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PubMed2002

Hypospadias and bladder exstrophy.

Reconstructive surgery for patients with hypospadias, bladder exstrophy and cloacal exstrophy is challenging. Over the last few years, more and more data have become available concerning incidence and epidemiology, as well as short- and long-term results of surgical techniques. The tubularized incised plate urethroplasty for hypospadias repair continues to gain in popularity. However, the results of long-term studies of larger series supporting this enthusiasm are still missing. Buccal mucosa is more frequently used in complex hypospadias cases with good short- and long-term results. The one-stage approach of exstrophy repair seems to be promising. However, failures, which require very complex reconstructions, are also reported. Urodynamic studies demonstrated severe abnormalities of bladder function even in continent patients. Therefore, one must question the normality of voiding patterns, and the price to be paid in achieving continence for patients with exstrophy. Achievement of continence for urine and stool is possible for patients with cloacal exstrophy. The colon should be preserved and should not be used for bladder or vaginal reconstruction. The incidence of neoplasia in patients with bladder exstrophy seems to be higher than expected. However, only one cohort study is presently available.

Bladder Exstrophy↗
PubMed2005

Fixation of bilateral pelvic osteotomies with external fixator in exstrophy bladder complex.

OBJECTIVE: To present the early results of pelvic osteotomies performed for repair of exstrophy bladder. METHODS: Five cases of exstrophy bladder were treated with closure following bilateral iliac osteotomies. Three patients underwent closure of pubic symphysis diastasis by use of external fixator, one by screws and cerclage wires, and one by use of K-wires and suture. The patients were followed up by the pediatric urologist and orthopedic surgeon. RESULTS: All patients achieved a closure of diastasis and a tension free repair after the index surgery. The average follow-up was 3.6 years with range of 4 months to 6 years. All osteotomies healed within two months and had closure of the diastasis, except one which had a partial failure with loss of 50% correction. No patient had any wound dehiscence or breakdown of the bladder repair. Preoperative mean diastasis of symphysis pubis was 6 cm (range; 4.5 cm to 7 cm) and post operative mean diastasis was 3.5 cm with the range of 2.5 cm to 4 cm at 12 months follow up. All patients achieved urinary continence post operatively and were passing urine per-urethra with satisfactory urinary control as followed-up with the pediatric urologists. CONCLUSION: Bilateral iliac osteotomies and use of external fixator in our series was found to be helpful in achieving a tension free closure and preventing dehiscence of the repair.

Adult↗
PubMed1989

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 60 cc). 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 54.5 cc. 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.

Bladder Exstrophy↗
PubMed1996

A combined vertical and horizontal pelvic osteotomy approach for primary and secondary repair of bladder exstrophy.

PURPOSE: We describe a new combined horizontal and vertical pelvic osteotomy procedure for bladder exstrophy. MATERIALS AND METHODS: A total of 36 patients with the bladder exstrophy complex underwent this procedure during a 3-year period (8 primary and 18 secondary bladder closures, and 6 at bladder neck reconstruction). RESULTS: There were no instances of dehiscence and only a minor bladder prolapse in 1 patient with cloacal exstrophy. Two patients had a transient femoral nerve palsy and there was 1 superficial pin infection. Urological complications included symptomatic urinary tract infections in 5 patients, acute epididymitis in 1 and bladder calculi in 2. CONCLUSIONS: This new osteotomy procedure is of great benefit in initial or repeat closure of bladder exstrophy and may help in eventually achieving continence.

Bladder Exstrophy↗
PubMed1994

Inguinal hernias associated with bladder exstrophy.

OBJECTIVE: To determine the incidence and complications of inguinal hernias in patients with bladder exstrophy. PATIENTS AND METHODS: A retrospective review of inguinal hernias and their management in 70 consecutive patients (50 boys and 20 girls) with bladder exstrophy managed by staged reconstruction. Follow-up data were available for 69 patients. RESULTS: During a mean follow-up period of 5.9 years (range 0.3-14), 42 (86%) boys and three (15%) girls developed inguinal hernias. These were bilateral in 35 (78%) cases. All but three of these patients presented during infancy. In girls, no instance of incarceration or recurrence was noted. In contrast, 14 (29%) boys developed an incarcerated hernia and seven (17%) developed recurrent inguinal hernias. All recurrences occurred in boys under 2 years of age, were associated with an indirect sac and were unrelated to the seniority of the surgeon performing the initial herniotomy. No recurrences occurred in eight boys undergoing inguinal herniotomy at the time of bladder closure. CONCLUSIONS: Boys with classical bladder exstrophy managed by staged reconstruction have a much higher incidence of inguinal hernias than previously recognized. Most are bilateral, present in early infancy and are prone to incarceration. Bilateral groin exploration and meticulous inguinal herniotomy at the time of bladder closure may reduce morbidity.

Bladder Exstrophy↗
PubMed2005

Early outcome following complete primary repair of bladder exstrophy in the newborn.

PURPOSE: Complete primary repair of bladder exstrophy (CPRE) represents a paradigm shift from the staged approach for surgical management. We present early clinical outcomes in our patients following CPRE. MATERIALS AND METHODS: From 1996 to 2004 all newborns with bladder exstrophy were treated with CPRE within 48 hours of birth. We reviewed parameters including transfusions (packed red blood cells), urethral meatal position, complications, findings on renal ultrasound and renal scan, and post-CPRE procedures and infections. RESULTS: A total of 16 boys and 7 girls were treated with CPRE. Followup ranged from 8 to 96 months. Twelve boys (75%) and 2 girls (29%) received packed red blood cells at CPRE (p = 0.066). The tubularized urethral plate could not be brought to the penile tip, resulting in hypospadias in 9 of 11 boys (82%) with the running suture technique and only 1 of 5 boys (20%) with the interrupted technique (p = 0.036). Of the 23 patients 6 had a total of 8 complications after CPRE. Vesicoureteral reflux was present in 17 of 23 patients. After CPRE a total of 93 endoscopic/surgical procedures (median 4, range 0 to 16 per patient) were performed. Five patients had 1 to 4 episodes of pyelonephritis, 16 of 23 had 1 or more episodes of asymptomatic bacteriuria and 5 had cortical defects on renal scan. CONCLUSIONS: We recommend that urethral closure during CPRE be performed with interrupted suture technique to prevent hypospadias. An aggressive approach should be taken toward reflux in the setting of urinary infection. Consideration should be given for repair of all aspects of the defect during CPRE, including bilateral ureteral reimplantation.

Bladder Exstrophy↗
PubMed2001

Evaluation of the bony pelvis in classic bladder exstrophy by using 3D-CT: further insights.

OBJECTIVES: To provide a complete look at the bony pelvis in children with classic bladder exstrophy: dimensions, orientation, and relationships. METHODS: Three-dimensional computed tomography was used in 6 boys and 1 girl, 5 of whom underwent primary closure and 2 who underwent reclosure at 4 and 8 months. These exstrophy pelves (intrapelvic angles and osseous dimensions) were compared with 26 age and sex-matched controls. RESULTS: The iliac wing angle was 11.4 degrees larger in the classic bladder exstrophy cases. The sacroiliac joint angle was 9.9 degrees more externally rotated in the exstrophy cases. The pelvis was rotated 14.7 degrees in the superoinferior plane in the exstrophy cases. The mean pubic diastasis was 4.2 cm (0.6 cm in controls). The inter-triradiate distance in the patients with classic bladder exstrophy averaged 6.0 cm (4.2 cm in controls). CONCLUSIONS: These new findings provide a better understanding of the bony pelvis, especially its posterior portion, in patients with classic bladder exstrophy. The results of this study revealed the orientation of the sacroiliac joints to be more externally oriented than previously thought and the pelvis to be rotated inferiorly, a previously unknown observation. Both of these factors will be important in the planning of newer osteotomies and pelvic reconstruction.

Adult↗
PubMed2001

The newborn exstrophy bladder inadequate for primary closure: evaluation, management and outcome.

PURPOSE: The surgical approach to the small newborn exstrophy bladder inadequate for primary closure remains undetermined. Various methods for long-term management have been implemented. We evaluated our experience with late primary closure of the small exstrophied bladder template. MATERIALS AND METHODS: Our institutional database of patients treated and followed for the exstrophy-epispadias complex was reviewed. Of these patients 19 had a bladder template that was too small to close in the newborn period. The treatment and outcome of these 19 patients were reviewed. RESULTS: Of the 19 children who had delayed closure due to a small bladder template 14 were males and 5 were females. Followup from birth ranged from 2 to 36 years (mean 18 years). Primary closure was performed at a mean patient age of 13 months (range 6 months to 2 years). Pelvic osteotomy was performed in 16 patients. Of the 19 patients 9 achieved continence after gaining a bladder capacity sufficient for bladder neck reconstruction, 4 required enterocystoplasty to augment bladder volume and perform clean intermittent catheterization (2 per stoma and 2 per urethra), 1 required a colon conduit for an extremely small bladder, and 1 underwent cystectomy and ureterosigmoidostomy for rhabdomyosarcoma. Four patients are currently incontinent, including 3 who are awaiting bladder neck reconstruction and 1 who has frequent nighttime incontinence that is medically managed. CONCLUSIONS: Delayed primary closure of the small bladder exstrophy template can allow the native bladder tissue adequate time to grow to a size feasible for successful closure. Epispadias repair can usually be performed at the same time and is facilitated by prior testosterone administration. Bladder neck reconstructive techniques have achieved continence without the need for augmentation or bladder replacement in 47% of the patients in our series. For patients who do not achieve adequate capacity for bladder neck reconstruction, preservation of the native bladder template facilitates future augmentation and ureteral reimplantation, thus requiring use of less bowel in the growing child.

Adolescent↗
PubMed1988

Embryology of bladder exstrophy.

A hypothesis in respect to the teratogenesis of bladder exstrophy and its variants is offered. The central feature of this hypothesis is the abnormal persistence of the caudal position of the insertion of the body stalk on the embryo. As a consequence of this, the normal advance and interposition of mesenchymal tissue to the midline becomes impossible. The cloaca cannot be translocated backwards into the body cavity, and the cranial end of the cloacal membrane remains in contact with the inferior aspect of the low-set body stalk. This, in contrast to the previously proposed abnormal rostral extension of the cloacal membrane, causes a wedge-effect resulting in the lateralization of the abdominal wall structures and also in the prevention of the midline fusion of the genital hillocks (labioscrotal or genital folds). A cloacal membrane normally is an unstable structure lacking mesoderm, and it retains these characteristics in the superficial and infraumbilical position to be described. It has a strong tendency to disintegrate. It may rupture at variable times and to a variable extent. The consequence of such an embryonic event is either a typical bladder exstrophy or one of the variants of the exstrophy malformation. Three different variants are presented that the proposed embryologic hypothesis can readily explain.

Animals↗
PubMed2003

[Psychological, sexual and social aspects of bladder exstrophy in adulthood. Analysis of 3 cases].

Rare congenital malformation, bladder exstrophy is usually treated soon after birth. Based on three cases report of bladder exstrophy in adulthood (two men and one women aged from 21 to 25 years), the authors emphasize the importance of psychological, sexual, and social disorders caused by this affection treated at this age. Urinary problems were excluded of this study. The authors stress the necessity of an early management of this malformation, if possible at birth in order to minimize these consequences.

Adult↗
PubMed1996

Hip configuration and function in bladder exstrophy treated without pelvic osteotomy.

Nine children with congenital bladder exstrophy treated without pelvic osteotomy were analyzed clinically and radiologically at a mean age of 13 years (range 9-16 years). The acetabular and femoral version angles were measured by computed tomography (CT) imaging. Two of the children had a slight waddling gait, but none of them had any pain and they could participate in sports without problems. They had a normal range of hip movements and a normal foot progression angle. The acetabulum was retroverted by an average of 5 degrees, but was balanced by an increased anteversion of the femur which on the average was 10 degrees-20 degrees higher than normal. The femoral head in all hips was spherical, and no hip showed dysplasia. In bladder exstrophy, retroversion of the acetabulum was balanced by an increased anteversion of the femur, resulting in a normal range of hip movements and a normal gait in later childhood.

Adolescent↗
PubMed2001

Determinants of continence in the bladder exstrophy population: predictors of success?

OBJECTIVES: To delineate factors that may predict eventual urinary continence after bladder neck reconstruction (BNR) in the bladder exstrophy population. METHODS: The records of 65 patients who underwent all phases of bladder exstrophy reconstruction at our institution between 1975 and 1997 with greater than 1-year follow-up were reviewed and data analyzed. RESULTS: Fifty patients (77%) are continent day and night and voiding per urethra without need for augmentation or intermittent catheterization. Nine (14%) patients have social continence, dry for more than 3 hours during the day. Two patients required continent diversion for continence after failed BNR. Four patients are completely incontinent. The mean age of BNR was 4 years with a mean and median capacity of 93 and 85 cc (range 45 to 175). Analysis of bladder capacity measurements prior to BNR revealed that patients with a bladder capacity greater than 85 cc median capacity at the time of BNR had better outcomes. No correlation was found between the age of BNR and obtaining eventual continence. The mean time to daytime continence was 14 months (range 4 to 21) and the mean time to nighttime continence was 22 months (range 11 to 33). CONCLUSIONS: Determinants of continence in the bladder exstrophy population are multifactorial. In our experience, 77% of patients are completely dry, day and night, and 91% can achieve social continence, being dry for at least 3 hours. However, with careful evaluation of bladder capacity and bladder growth, urinary continence may be improved in this population with better patient selection.

Bladder Exstrophy↗
PubMed2001

Cystometric evaluation of reconstructed classical bladder exstrophy.

OBJECTIVE: To evaluate the association of urodynamic variables with continence and upper tract status after reconstruction in patients with classical bladder exstrophy. PATIENTS AND METHODS: Thirty-one patients with bladder exstrophy were assessed 1 year after a modified bladder neck reconstruction. The evaluation included a detailed history, radioisotope renography, voiding cysto-urethrography, ultrasonography and artificial slow-filling cystometry. RESULTS: Fifteen of the 31 patients were satisfactorily continent; their maximum cystometric capacity was higher than that of the incontinent patients. The compliance, assessed as the maximum bladder capacity at a detrusor pressure of < 20 cmH2O, was significantly higher in the continent patients. There was a 45% incidence of unstable contractions in the 31 patients. Persistent sphincteric activity was detected on electromyography in 10 patients during voiding. Twenty-one patients could initiate a detrusor contraction during voiding. The residual volume was significant in nine of the 21 patients who attempted to void. Patients with a high end-fill pressure (> 40 cmH2O) had a significantly higher incidence of unobstructive hydronephrosis than had patients who had an end-fill pressure of < 40 cmH2O. CONCLUSIONS: Bladder abnormalities are common after reconstruction of bladder exstrophy, with poor compliance, small capacity and unstable contractions. These factors hinder any increase in capacity and cause persistent incontinence. Hypocompliance and high end-fill pressure can lead to upper tract damage even in continent patients. Detailed urodynamic evaluation is vital to assess the results and to plan subsequent treatment.

Bladder Exstrophy↗
PubMed1991

[Invasive adenocarcinoma with epidermoid carcinoma on the site of bladder exstrophy. Histochemical and immunocytochemical study].

To date, one hundred and ten cases of malignant neoplasms arising from untreated bladder exstrophy have been reported. We describe another case of uncorrected bladder exstrophy with invasive adenocarcinoma and in situ squamous carcinoma discovered in a 51 year old woman. Postoperative radiotherapy was performed after cystectomy with nephroureterectomy and hysterectomy. The neoplastic samples were investigated by mucin histochemistry and immunocytochemistry. The epithelium lining the tumor villi and the surrounding mucosae was colonic with mucin histochemistry and reacted to anti KL1, EMA and ACE antibodies. These features were those of colonic and vesical adenocarcinomas. Although early vesical reconstruction is the best oncologic prevention, patients with bladder exstrophy run the risk of sigmoid adenocarcinoma after cystectomy and urinary diversion including ureterosigmoidostomy.

Adenocarcinoma↗
PubMed2004

Loss of the penile glans and/or corpora following primary repair of bladder exstrophy using the complete penile disassembly technique.

PURPOSE: The technique of penile disassembly is increasingly being used for the surgical repair of classical bladder exstrophy. We describe the complications and discuss the possible etiologies of genital injuries we have seen following this operation. MATERIALS AND METHODS: A review of the records of patients evaluated for management of genital complications following complete repair of bladder exstrophy from 1996 to 2003 was performed. RESULTS: Nine patients were evaluated for genital injuries following complete repair of bladder exstrophy using the penile disassembly technique. Injuries included the loss of 1 hemiglans and penile urethra in 2 cases; loss of 1 hemiglans and distal corpora in 2; loss of bilateral glans, distal corpora and penile urethra in 2; loss of 1 hemiglans, 1 corporal body, urethral plate and penile shaft skin in 1; loss of 1 hemiglans, distal corporal body and a portion of the urethra in 1; and loss of 1 hemiglans in 1. The exact etiology of these complications is unknown, and possibilities include, a technical mishap, induction of venous congestion/arterial spasm or disruption of a congenitally abnormal blood supply. CONCLUSIONS: The finding that primary repair of bladder exstrophy using the penile disassembly technique is associated with the risk of partial or complete penile loss dampens our enthusiasm for this procedure. The association of genital injuries with penile disassembly has resulted in a modification of this surgical technique that would hopefully lessen its risk.

Bladder Exstrophy↗
PubMed1997

Rectus abdominis muscle flap (RAMF) technique for the management of bladder exstrophies: late clinical outcome and urodynamic findings.

OBJECTIVE: To investigate the efficacy of the rectus abdominis muscle flap (RAMF) technique for the closure and augmentation of small, non-compliant bladder exstrophies. PATIENTS AND METHODS: The RAMF technique was used in two girls and two boys (mean age at operation, 31.7 months, range 3-72) with bladder exstrophy. The clinical outcome and urodynamics were assessed during a follow-up of 29 months to 6 years (mean 49.2 months) and included imaging, cystoscopy, biochemical and microbiological studies. RESULTS: There were no urinary tract infections, metabolic problems or electrolyte disturbances and kidney function remained normal in all patients. Radiography confirmed intact function and anatomy of the urinary tract and cystoscopy showed complete coverage of the inner peritoneal layer of RAMF with uroepithelium. No stone formation or mucus production were detected. Currently, three patients void using clean intermittent catheterization through the native urethra and the fourth through an appendiceal Mitrofanoff valve. However, the bladder volume was insufficient at the late follow-up and three patients required bladder augmentation. CONCLUSIONS: The RAMF technique is a good alternative for closing bladder exstrophies and achieves an increase in bladder capacity; however, although there is a mild improvement in bladder capacity and compliance. RAMF should not be used as a bladder augmentation procedure. The technique is indicated in the closure of large bladder defects, bladder exstrophies with small, inelastic, non-compliant bladder remnants and failed primary closures.

Bladder Exstrophy↗
PubMedDate not supplied

The hip in adults with classic bladder exstrophy: a biomechanical analysis.

We studied the untreated pelvic deformity in 14 adult patients with bladder exstrophy to determine the relative hip-joint force and stress and their effects on the clinical status of the hip. Pelvic radiographs were used for biomechanical analysis to calculate joint force and joint stress (force/area) relative to partial body weight, which allowed comparison between patients and age-matched controls. IOWA hip ratings were used for clinical evaluation, and hips were scored radiographically for degenerative joint disease. The mean relative joint force and joint stress was significantly higher for exstrophy patients (p < 0.001). In addition, the mean distance from the body center to the center of the femoral head was significantly increased (p < 0.001) in bladder exstrophy patients. An increase in diastasis correlated with an approximate 30% increase in the distance from the center of the femoral head to the body midline. The mean distance from the greater trochanter to the femoral head center was significantly less (p < 0.02) and the center-edge angle was significantly decreased in exstrophy patients (p < 0.05). Two patients' hips showed significant subluxation, and one of these showed degenerative hip disease in association with poor IOWA and radiographic scores. The force and stress on the hip joint are increased in untreated adult bladder exstrophy patients. Further longitudinal study is indicated to validate these findings and to determine whether clinically important degenerative changes are occurring. This could affect treatment recommendations in childhood.

Adolescent↗