon conduit, and 35 are awaiting a definitive continence procedure. CONCLUSIONS: Osteotomy has a proven track record in the field of exstrophy reconstruction, and the benefit especially in re-operative closure is emphasized by the present results. The surgical morbidity with the 'combined osteotomy' is low, cosmetic results are excellent and the effect on success of closure is clearly advantageous.

Applications of the modified Cantwell-Ransley epispadias repair in the exstrophy-epispadias complex.

OBJECTIVE: The Cantwell-Ransley technique is the most popular and widely used approach to epispadias repair. This is an evaluation and update of the long-term results of using the modified Cantwell-Ransley technique for epispadias repair. PATIENTS AND METHODS: The modified Cantwell-Ransley epispadias repair technique was performed on 129 boys of which 97 had classic bladder exstrophy and 32 complete epispadias. For 106 boys this was primary urethral repair (82 with classic exstrophy, 24 with epispadias) and for the other 23 boys it was a repeat repair (15 with exstrophy, eight with epispadias). RESULTS: At a mean follow-up of 88 months, 120 had a penis that was inclined downward or horizontally while standing. In patients with exstrophy, fistulae were noted in 16% and 33% after primary and repeat urethral repair, respectively. In patients with epispadias, fistulae were noted in 13% and 25% after primary and secondary repair, respectively. In total, five boys with a fistula appearing in the immediate postoperative period following primary urethral repair demonstrated spontaneous healing by 3 months' follow-up. Urethral stricture requiring treatment developed in nine patients. Minor wound infection and skin separation occurred in nine with exstrophy and three with epispadias. Endoscopic examination or catheterization in 120 cases revealed an easily manipulated neourethra. Of 15 sexually active patients, all reported orgasms and ejaculation with a straight penis on erection, although one has complained that his penis is shorter since surgery. CONCLUSIONS: The modified Cantwell-Ransley technique for epispadias repair produces durable functional and cosmetic results, and fewer major complications than seen with other repairs. Fistulae occurring after primary urethral repair may close spontaneously, but all those occurring after repeat closure will require further surgery.

Coping with bladder exstrophy: diverse results from early attempts at functional urinary tract surgery.

OBJECTIVES: To report the long-term surgical outcome in a group of patients with bladder exstrophy treated from 1960 to 1982, and to assess physical health, social integration and sexual function, as attempts at functional closure during the development of this surgery resulted in patients with differing surgical status of the genitourinary organs. PATIENTS AND METHODS: A review of medical record archives revealed 36 patients; of these, seven had died, six were untraceable and six declined to participate, leaving 15 evaluable subjects (seven men and eight women, mean age at follow-up, 35 years). Data were collected from medical records and direct interviews were conducted using a semi-structured questionnaire, after obtaining informed consent. The Short Form-36 (SF-36) v2 Health Survey instrument was used to assess health and well-being. RESULTS: Three patients have retained the use of their bladder, and 11 had initial bladder closure but required (at a mean age of 3.1 years) various urinary diversions. Eight patients had a pelvic osteotomy. Of 27 functioning renal units, 25 are in reasonable to good condition. Six men and six women had genital reconstruction. Four men are capable of penetrative intercourse and ejaculate; six women manage penetrative intercourse and five have orgasms. All patients attended mainstream school and 13 achieved examination success. Nine patients took vocational training and 11 work full-time. Five patients are married and five are in long-term relationships. Two men have achieved three pregnancies and one women has had a child. The mean total SF-36 score (maximum 3600) was 2763 in men and 2235 in women. CONCLUSIONS: Surgery for bladder exstrophy has been developing for more than 40 years and the legacy of early attempts at functional closure is a population of adults who have a diversity of lower urinary tracts, good preservation of renal function overall and acceptable sexual function. They are usually robust, healthy and well-adjusted individuals functioning well in society, often in full-time employment and long-term relationships. Adolescent follow-up must be clearly focused, incorporating a multidisciplinary team approach to facilitate a seamless transition into adulthood.

Outcome and survival with nonsurgical management of renal cell carcinoma.

OBJECTIVE: To document long-term survival in patients with renal cell carcinoma (RCC) in whom the primary tumour was left in situ and treatment limited to palliative and symptomatic measures. PATIENTS AND METHODS: All patients with a diagnosis of RCC from January 1994 to January 1999 and in whom the primary tumour was left in situ were identified from hospital records (nine women and 16 men, mean age 69 years). The tumour stage was T1-T4. RESULTS: The mean survival overall was 19.3 months; patients with locally advanced disease, i.e. stage >or= T3a, had a mean survival of 16.9 months. CONCLUSIONS: There is renewed interest in the management of advanced RCC, with data supporting cytoreductive nephrectomy with systemic biological therapy. These results confirm that such patients with or without metastatic disease can survive for a considerable period with no aggressive surgical or systemic measures, and such intervention may offer no significant advantage in outcome and survival over supportive treatment alone.

Testicular microlithiasis needs long-term surveillance.

We present a case of testicular microlithiasis in which testicular biopsy failed to predict the development of a testicular tumour.

Neuropsychological deficits and real-world dysfunction in cerebral revascularization candidates.

An assessment strategy using multiple levels of observation permitted description of problems in everyday living experienced by cerebral revascularization candidates. We contrasted the neuropsychological deficits and real-world dysfunction displayed by candidates for cerebral revascularization with that manifested by patients with severe spinal complaints. Correlations between selected neuropsychological scores and life quality measures were modest. Prediction of real-world performance in individuals likely will require multivariate combinations of measures. Relationships between life quality measures and neuropsychological scores may differ for patients with known or suspected neurological disease and patients with disorders which do not threaten cognitive functioning.

Cerebral angiography and neuropsychological measurement: the twain may meet.

In a sample of 31 cerebral revascularization candidates, severity and dissemination of atherosclerosis on the cerebral angiogram were correlated with two of three global indicators of neurobehavioral impairment. Additionally, the angiographic rating was correlated with age and with an index of medical risk factors, but not with duration of the longest symptomatic episode. It seems likely that several variables, particularly collateral circulation, help to determine whether a given pattern of stenoses results in neuropsychological dysfunction and what type of behavioral deficit occurs. In many cases, the configuration of neuropsychological test scores may not directly mirror the pattern of cerebrovascular stenoses.

Can neuropsychological tests detect the sites of cerebrovascular stenoses and occlusions?

Conventional neuropsychological data may not mirror the site of cerebrovascular stenosis in a patient with mild to moderate symptoms. In this study, three groups of cerebral revascularization candidates failed to differ on an extended Halstead-Reitan battery: patients with symptoms and angiographic results referable to the carotid arterial system, subjects with clinical signs and neuroradiological findings referable to the vertebrobasilar arterial system, and patients with symptoms of cerebral ischemia but negative angiograms. Consistent with previous work, these patients generally were mildly impaired on neuropsychological tests, even though many were not symptomatic at the time of assessment. Neuropsychological tests seem to be sensitive to the presence and severity of cerebral ischemia, but in isolation may not be sufficient to determine the loci of vascular stenoses. The incongruence between angiographic and neuropsychological findings underlines the importance of distinguishing among different levels and types of measurements in defining subgroups of cerebral revascularization candidates. Collaboration among neuroscientists will further our understanding of the interrelationships among neurodiagnostic tests.