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PubMed2001

Complete repair of bladder exstrophy: preliminary experience with neonates and children with failed initial closure.

PURPOSE: The surgical repair of bladder exstrophy remains challenging for the pediatric urologist. We present our preliminary experience with complete primary repair of exstrophy in neonates and children with failed initial closure. MATERIALS AND METHODS: Between November 1998 and April 1999, 9 boys and 2 girls with bladder exstrophy underwent complete repair. This procedure was performed in the first 72 hours of life in 4 boys and at age 3 months in 1 girl. Complete repair with osteotomy was performed after failed initial closure in 5 boys and 1 girl at a mean age of 28 months (range 15 to 36). The bladder and urethra were closed in continuity and epispadias was repaired by total penile disassembly. All patients were kept in a spica cast for 3 weeks. Ureteral stents and suprapubic tube were removed 10 and 14 days, respectively, after surgery. Ultrasound was performed preoperatively and every 3 months postoperatively, voiding cystourethrography was done 6 to 12 months after surgery. RESULTS: Mean followup is 14 months (range 12 to 17). All repairs were successful including 1 case of penile scrotal duplication. Concomitant augmentation was done in 2 girls due to a small bladder plate. Complete closure resulted in hypospadias in 3 of the 9 boys. No patient had dehiscence or fistula. Serial followup ultrasound revealed no hydronephrosis and normal renal growth. Febrile urinary tract infection occurred in 1 case 2 months after surgery and was managed conservatively. Bladder capacity was 200 and 270 ml., at 6 months, respectively, in the 2 patients with an augmented bladder and both are dry on intermittent catheterization. The 4 patients in whom the closure was performed at birth are voiding with dry intervals with mean bladder capacity of 75 cc at 1 year (range 60 to 90). The 5 older children had a mean bladder capacity of 120 cc (range 70 to 150) at 6 months, of whom 2 are completely continent and 3 have 1 to 3 hours of dry intervals. CONCLUSIONS: Complete repair of bladder exstrophy is feasible in neonates and children after failed initial closure with minimal morbidity. There is no short-term evidence of worsening reflux or hydronephrosis. Longitudinal followup with adequate recording is required for continence evaluation. This technique may minimize the future need of bladder neck reconstruction and augmentation in patients with exstrophy.

Bladder Exstrophy↗
PubMed2000

Decreased linear growth associated with intestinal bladder augmentation in children with bladder exstrophy.

PURPOSE: We determine if enterocystoplasty results in delayed linear growth using a case controlled study to observe the effects of intestinal bladder augmentation on growth in patients with bladder exstrophy. MATERIALS AND METHODS: A total of 50 patients who had undergone bladder augmentation for incontinence due to classic bladder exstrophy were selected from our patient database and matched for gender, age and type of exstrophy with 50 patients who had nonaugmented bladder exstrophy. Patients were then contacted and asked to permit the pediatricians to release growth charts. Once consent was obtained the charts were requested from the pediatricians, and evaluable data, defined as at least 1 height before and after augmentation, were obtained for 17 of 50 (34%) augmented and 15 of 50 (30%) nonaugmented cases. RESULTS: Mean age at surgery was 7.7 years. Delayed growth as defined by a postoperative decrease in percentile height occurred in 14 of 17 (82%) augmented cases (mean loss 15.6 percentile points). Delayed growth after age 7.7 years occurred in 5 of 15 (33%) controls but average growth for the entire group was 6.7 percentile points (p = 0. 014). Mean followup was 5.7 years (median 4.9) for the augmented group and 7.3 years (median 8.2) for the control group. CONCLUSIONS: Intestinal bladder augmentation is associated with a nearly universal decrease in percentile height. Close long-term followup of these patients and analysis of subtle metabolic alterations may provide information to help minimize or prevent growth impediment in the future.

Adolescent↗
PubMed2004

Continence and classic bladder exstrophy treated with staged repair.

PURPOSE: Urinary continence rates after reconstruction of the urinary tract for classic bladder exstrophy are variable. We review our experience with procedures required to optimize continence. MATERIALS AND METHODS: We retrospectively reviewed the charts of patients with classic bladder exstrophy undergoing staged reconstruction between 1976 and 2001. Continence was reviewed and associated with the procedures performed. Patients were considered continent if dry for greater than 3 hours with no stress incontinence and dry at night. Partial continence required dryness for 1 to 3 hours, minor stress incontinence and occasional nighttime leakage. Incontinent patients were dry for less than 1 hour, had significant stress incontinence or were wet at night. Patients were followed for a median of 12.5 years. RESULTS: Continence was achieved in 43 of 48 patients (90%), partial continence in 4 (8%) and 1 (2%) remains incontinent. Continence occurred before bladder neck repair in 4 patients. Bladder neck repair alone (without augmentation) was performed in 38 patients at a median of 4.25 years, resulting in continence in 13 patients (34%). However, 6 of these 13 patients later required augmentation. Augmentation was performed in 33 patients, with 30 (91%) attaining continence after this procedure. Of those continent patients 10 void per urethra, and 8 void and use clean intermittent catheterization. Of the patients treated with bladder augmentation 93% required clean intermittent catheterization. CONCLUSIONS: Continence can be achieved in most patients with classic bladder exstrophy. However, augmentation was required in 30 of 43 patients (70%). Bladder neck reconstruction alone resulted in continence in only a third of the cases.

Bladder Exstrophy↗
PubMed1982

Pregnancy following repair of anal and vaginal atresia and bladder exstrophy.

Pregnancy following surgical correction of bladder exstrophy with anal and partial vaginal atresia is extremely rare. These defects, plus the absence of the pubic symphysis and anatomic deficiencies of the urogenital diaphragm, pose extraordinary obstetric and surgical problems. This report is based on the experience of a 21-year-old patient who had successful pregnancy following curative repair of these defects. Progressive uterine prolapse occurred during pregnancy to the extent that at the time of delivery the presenting part of the fetus was below the pelvic outlet. Cesarean section was performed at 36 weeks' gestation when the cervix was 4 cm dilated with a single footling breech presentation. Postpartum surgical management of the uterine and vaginal prolapse is described.

Adult↗
PubMed2002

Classic bladder exstrophy in a nonhuman primate: a comparative analysis.

OBJECTIVES: To describe the pelvic floor musculature and bony pelvic anatomy in a case of naturally occurring classic bladder exstrophy in a rhesus monkey, the first reported case in the animal population since 1832, and compare the results to exstrophy seen in human newborns. METHODS: A 7-day-old male rhesus monkey with classic bladder exstrophy was examined by a pediatric urologist and primate veterinarian before being killed. A multidetector row computed tomography study with three-dimensional reconstruction was obtained, and a comparison computed tomography study of a 17-day-old male human with exstrophy was also reconstructed three dimensionally. The bony pelvis and pelvic floor muscular anatomy of both subjects were then examined and compared. RESULTS: On gross examination, a similar appearance of classic bladder exstrophy in the rhesus monkey and human newborn were noted, including an open exposed bladder, associated penile epispadias, and widely separated pubic bones. The evaluation of the three-dimensional models showed a similar orientation of the bony pelvis in both the rhesus and the human newborn. The iliac wings were significantly rotated outward, and the sacroiliac joint was 10 degrees wider than that seen in normal children. The exstrophy pelvic floor in both the rhesus and the newborn was markedly flattened, with approximately 33% of the muscle located anterior to the rectum to support the pelvic structures (normal children have 50% of their levator ani anterior to the rectum). CONCLUSIONS: By using advancements in imaging modalities, this study illustrated that naturally occurring classic bladder exstrophy in the human newborn and rhesus monkey were identical in both external appearance and internal anatomy.

Animals↗
PubMed1996

One-stage reconstruction of bladder exstrophy.

From 1984 to 1990 a primary one-stage reconstruction of bladder exstrophy was performed at our hospital on 15 consecutive infants. Reconstruction included Cohen's ureteral reimplantation, bladder neck reconstruction according to Young-Dees, bladder closure, symphyseal bone approximation, abdominal wall closure, and repair of epispadias in females. Postoperatively, all patients were immobilized by overhead extension. Iliac osteotomy was done in 9 children, but was abandoned in recent years for infants less than one month old Follow-up examinations included an interview with the parents, clinical examinations, blood and urine analyses, uroflowmetry, and ultrasound scan of the abdomen. Nuclear renal scan, i.v. pyelography, and cystography were carried out as indicated. At the time of last evaluation the children were between 3 and 9 years of age. The cosmetic results were satisfactory in all cases. Eight of 15 children were dry day and night with micturition intervals more than 3 hours during day time (complete continence). Five children were dry during day time with micturition intervals up to 3 hours, but occasionally wet their beds (partial continence). Two children were incontinent. Eleven of 15 children had a bladder capacity of more than 100 ml. Renal function was normal in all patients. Two children showed a mild VUR, and no other abnormalities of the upper urinary tracts were found. Iliac osteotomy did not influence the functional or cosmetic results, but in two patients asymmetry of pelvic bones with consecutive lumbar scoliosis was found. Comparing these results with reports on staged reconstructive procedures, we conclude that by primary complete one-stage reconstruction a well acceptable success rate is achieved, and that by this method possibly some advantages are offered to patients with bladder exstrophy.

Bladder Exstrophy↗
PubMed1996

Continence after bladder-neck reconstruction in patients with bladder exstrophy and pubic diastasis.

OBJECTIVE: To review the results of bladder-neck reconstruction in patients with repaired bladder exstrophy and pubic diastasis. PATIENTS AND METHODS: Nine girls (mean age 7 years, range 4-17) and four boys (mean age 9 years, range 5-15) underwent a modified Young-Dees-Leadbetter bladder-neck reconstruction with augmentation cystoplasty (YDL-C). The patients were reviewed retrospectively (follow-up, 1-6 years) to assess continence, particularly in relation to the degree of pubic diastasis measured on an appropriate abdominal radiograph. RESULTS: Ten patients were continent; seven girls and one boy are managed by clean intermittent catheterization (CIC) and one girl and one boy void normally. One girl who would not allow CIC and one boy in whom CIC was not possible are incontinent and are scheduled for construction of a continent diversion. One incontinent boy who also would not allow CIC was lost to follow-up. Public diastasis had no bearing on the surgical results, the 10 continent patients having diastasis ranging from 4 to 9 cm (mean 5.5 cm) and the incontinent patients a diastasis of 3.4 and 6.5 cm (mean 4.5 cm). CONCLUSION: Young-Dees-Leadbetter bladder-neck reconstruction with augmentation cystoplasty is a satisfactory operation in patients with bladder exstrophy. We believe that the rate of continence reflected a competent tubularization with an adequate bladder capacity and assured bladder emptying. A closed pelvis with approximated public bones is not necessary to achieve this objective.

Adolescent↗
PubMed2004

Severe perineal pain after enterocystoplasty in bladder exstrophy.

OBJECTIVE: To describe a previously unreported complication (severe perineal pain) after bladder reconstruction and enterocystoplasty in patients with bladder exstrophy. PATIENTS AND METHODS: The notes were reviewed retrospectively for four patients (two boys and two girls) with classical bladder exstrophy who had severe penile or perineal pain after bladder reconstruction. They were all continent and using intermittent catheterization. A range of conservative management failed and all patients subsequently required excision of their native bladders between 1997 and 2000. RESULTS: All four patients had perineal or penile pain which began 4 months to 8 years after bladder augmentation. Investigations included plain abdominal X-ray, renal and bladder ultrasonography, computerized tomography of the pelvis, video-urodynamics and cysto-urethroscopy. When therapeutic interventions such as more frequent bladder washouts, analgesic and anticholinergic drugs, and cystolithotomy (two patients) were unsuccessful in alleviating the symptoms, all had their native bladder excised. Histological examination of the excised tissue showed neither normal urothelium nor enteric mucosa at the margins of the excision; two patients already had squamous metaplasia within what represented the bladder, and in the others squamous epithelium was present amongst the enteric mucosa. All four children were pain-free with a follow-up of 2-6 years. CONCLUSION: All four patients developed severe referred bladder pain that was probably secondary to the abnormal retained bladder remnants. Cystectomy cured the pain and may also have removed a potential site of future malignant tumour.

Bladder Exstrophy↗
PubMed1998

[Reconstruction of bladder exstrophy: retrospective study of 57 patients with evaluation of criteria in favor of the acquisition of continence].

In order to evaluate the results of staged surgical reconstruction in terms of urinary continence, spontaneous voiding and preservation of the upper urinary tract, we reviewed the charts of 42 boys and 15 girls treated at Hôpital Saint-Joseph for bladder exstrophy between 1965 and 1995. All patients underwent staged repair, associating bladder closure, bladder neck plasty, then genitoplasty, including bladder augmentation in 7 cases (12%). Criteria for good outcome in terms of continence are defined and factors influencing outcomes are reviewed. A total of 38 patients (67%) achieved good or acceptable urinary continence (22 or 39% and 16 or 28%, respectively). Outcome was poor in 19 patients, including 13 (23%) who underwent secondary urinary diversion. Bladder stones, which developed in 13 patients (23%), were the commonest long-term complication of bladder exstrophy reconstruction. Ten patients who underwent pelvic osteotomies ultimately had better continence and 9 are dry. Bladder neck reconstruction was performed at a later age overall (mean 10 years). Repeat bladder neck reconstruction was generally associated with poor results. The technique of bladder neck reconstruction did not appear to influence outcome. This retrospective study confirms that a carefully planned surgical reconstruction for bladder exstrophy can lead to satisfactory long-term urinary continence in most patients. Factors contributing to successful results include early bladder closure, pelvic osteotomy, adequate bladder neck reconstruction with bladder neck suspension in girls, and a motivated child and family. Alternatives to surgical reconstruction should be discussed. Ultimate predictors of outcome in bladder exstrophy repair are difficult to ascertain.

Adolescent↗
PubMed2004

Pelvic development in the rabbit embryo: implications in the organogenesis of bladder exstrophy.

Current theses of the development of bladder exstrophy and its variants rely on defective evolution of the urinary tract and cloacal membrane. They do not account satisfactorily for the clinical features reported in children affected by exstrophy, especially the pelvic bone anomaly. We herein describe the normal development of the pelvic ring in the rabbit embryo and its chronological relationship with the lower urinary tract organogenesis. Our results suggest that these events are intricated and allow us to propose a novel mechanism to explain exstrophies.

Animals↗
PubMed1996

Long-term results of ureterosigmoidostomy in children with bladder exstrophy.

PURPOSE: We evaluated long-term results of patients with bladder exstrophy who underwent ureterosigmoidostomy. MATERIALS AND METHODS: Of 4 women and 23 men monitored at our institution 16 (59%) underwent primary diversion by ureterosigmoidostomy, while 11 (41%) underwent primary bladder closure or an ileal conduit procedure before conversion to ureterosigmoidostomy. Average followup after ureterosigmoidostomy was 17 years. RESULTS: Significant upper urinary tract changes developed in 18% of the patients. Metabolic acidosis was well compensated in most patients but 2 had problems with urinary retention leading to hyperammonemia and acidosis. Of the 19 patients monitored with biennial colonoscopy benign polyps were removed in 4. Daytime continence was achieved in 92% of cases and nighttime continence in 58%. CONCLUSIONS: Our experience with ureterosigmoidostomy in children with bladder exstrophy has been favorable through long-term followup. With proper imaging, metabolic surveillance, biennial colonoscopy and nonsteroidal anti-inflammatory drugs we offer ureterosigmoidostomy as a viable alternative for patients with small bladders.

Bladder Exstrophy↗
PubMed1996

Bilateral anterior pubic osteotomy in bladder exstrophy closure.

PURPOSE: We report our clinical experience with anterior pelvic osteotomy in 16 patients who underwent surgery for bladder exstrophy. The technique and its difficulties are discussed. MATERIALS AND METHODS: Anterior pelvic osteotomy of the superior ramus of the public bone is a simple and efficient method to facilitate symphyseal approximation and abdominal wall closure without or with low tension on the suture lines in neonates who undergo surgery for bladder exstrophy. Older children in whom surgery has been delayed can also benefit from this method. Compared to other methods of osteotomy in exstrophy surgery it does not require additional incisions, nor does the patient need to be repositioned on the operating table. A successful operation does not depend on any particular orthopedic skills and it can easily be done by the pediatric urologist. RESULTS: Immediate postoperative results regarding abdominal wall closure were excellent in all 16 patients. However, major postoperative complications developed in 2 patients. Despite antibiotic prophylaxis a severe soft tissue infection developed in 1 child, resulting in complete bladder dehiscence. In another patient an obturator nerve injury resulted in transient palsy, which resolved completely. While the first complication was not related to osteotomy, the second was osteotomy related. CONCLUSIONS: Bilateral superior ramotomy of the pubic bones is a new alternative, easily performed technique to optimize bladder exstrophy surgery in children.

Bladder Exstrophy↗
PubMed2005

A reciprocal translocation 46,XY,t(8;9)(p11.2;q13) in a bladder exstrophy patient disrupts CNTNAP3 and presents evidence of a pericentromeric duplication on chromosome 9.

A patient with sporadic bladder exstrophy and de novo apparently balanced chromosomal translocation 46,XY,t(8;9)(p11.2;q13) was analyzed by fluorescence in situ hybridization (FISH) and molecular methods. We were able to map both translocation breakpoints to single genomic clones. The chromosome 8p11.2 breakpoint was mapped to BAC clone RP4-547J18, predicted to contain several hypothetical genes. Characterization of the chromosome 9q13 breakpoint indicated a disruption in the 5' region of CNTNAP3 within BAC RP11-292B8. This observation suggests possible involvement of CNTNAP3 in the etiology of bladder exstrophy. Additionally, FISH analysis identified several genomic copies of CNTNAP3 on both sides of the chromosome 9 centromere flanking the polymorphic heterochromatin. Northern blot analysis of lymphoblast and bladder RNA confirmed CNTNAP3 transcripts in these tissues and did not show abnormal CNTNAP3 expression in the proband and two unrelated patients with bladder exstrophy. The identification of multiple copies of three BAC clones in the proband, his parents, and unrelated controls suggests that duplications of CNTNAP3 and the surrounding genomic region have occurred as a result of repeated events of unequal crossing over and pericentric inversions during chromosome 9 evolution.

Base Sequence↗
PubMed1998

Somatic function, mental health and psychosocial functioning in 22 adolescents with bladder exstrophy and epispadias.

PURPOSE: We report the long-term somatic outcome, mental health and psychosocial adjustment in adolescents with bladder exstrophy and epispadias. MATERIALS AND METHODS: A total of 22 adolescents 11 to 20 years old (median age 14.5), including 19 with bladder exstrophy and 3 with epispadias, were assessed for urogenital status, stoma, renal and bowel function, anorectal physiology, mental health and psychosocial functioning by physical examinations, semistructured interviews and standardized questionnaires. The parents of 21 patients were interviewed and completed questionnaires. Information was also obtained on control groups. RESULTS: Of the 22 patients 9 (41%) had no urinary diversion and were urinary incontinent, 6 (27%) had persistent fecal staining and anal canal pressures that were lower than the controls, 10 (59%) were dissatisfied with the penile appearance and 11 (50%) met the criteria for psychiatric diagnoses. The main predictors of mental health were parental warmth and patient genital appraisal in the 11 to 14-year age group, and parental warmth and urinary continence function in the 15 to 20-year age group. Psychosocial dysfunction was predicted by fecal incontinence in the younger group and worries about future sexual relationships in the older group. CONCLUSIONS: The present multimodal outcome study revealed that adolescents with bladder exstrophy and epispadias had significant physical and mental problems. Genital malformation, and urinary and fecal incontinence may have a negative impact on mental health and psychosocial functioning. Our findings emphasize the need to include psychosocial experts on health care teams to reveal the amount of distress caused by these anomalies and to offer psychosocial support.

Adolescent↗
PubMed1983

Reconstruction of abdominal wall and mons pubis in females with bladder exstrophy.

We report on 3 patients with bladder exstrophy who had considerable scar deformities of the lower abdomen after primary closure of the bladder and/or urinary diversion. The scar tissue was excised, and augmentation of the abdominal wall and mons veneris was achieved by the use of a pedicled, vascularized groin flap with integrated bone from the iliac crest. This procedure is recommended for patients with faulty closure of the pelvic ring and severe defect of the lower abdominal wall.

Abdominal Muscles↗
PubMed2002

Augmentation ureterocystoplasty in bladder exstrophy: 5-year follow-up in two cases.

OBJECTIVE: To report two cases of bladder exstrophy managed successfully by augmentation ureterocystoplasty (UCP) together with bladder neck surgery and continent diversion. PATIENTS: Two boys, age 5 and 1 years respectively, had augmentation UCP. The left refluxing megaureter was used in the first case together with bladder neck reconstruction. In the second patient, bilateral obstructed magaureters were used in tandem together with bladder neck division. Both patients had appendicovesicostomy according to Mitrofanoff. RESULTS: In follow-up for over 5 years, both patients are continent with improved upper urinary tracts and normal or stable renal function. They are managed by clean intermittent catheterization (CIC) via the continent stoma with bladder capacities of 220 cc and 150 cc, respectively. Cystogram showed no vesicoureteric reflux (VUR). The procedures were considered to be successful although one patient was on oxybutinin because urodynamics showed high intravesical pressures. CONCLUSIONS: Augmentation UCP should be considered in patients with bladder exstrophy when a suitable megaureter is available. This may be combined with simultaneous bladder neck reconstruction together with a continent diversion.

Bladder Exstrophy↗
PubMed1978

Gas cystometry in cases of continent bladder exstrophy.

Cystometry in 21 cases of closed continent bladder exstrophy revealed that 14 patients had a normal reflexic bladder. A simultaneous anal plug electromyogram showed an appropriate response in all patients when correlated with a cystometrogram.

Adolescent↗