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PubMed1994

The modified rectal bladder (the augmented and valved rectum) for urine diversion in children.

OBJECTIVES: Urinary diversion is usually the procedure of choice for children having complicated primary closure for bladder exstrophy. We introduce the modified rectal bladder as a low pressure and functionally isolated rectal reservoir as a bladder substitute for these cases. METHODS: Modified rectal bladder urinary diversion was done on 15 children as a low pressure and functionally isolated rectal reservoir via the adoption of sigmoid intussuscepted valve and the rectal patching with detubularized sheet of ileum. Fourteen of these children are currently evaluable, with follow-up ranging from 16 to 72 months (median 55 months). All of them are subjected to thorough history-taking, clinical examination, laboratory and radiologic investigations, and urodynamic study. RESULTS: A high rate of urinary continence was achieved and so far the upper urinary tract and the metabolic status were preserved. Reflux to the colon and kidneys was prevented. Urine samples from the renal pelvis through percutaneous needle aspiration revealed sterile cultures in 82% of the renal units (23 of 28). CONCLUSIONS: Our results demonstrate the distinct advantages of the modified rectal bladder over the conventional methods of urinary diversion to the rectum or the abdominal reservoirs coupled to the skin via continent catheterizable stomas.

Bladder Exstrophy↗
PubMed1988

Augmentation cystoplasty in the failed exstrophy reconstruction.

Of the 148 patients with bladder exstrophy seen at this institution during the last 10 years 12 have ultimately required bladder augmentation. In 4 cases augmentation was performed for an inadequate bladder capacity, upper tract decompensation and dry interval of less than 1 hour after bladder neck reconstruction and epispadias repair. Likewise, 3 patients underwent augmentation for an inadequate bladder capacity and dry interval of less than 2 hours after bladder neck reconstruction and epispadias repair. Of these 7 patients 3 had undergone 2 previous bladder neck reconstructions, while 4 had undergone 1 prior repair. Five augmentations were performed for an inadequate bladder capacity before any type of continence procedure had been done. Nine patients underwent adjunctive procedures in addition to bladder augmentation, including a Young-Dees-Leadbetter procedure in 4, an artificial urinary sphincter in 3, transureteroureterostomy and psoas hitch in 1, and a Mitrofanoff procedure and bladder neck closure in 1. Of the 12 patients 11 are continent, although 9 require intermittent catheterization. There were no major complications. However, 1 artificial urinary sphincter was removed for erosion 2 years after placement. Augmentation cystoplasty has provided prolonged stability of the upper tracts and continence in these patients, and it has proved to be a successful alternative to urinary diversion in this select group of exstrophy failures.

Bladder Exstrophy↗
PubMed1992

Continent appendicocolostomy: a variation of the Mitrofanoff principle in pediatric urinary tract reconstruction.

There are numerous options for continent urinary reconstruction in children. If the appendix is available and the bladder capacity is satisfactory, a Mitrofanoff procedure may be performed using the appendix as a catheterizable tube implanted into the bladder. However, in many patients the bladder is absent or too small for this type of reconstruction. An alternative technique, termed appendicocolostomy, is to implant the appendix under the tenia of a detubularized patch of cecum or sigmoid colon, which becomes part of a continent neobladder or is attached to the bladder itself. This procedure has been used in 12 children and 1 adult undergoing continent reconstruction. Urological diseases included bladder exstrophy (10 patients) and a neuropathic bladder (3). Two patients underwent undiversion of a sigmoid conduit, while in 7 of the remaining patients the bladder was included in the reservoir. In 1 patient histological examination of the tip of the appendix revealed an incidental invasive carcinoid tumor necessitating appendectomy and revision of the reconstruction. Otherwise, no complications have occurred. Three adults were scheduled for this procedure but the appendix was diseased and an alternative form of diversion was necessary. Followup ranged from 2 months to 4 years. All patients are totally dry with a capacity of 300 to 750 cc (mean 475 cc). The appendicocolostomy is a superior form of conduit reconstruction that should be considered when the appendix is available during continent reconstruction. However, in adults the appendix may be fibrotic, precluding its use. Because most boys with exstrophy and a small bladder capacity requiring augmentation cystoplasty must perform intermittent catheterization, attachment of the appendix to the reservoir is an alternative that allows for easier and less painful intermittent catheterization than catheterization of the reconstructed epispadiac penis.

Adolescent↗
PubMed1993

Differential diagnosis of the nonvisualized fetal urinary bladder by transvaginal sonography in the early second trimester.

OBJECTIVE: To describe the pathologic situations associated with the persistently nonvisualized fetal urinary bladder in the early second trimester. METHODS: We analyzed retrospectively 13,458 ultrasound examinations performed between 12-16 weeks' gestation in search of fetal malformations. Seventy percent of the patients were at low risk and 30% comprised a high-risk group for the detection of fetal anomalies. RESULTS: Seven cases of persistent nonvisualization of the fetal urinary bladder were detected by transvaginal sonography among 13,458 fetuses. In one case bladder exstrophy, and in another bilateral multicystic dysplastic kidneys were diagnosed by postabortal examination. Five cases were associated with bilateral renal agenesis. CONCLUSION: When one fails persistently to demonstrate the fetal urinary bladder during an examination lasting 30 minutes or longer, urinary tract pathology should be highly suspected. Failure to visualize the bladder may be associated with bladder exstrophy, bilateral renal agenesis, or late onset of bilateral multicystic dysplastic kidneys.

Adult↗
PubMed1996

Prenatal diagnosis of fetal bladder and cloacal exstrophy by ultrasound. A report of three cases.

BACKGROUND: Bladder and cloacal exstrophy can be diagnosed with prenatal ultrasound. CASES: Three cases of bladder and cloacal exstrophy were diagnosed prenatally by ultrasound and confirmed at birth. The ultrasound findings were a soft tissue mass in the lower abdominal wall (which appeared larger and more heterogeneous in cloacal exstrophy than in bladder exstrophy), absent bladder, malformation of the external genitalia and normal kidneys along with normal amniotic fluid volume. CONCLUSION: Prenatal diagnosis of these defects will allow appropriate referrals prior to birth.

Abortion, Therapeutic↗
PubMed1994

The fate of the adult exstrophy patient.

Between 1968 and 1993, 101 patients with bladder exstrophy or incontinent epispadias underwent surgery at our hospital. The standard procedure was ureterosigmoidostomy and additional genital reconstruction. Of the 56 patients who have reached adulthood 45 could be interviewed regarding social integration, sexuality and fertility. All patients have a functioning urinary diversion. Of the 45 patients questioned 41 have completed vocational training or are currently in training, 3 are unemployed and 1 lives at a therapeutic center. Among the patients 29 are married or have a steady partner. All women engage in sexual intercourse and 2 have delivered 3 children by cesarean section. All men achieve erection. Of the 28 men who underwent reconstruction of the external genitalia 11 have a penile deviation, which is distressing in only 2. Only 3 of the men are dissatisfied with the cosmetic result and 33% had epididymitis, necessitating 2 orchiectomies and 3 vasectomies. No patient with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas all 5 who did not undergo genital reconstruction had normal ejaculation and 2 have fathered children. Male patients with genital reconstruction and closure of the urethra have a high risk of infertility. Our patients demonstrate that the cosmetic results after genital reconstruction are satisfactory. However, in male patients, surgery is performed at the expense of fertility. Because this corrective procedure is usually performed during childhood, the parents must be informed of these consequences before surgical correction.

Adolescent↗
PubMed1983

Adenocarcinoma in exstrophy of the bladder--the last case in Scandinavia? A case report and review of literature.

There have been 80 cases of carcinoma in exstrophied bladders described previously. We describe another such case in which the specimen was investigated by mucin histochemical and immunohistochemical techniques. The epithelial lining of the bladder was colonic with colon-specific mucin histochemistry and contents of carcinoembryonic antigen. In many foci there were dysplasia of the colonic epithelium and multiple colonic exophytic adenocarcinomas.

Adenocarcinoma↗
PubMed2000

How well do patients with exstrophy actually void?

PURPOSE: Achieving continence and preserving renal function are goals in the care of patients with bladder exstrophy. The Young-Dees-Leadbetter bladder neck reconstruction should ideally provide continence and normal voiding dynamics without the need for intermittent catheterization. We review our experience with bladder neck reconstruction in this population with emphasis on voiding dynamics among those patients doing well. MATERIALS AND METHODS: We retrospectively analyzed all patients with the exstrophy-epispadias complex seen at our institutions since 1985. We reviewed staged reconstruction in 53 patients, including 31 with classic bladder exstrophy, 4 with exstrophy variants and 18 with incontinent epispadias. Patients with additional neurogenic dysfunction were excluded from study. Subjective and objective data regarding voiding function and complications were collected. RESULTS: Complete reconstruction for continence was performed in 38 cases, of which 11 that required bladder augmentation with bladder neck reconstruction or who had a different primary continence procedure were excluded from study. The remaining 27 patients treated with the Young-Dees-Leadbetter bladder neck reconstruction had 2 or more years of followup (mean 5.9). Dry intervals of at least 2 hours were achieved by 18 patients and all were considered by parents to void well. Despite near or total subjective continence and "good" voiding, 13 of these 18 patients (72%) have clinical problems related to emptying, which include recurrent urinary tract infections in 10, epididymitis in 2 and bladder calculi in 4. Objective urodynamic parameters confirm poor voiding in most patients. CONCLUSIONS: Bladder neck reconstruction in patients with exstrophy can achieve continence without intermittent catheterization. In our experience patients who achieve these goals have an alarming frequency of clinical and urodynamic problems related to emptying. One must question the normalcy of the voiding pattern and price to achieve continence among patients with exstrophy.

Bladder Exstrophy↗
PubMed2004

Linear growth after enterocystoplasty in children and adolescents: a review.

The interposition of bowel in continuity with the urinary tract has allowed for the preservation of renal function and continence in children with bladder exstrophy, as well as neurogenic and valve bladders. Although bladder augmentation with ileum or colon has been shown to be safe, the long-term effects of metabolic acidosis in addition to abnormalities in linear growth and bone metabolism remain largely unknown. We reviewed the literature to critically examine linear growth in children who have had bladder augmentation with a particular emphasis on the correlation between acid-base status, bone mineralization and growth. The majority of studies suggest that linear growth is not affected by bladder augmentation. In the short-term, children post-augmentation have varying degrees of metabolic acidosis which, overtime, appears to resolve with no affect on linear growth. In a single study, bladder augmentation led to significant bone demineralization almost a decade after surgery, however, even in these children no decrease in linear growth was noted. No alterations in bone density levels were seen with short-term follow-up.

Acid-Base Equilibrium↗
PubMed1989

Closure of the exstrophic bladder: an evaluation of the factors leading to its success and its importance on urinary continence.

To determine what factors could affect the success of initial bladder closure in classical bladder exstrophy, 80 patients who have completed staged bladder reconstruction were reviewed. Evaluations comparing early bladder closure performed with or without bilateral iliac osteotomy versus delayed closure performed with iliac osteotomy revealed no statistical difference among the 3 populations in regard to the development of bladder dehiscence or onset of urinary continence (p greater than 0.5). Individuals undergoing delayed bladder closure without iliac osteotomy had no notable difference in the incidence of bladder dehiscence (p greater than 0.5) but they had a statistically significant difference in the ability to gain urinary continence (p less than 0.01). Bladder prolapse, abdominal distension or loss of urinary drainage catheters was significantly related to bladder dehiscence (p less than 0.02). Urinary continence was obtained in 75 per cent of the patients with successful bladder closure, compared to 30 per cent if the initial closure dehisced (p less than 0.01).

Anti-Bacterial Agents↗
PubMed1989

Posterior sagittal anorectoplasty for pediatric recurrent rectal prolapse.

The recent use of the posterior sagittal anorectoplasty for repair of high imperforate anus has demonstrated several advantages: elimination of laparotomy, more direct approach, easier division of rectourethral fistula, more exact identification of the muscles of fecal continence, proper relocation of anorectum within these muscles and sphincters, and virtual elimination of postoperative anal prolapse. It is this latter advantage that attracted us to use this procedure for the repair of a recurrent rectal prolapse in a 1-year-old girl who also had a recurrent bladder exstrophy. The latter probably contributed to her constantly pushing out her rectum, which easily admitted two fingers. Two attempts were made to repair the rectal prolapse using the subcutaneous Thiersch's perianal technique; however, each was successful for only 6 weeks. When her recurrent bladder exstrophy was repaired, we also repaired her recurrent rectal prolapse using the posterior sagittal anorectoplasty. The midline sacrococcygeal incision was carried down to but not through the external sphincter, and the patulous rectum was plicated back to a normal size. Reapproximation of the levator sling and lower muscle complex then incorporated the plicated rectum. Both repairs remain intact after 1 year.

Anal Canal↗
PubMed2004

Ultrastructure of the bladder in classic exstrophy: correlation with development of continence.

PURPOSE: Successful initial surgical management of bladder exstrophy does not always lead to continence. We evaluated the ultrastructure of the exstrophic bladder using electron microscopy (EM) at various stages of reconstruction to determine whether morphology could correlate with the potential for continence. MATERIALS AND METHODS: Bladder specimens obtained from 32 patients undergoing various stages of exstrophy reconstruction were evaluated by EM. Specimens were obtained at primary newborn closure (group 1-10), reclosure following failure (group 2-2), bladder neck reconstruction (group 3-9) and augmentation cystoplasty (group 4-11). Evaluation was performed by a single anatomist with experience with EM. Biopsies were separated into those with good, intermediate or poor ultrastructural parameters and then correlated clinically. RESULTS: In group 1, 4 children had good and 2 had intermediate parameters. All showed increased bladder volumes at followup. Four patients had poor parameters and poor bladder growth. The 2 group 2 children had poor parameters and bladder growth. In group 3, 7 of 9 patients had good and 1 had intermediate parameters. Eight of the 9 patients are doing well. Only 3 of the 11 group 4 children had good parameters and an additional 2 had intermediate parameters. CONCLUSIONS: Ultrastructural evaluation can identify changes in the bladder that may portend a poor prognosis for eventual continence. Although the correlation was good at bladder closure, some patients with good parameters still had failed reconstruction. Since this is a small study, we continue to recommend reconstruction in all patients who have an adequate bladder template.

Biopsy↗
PubMed1996

Rectus abdominis muscle flap (RAMF) technique for the management of large bladder defects: urodynamic findings in a rabbit model.

OBJECTIVE: To investigate the efficacy of the rectus abdominis muscle flap (RAMF) technique for the closure and augmentation of small, inelastic bladder extrophies using a rabbit model with a large bladder defect. MATERIALS AND METHODS: Fifteen New Zealand White rabbits were studied in two groups; nine rabbits in group 1 underwent resection of 65% of the bladder and then bladder augmentation using a full-thickness RAMF, including peritoneum, and six rabbits in group 2 (control) underwent a similar bladder resection but the bladder remnant was closed primarily. Urodynamic investigations were carried out in both groups preoperatively and 4 weeks after surgery. Intravenous pyelography (IVP), voiding cystourethrography (VCUG), urodynamic and histopathological investigations were also carried out for up to 4 months after surgery. RESULTS: As assessed by IVP, the urinary system appeared normal, with no signs of upper urinary tract dilatation and VCUG showed a normal voiding pattern. After surgery, the bladder capacity and compliance decreased in both groups, but both variables partially recovered in group 1, although not significantly. CONCLUSIONS: This animal model indicates that the RAMF technique is a suitable alternative for the management of large bladder defects and bladder exstrophies with small, inelastic, non-compliant bladders.

Animals↗
PubMed2005

Can epithelized neobladder-cutaneous fistula provide urinary continence?

The paper presents a case of a boy born with a bladder exstrophy, who underwent several surgical procedures, including bladder augmentation. During the last procedure, the patient received a suprapubic catheter that accidentally slipped out. The epithelized neobladder-cutaneous fistula resulted in a continent and easily catheterizable abdominal stoma.

Adolescent↗
PubMed1998

Consequences of prenatal ultrasound diagnosis: a preliminary report on neonates with congenital malformations.

OBJECTIVE: The aim of the present study was to examine the sensitivity of prenatal ultrasound diagnosis in neonates referred for surgery, and to test whether a prenatal versus postnatal diagnosis influenced mode of delivery and neonatal outcome of these infants. PATIENTS: Thirty-six consecutive neonates with congenital diaphragmatic hernia, abdominal wall defects, bladder exstrophy and meningomyelocele were included. RESULTS: The sensitivity of prenatal ultrasound for diagnosis of the congenital malformations was 7/36 (19%) at 17-18th week of gestation, and overall 13/36 (36%). Overall sensitivity was 2/8 in neonates with congenital diaphragmatic hernia, 6/12 in neonates with abdominal wall defects, 5/13 in neonates with meningomyelocele, whereas none of three cases with bladder exstrophy were detected prenatally. No significant improvement in neonatal morbidity was found comparing the prenatally and postnatally diagnosed groups. The neonatal survival rate was 10/13 (77%) in the prenatally diagnosed group and 22/23 (96%) in the postnatally diagnosed group (p=0.12). CONCLUSIONS: The sensitivity of prenatal ultrasound in diagnosing the congenital malformations under study in a low risk population was 19% at 17-18th week of gestation and 36% throughout the pregnancy. Prenatal diagnosis altered management of labor, but caused no improvement in neonatal outcome.

Congenital Abnormalities↗
PubMed2000

Urethral duplication in the male: review of 16 cases.

PURPOSE: Urethral duplication is a rare congenital anomaly. The clinical presentation varies because of the different anatomical patterns of this abnormality. We describe our experience with 16 male patients with this anomaly. MATERIALS AND METHODS: We retrospectively reviewed the records of 16 male patients treated for urethral duplication in the last 10 years. Age at presentation ranged from newborn to 8 years. Evaluation included ultrasound, voiding cystourethrography, retrograde urethrography and endoscopy. RESULTS: A blind ending duplicated urethra (type I) was present in 4 patients, 2 independent urethras with distinct bladder necks (type IIA1) in 6, 2 urethras originating from a common bladder neck (type IIA2) in 4, and complete urethral and bladder duplication (type III) in 2. Six patients had associated vesicoureteral reflux. Duplication was an incidental finding at epispadias repair in 3 patients with bladder exstrophy, at hypospadias repair in 1 and at hydrocele repair in 1. One patient with bilateral dysplastic kidneys died in the newborn period. Surgical management included excision of the duplicated urethra in 8 cases and urethroplasty using a pedicle flap in 2, while surgical management was not required in 3. CONCLUSIONS: Urethral duplication is a rare congenital anomaly with a variable clinical presentation. This pathological condition may easily be under diagnosed, especially in patients with other associated anomalies, such as hypospadias or bladder exstrophy. Surgical management should be planned individually according to the anatomical findings of the abnormality.

Child↗