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PubMed2000

Bladder neck reconstruction in classic bladder exstrophy: the role of osteotomy in the development of continence.

OBJECTIVE: To assess the role of osteotomy at the time of bladder neck reconstruction (BNR) for continence in classic bladder exstrophy, in which closure of the pelvic ring and reconstitution of the pelvic diaphragm may affect eventual continence. PATIENTS AND METHODS: The results of using osteotomy at the time of BNR in 29 children were reviewed. The mean bladder capacity before BNR was 76 mL. The indications for osteotomy were a wide pubic diastasis and a soft intersymphyseal bar. After osteotomy, all children were maintained in external fixation and lower-extremity traction for 6-8 weeks. RESULTS: Complications of osteotomy were limited to a partial femoral nerve palsy (one patient) and delayed union of fragments (one patient). Complications of BNR included urethral stricture (five patients) and bladder calculi (six patients). Continence results were modest, with 11 of 29 children (38%) dry during the day (dry interval >3 h) and eight of 29 (28%) dry at night. Eight children had daytime dry intervals of approximately 3 h. The mean preoperative bladder capacity in children who were dry both day and night was 101 mL. CONCLUSIONS: The preoperative bladder capacity remains a key determinant for the attainment of continence after BNR in the reconstruction of classic bladder exstrophy. Osteotomy allows pelvic closure and thus improves cosmesis of the mons and stabilizes the BNR in patients with a soft intersymphyseal bar, but seems to have no effect on continence when performed at the time of bladder neck plasty.

Bladder Exstrophy↗
PubMed1995

Operative reconstruction of the external and internal genitalia in female patients with bladder exstrophy or incontinent epispadias.

PURPOSE: Urologists and gynecologists rarely encounter questions on cohabitation and pregnancy in female patients with bladder exstrophy or incontinent epispadias. MATERIALS AND METHODS: Until 1994, 41 female patients underwent surgery at our institution (vaginal cutback or vaginoplasty in 23, correction of the external genitalia in 25 and uterus fixation to correct or prevent prolapse in 13). RESULTS: Followup was available in 37 patients (mean 16.8 years, with followup of 18 years or longer in 19). Of the patients 94% are satisfied with the cosmetic results. All adults engage in sexual intercourse, 4 delivered 6 children by cesarean section and 5 presently desire children. CONCLUSIONS: Fertility in patients with bladder exstrophy or incontinent epispadias is normal, and pregnancy is possible. Patients and parents should be well informed. Besides urinary tract reconstruction, correction of the external and internal genitalia should be discussed in detail.

Adolescent↗
PubMed1990

Inguinal pathology and its association with classical bladder exstrophy.

One hundred thirty-four cases of classical bladder exstrophy, managed at our institution, were reviewed. Fifty-six percent of the boys and 15% of the girls developed inguinal hernias over an average follow-up time-span of 10 years. Thirty-one percent of the patients with hernias underwent repair at the time of initial bladder closure. Forty-six percent of the patients who developed a hernia were diagnosed during the first year following their initial procedure. More than 50% of the individuals in the latter category presented with an incarcerated hernia, and required emergent management. Boys managed by staged reconstruction had a statistically significant risk of developing an inguinal hernia (P less than .001) compared with boys undergoing primary cystectomy and diversion. We believe the increased incidence of herniation with this congenital anomaly is secondary to a lack of obliquity of the inguinal canal, due to pubic diastasis along with an increased elevation of intraabdominal pressure following initial closure of the abdominal wall and bladder plate. To decrease the attendant morbidity of incarcerated hernias in this population, we stress the need for careful physical examination of the inguinal region and spermatic cord prior to surgery, along with repair of the patent processus vaginalis at the time of initial repair.

Bladder Exstrophy↗
PubMed2006

Long-term followup of patients after redo bladder neck reconstruction for bladder exstrophy complex.

PURPOSE: The aim of this study was to determine whether redo bladder neck reconstruction is effective in achieving continence after a failed bladder neck reconstruction procedure. MATERIALS AND METHODS: We retrospectively reviewed the hospital records of patients with bladder exstrophy who had undergone redo bladder neck reconstruction. There were 30 patients in the study, including 20 boys and 10 girls. Mean patient age at redo bladder neck reconstruction was 9.3 years (range 3.2 to 15.5). The patients were divided into 3 groups on the basis of the preoperative pattern of incontinence--incomplete wetters, complete wetters and those on continuous suprapubic drainage. Of the patients 15 already had undergone bladder augmentation, 12 had undergone a Mitrofanoff procedure and 12 had been treated with bulking agents injected in the bladder neck in an attempt to achieve continence. Four patients had undergone more than 1 bladder neck procedure. The patients were investigated with a combination of noninvasive urodynamics, cystoscopy, cystogram and ultrasound. All patients underwent Mitchell's modification of Young-Dees-Leadbetter bladder neck reconstruction. Additional procedures performed included augmentation cystoplasty and Mitrofanoff formation. RESULTS: Mean followup was 6.9 years (range 1.2 to 15.5). Postoperatively 28 patients were using clean intermittent catheterization to empty the bladder (5 per urethra, 23 via Mitrofanoff). Two patients remained on continuous suprapubic catheter drainage. A total of 18 patients (60%) were dry postoperatively (80% of girls and 50% of boys). Among dry patients only 3 were performing clean intermittent catheterization per urethra and 15 via a Mitrofanoff channel. No patient was able to void per urethra without the need for clean intermittent catheterization. The 2 patients on continuous suprapubic catheter drainage continued to remain so. At night only 50% of the patients were dry (5 on free drainage, 4 on clean intermittent catheterization, 6 not on any drainage). Those patients who did not respond satisfactorily to redo bladder neck reconstruction underwent subsequent additional procedures, which included injection of bulking agents (3 patients), insertion of an artificial urinary sphincter (1), Mitrofanoff formation (2) and bladder augmentation plus Mitrofanoff channel (1). Postoperative complications included difficulty with clean intermittent catheterization (8 patients), perivesical leak (1), recurrent epididymo-orchitis (1), upper urinary tract dilatation (2) and incisional hernia (1). Bladder neck closure was being considered in 5 patients. CONCLUSIONS: In our experience redo bladder neck reconstruction cannot achieve continence with volitional voiding per urethra. Although redo bladder neck reconstruction can render a significant number of patients dry, it is only effective if performed in conjunction with augmentation. Failure of the initial bladder neck reconstruction may be a reflection of a bladder that is of inadequate capacity and/or compliance. Therefore, bladder augmentation should be considered in all patients requiring redo bladder neck reconstruction. Bladder neck closure may be a better alternative to redo bladder neck reconstruction.

Adolescent↗
PubMedDate not supplied

[The choice of the method for the surgical treatment of bladder exstrophy].

129 children with exstrophy of the bladder underwent primary surgical reconstruction according to G. A. Bairov. Morphofunctional findings in these children gave grounds for determination of three degrees of the bladder congenital defects. 39 patients had defect of the 1st degree, 53 of the 2nd and 37 of the 3d degree. The results of plastic reconstruction of the bladder with local tissue support the validity of such procedure only for patients with the congenital defect degree I. For degree II the benefit is relative. In degree III plastic surgery in contraindicated.

Abnormalities, Multiple↗
PubMed1991

[Genital prognosis of boys with bladder exstrophy or epispadias with incontinence. Apropos of 14 cases].

Bladder exstrophy and epispadias with incontinence are associated with urinary tract and genital anomalies. The genital and sexual aspects were studied in 14 adolescent or adult males (12 exstrophies and 2 epispadias). The appearance of the penis was satisfactory in fifty percent of cases. Erections were always preserved but normal ejaculations were present in only one half of cases. Fertility potential was reduced. The improvement of surgical technique, and especially penile lengthening, has greatly improved sexual intercourse for these patients.

Adolescent↗
PubMedDate not supplied

[Carcinoma of the large intestine following ureterosigmoidostomy in a patient with bladder exstrophy].

The paper reports a case of intestinal occlusion due to adenocarcinoma of the large intestine as a sequel to ureterosigmoidostomy for exstrophy of the bladder which was subsequently converted to ureteroileocutaneostomy. Inadequate follow-up did not allow an early diagnosis to be made. The authors underline the need for annual colonoscopy in all patients suffering from or with previous ureterosigmoidostomy given the high incidence of malignant transformation of the colon.

Adenocarcinoma↗
PubMed1988

Long-term followup of 103 patients with bladder exstrophy.

We reviewed 103 patients with exstrophy of the bladder. Followup was more than 15 years in 51 patients. Initial management consisted of primary bladder closure in 32 patients and urinary diversion in 71. Urinary continence, renal function, urinary tract infections and development of malignant lesions were evaluated. Factors leading to success or failure were analyzed. Although the highest continence rate (83 per cent) was achieved in 40 patients with ureterosigmoidostomy, renal functional deterioration was highest in this group, with 70 per cent of the evaluable renal units being abnormal. Furthermore, 10 per cent of this group died of renal failure and 23 per cent lost 1 kidney each. In 26 patients with an ileal conduit 69 per cent of the renal units evaluated were abnormal. Only 1 patient died of renal failure but 27 per cent lost 1 kidney each. Of 32 patients with primary bladder closure 31 had preservation of renal function. Twelve of 18 patients (67 per cent) in whom vesical neck reconstruction had been completed had total urinary continence and 3 (17 per cent) had partial continence. The incidence of significant urinary tract infections was highest in the ureterosigmoidostomy group (63 per cent) and next to the highest in the ileal conduit group (48 per cent). Malignant lesions developed in 8 patients (8 per cent).

Bladder Exstrophy↗
PubMed1983

Wound dehiscence in bladder exstrophy: an examination of the etiologies and factors for initial failure and subsequent success.

During the last 8 years 20 patients were seen for secondary or repeat closure for exstrophy of the bladder following dehiscence after initial closure. Various factors that may have contributed to the initial problems have been investigated. Wound infection (42 per cent) and bladder prolapse (46 per cent) were the major etiologies for initial failures. In 6 patients (30 per cent) colon conduits were required for bladder augmentation or colocolostomy, which emphasized the importance of achieving a good result at initial closure. Our successful protocol (only 5 per cent reclosure rate for 60 closures) is outlined.

Anti-Bacterial Agents↗
PubMed1993

Prostate size and configuration in adults with bladder exstrophy.

A total of 13 men born with classical bladder exstrophy underwent magnetic resonance imaging examination of the pelvis to evaluate the size and configuration of the prostate and pelvic organs. Mean patient age was 25.2 years (range 19 to 38). Of the patients 4 are voiding per urethram and 9 have undergone urinary division. Measurements included prostate volume, weight and maximum axial cross sectional area; pubic diastasis, and seminal vesicle size. The mean prostatic cross sectional area was 10.1 +/- 3.4 cm.2. The mean estimated prostatic volume and weight were 20.7 +/- 8.2 cc and 21.7 +/- 8.6 gm., respectively. The mean seminal vesicle length and width were 2.1 +/- 0.99 and 1.1 +/- 0.38 cm., respectively. The volume, weight and maximum cross sectional area of the prostate appear normal compared to published norms. In none of the patients did the prostate extend circumferentially around the urethra and the urethra was anterior to the prostate in all patients. Also, the puborectalis muscle group was widely separated and only provided lateral support of the prostate in patients who were continent and who had undergone prior posterior iliac osteotomy. Thus, the attainment of continence in this complex group of patients is multifactorial and prostate growth as evaluated by magnetic resonance imaging may not influence continence in these patients.

Adult↗
PubMed1979

[Surgical treatment of bladder exstrophy by functional reconstruction by stages (author's transl)].

A brief summary is given of the clinical statistical findings in 37 patients with exstrophy of the bladder, most of the whom have been reported in a previous paper. Four cases are presented for whom the method of operating by stages was applied. They have now been followed up for 17, 10, 8 and 6 years respectively and a very satisfactory functional result was obtained. The experience gained from the use of such a method clearly indicates that treatment should be carried out, whenever possible, by a one-stage operation, or by two stages within a short period of time, and at an early age. Two cases operated upon by this latter method of total primary functional reconstruction, with very encouraging morphological results, are reported.

Age Factors↗
PubMed2003

Pelvic floor magnetic resonance imaging after neonatal single stage reconstruction in male patients with classic bladder exstrophy.

PURPOSE: We evaluate a magnetic resonance imaging (MRI) protocol used to study the pelvic floor anatomy in male patients following neonatal single stage complete bladder exstrophy and epispadias repair with osteotomies. MATERIALS AND METHODS: From 1996 to 2002, 9 males underwent surgical correction of bladder exstrophy and epispadias with osteotomies within 1 to 12 days of birth. Pelvic floor MRI was conducted comparing this group to 5 aged matched male patients with no pelvic anatomical abnormality who underwent MRI for other illness. We compared various measurement of pelvic musculature by unpaired Wilcoxon test. RESULTS: Median followup was 3 years (range 0.5 to 5.3). All surgical procedures succeeded in closure of abdominal wall and genitalia defects. MRI data showed that in the exstrophy group symphyseal distance was significantly wider than that in controls (median 3.8 vs 1.1 cm). In addition, the levator ani fibers diverted more laterally (42 vs 22 degrees), the pelvic floor in coronal view was more flat (103 vs 80 degrees) and the anus was more anteriorly displaced (2.8 vs 4.4 cm). We also documented shorter anterior corporeal bodies in the exstrophy group (1 vs 2 cm). No statistical difference between the 2 groups was found in the dimensions of the levator and obturator muscles, sagittal angle of the pelvic floor, and the dimensions and angle of the posterior corporal bodies. Two patients achieved some degree of continence around the age of 4 years. They had the shortest symphyseal distance and sharpest angle of levator ani fiber divergence, and the bladder neck was more deeply located in the pelvic. CONCLUSIONS: We applied novel MRI parameters to the pelvic floor anatomy providing a new quantifiable approach. Our protocol is feasible and reproducible, allowing for future comparison of the impact of different surgical modalities, and correlation between anatomical findings and continence.

Anthropometry↗
PubMed2004

3-dimensional magnetic resonance imaging modeling of the pelvic floor musculature in classic bladder exstrophy before pelvic osteotomy.

PURPOSE: We provide a 3-dimensional (3D) model of the pelvic floor musculature in patients with classic bladder exstrophy using magnetic resonance imaging (MRI). MATERIALS AND METHODS: Five male infants 1 day to 12 months old underwent MRI of the pelvis, which was compared to pelvic MRI of 1 male infant without pelvic floor abnormalities. Of the patients 3 were studied before primary closure and 2 before reclosure. None of the patients had a prior pelvic osteotomy. While the entire pelvic floor was imaged, special attention was paid to the contours of the levator ani muscle group which were drawn on T1-weighted axial and coronal images. The overlap of contours in these 2 planes was used to construct a 3D model of this muscle group. The diastasis of the pubic symphysis was also measured for all patients on a plain pelvic radiograph. RESULTS: The levator ani muscle group conformed to an elliptical dome shape in the control. For the patients the 3D shape was somewhat irregular with an apparent kink in the ellipse. The elliptical shape of the group was described by a shape factor, s, which equals the ratio of the maximum height-to-the length of the base of the ellipse. The shape factor was equal to 0.176 in the control compared to a mean of 0.448 for the patients. There was no relationship between diastasis of the pubic symphysis and the extent of disproportionate curvature of the levator group. CONCLUSIONS: To our knowledge this is the first qualitative description of the pelvic floor anatomy in bladder exstrophy using MRI. Our model gives further insights into the true pelvic floor anatomy in exstrophy cases and is the first to suggest that abnormalities in the pelvic floor may not correlate with abnormalities of the bony pelvis.

Bladder Exstrophy↗
PubMed2004

[Descriptive epidemiology of bladder exstrophy in an area of Madrid].

OBJECTIVE: To describe the procedures and complications of bladder exstrophy closure, epispadias repair and bladder neck surgery to achieve urinary control. METHODS: Retrospective chart review of 11 patients with the exstrophy-epispadias complex. RESULTS: Early bladder closure is performed after birth with or without concomitant pelvic osteotomy. The preferred procedures are: the Cantwell-Ransley technique for epispadias repair, the Young-Dees-Leadbetter procedure for bladder neck reconstruction and ileocystoplasty with Mitrofanoff for bladder augmentation. CONCLUSIONS: Augmentation and continent diversion procedures can increase the functional capacity of the exstrophic bladder, and allow the vast majority of patients to achieve continence and preserve renal function. Bladder lithiasis is the most significant complication in these patients.

Bladder Exstrophy↗
PubMedDate not supplied

Rotational profile of lower extremities in bladder exstrophy patients with unapproximated pelvis: a clinical and radiologic study in children older than 7 years.

Fourteen patients (nine boys, five girls) with bladder exstrophy were analyzed radiologically and clinically. All were older than 7 years and had a pubic diastasis >2 cm. Anteroposterior and lateral center-edge angles were measured by direct radiography. Acetabular version, femoral anteversion, tibial torsion angles, and patellofemoral congruency angle were measured by computed tomography imaging. All were active with regard to their daily life and sports activities. The average foot-progression angle was +8 degrees . Spherical congruency was present in all hips, and none showed dysplasia. The average angle of acetabular version was apparently less than normal, but femoral anteversion angles were found to be increased. Increased external tibial torsion was observed in all patients. Twelve (71%) of 14 patients had positive congruence angles, the average being +6.1 degrees . Two patients had subjective complaints of patellofemoral instability. Increased femoral anteversion and external tibial torsion may lead to patellofemoral instability, and the bladder exstrophy patients should be followed up regarding this problem as well.

Adolescent↗
PubMed1993

Techniques to create continence in the failed bladder exstrophy closure patient.

We reviewed retrospectively 315 patients with bladder exstrophy treated at our hospital between July 1976 and April 1992 to assess the outcome of those who failed primary closure of the bladder. Of the patients 47 required reclosure of the bladder, including 28 who have undergone a procedure to restore urinary continence. Methods used to achieve dryness included bladder neck reconstruction in 18 patients, bladder neck reconstruction along with augmentation in 4, augmentation alone in 4, repeat bladder neck reconstruction in 1, and reclosure with creation of a continent stoma and augmentation in 1. Nine of 18 patients who underwent primary bladder neck reconstruction are dry on intermittent catheterization, while 8 of the remaining 9 are dry and voiding without catheterization. Four patients who underwent primary bladder neck reconstruction and augmentation, and 4 who underwent augmentation after bladder neck reconstruction are dry on intermittent catheterization. The patient who underwent reclosure, bladder augmentation and creation of a continent abdominal stoma is dry on intermittent catheterization. Virtually all patients who failed the initial closure and later bladder neck reconstruction for continence require augmentation and intermittent catheterization to remain dry. Of 28 patients who underwent salvage procedures only 1 had upper tract changes. With attention to detail and the use of a variety of reconstructive techniques children who have failed exstrophy closure can achieve continence and have stable renal function.

Bladder Exstrophy↗
PubMed2001

Long-term outcome analysis of low pressure rectal reservoirs in 33 children with bladder exstrophy.

PURPOSE: The augmented valved rectum and double folded rectosigmoid bladder represent 2 modifications of ureterosigmoidostomy. Both procedures improve continence by lowering the reservoir pressure. We present the outcome of these techniques on the upper urinary tract, continence, metabolic profile, linear growth and bone density. MATERIALS AND METHODS: Between March 1987 and May 1997, 22 boys and 11 girls with bladder exstrophy underwent urinary diversion to a low pressure rectal reservoir. Patient age at surgery ranged from 2 to 13 years (mean 5.4). The augmented valved rectum technique was used in 18 cases and the double folded rectosigmoid bladder method was used in 15. Serial followup ultrasounds were obtained and voiding proctography was performed 1 year postoperatively in all patients. Supine height was measured at last followup in all cases. Serum electrolytes, arterial blood gases, the results of which were plotted on an acid base nomogram, and bone density using dual energy x-ray absorptiometry were measured in all patients. Prophylactic alkalization was administered to all patients. RESULTS: Mean followup is 66 months (range 24 to 148). All patients are continent during the daytime with an emptying frequency of 3 to 5 times, and all are continent at night. No patient experienced pyelonephritis or clinical acidosis. The upper urinary tract was either improved or stabilized in 64 of 66 renal units. No patient had reflux to the upper urinary tract. All patients had normal serum creatinine, sodium, potassium, calcium and phosphorus. Hyperchloremia was noted in 19 of the 33 patients (57%), and arterial blood gases showed subclinical metabolic acidosis in 18 (55%). Supine height measurements at last followup revealed that 19 of the 33 patients (57%) had decreased linear growth (below 3rd percentile). All patients had significant reduction in bone density, and mean for age corrected bone density was 70% (standard deviation 10.9%). Both groups (augmented valved rectum and double folded rectosigmoid bladder) were comparable in regard to age, sex and followup duration. There was no statistically significant difference between the groups in any parameter measured. CONCLUSIONS: The augmented valved rectum and double folded rectosigmoid bladder provide preservation of the upper urinary tract with excellent continence rates. However, prophylactic alkalization and functional isolation of the reservoir do not prevent the long-term metabolic consequences. Subclinical metabolic acidosis and decreased linear growth are to be anticipated in more than 50% of patients. Moreover, significant bone demineralization is to be expected in all of these patients.

Adolescent↗