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PubMed2004

Current management of bladder exstrophy.

Bladder exstrophy is a complex anomaly involving the urinary, genital, and intestinal tracts and the musculoskeletal system. The diagnosis is made typically at the newborn examination or on fetal ultrasonography that is performed by an experienced observer. Management of bladder exstrophy presents several challenges, beginning with initial repair using the more conventional staged approach or the recently re-popularized complete primary repair technique. Major goals in the management of bladder exstrophy are preservation of normal kidney function, close observation for development of adequate bladder function including urinary continence, and provision of acceptable cosmesis and function of the external genitalia. This article provides a brief overview of bladder exstrophy and a detailed description of modern management.

Bladder Exstrophy↗
PubMed2004

Pregnancy in a couple with a male partner born with severe bladder exstrophy.

Bladder exstrophy is a rare congenital condition, with variable degrees of reproductive dysfunction, from mild exstrophy allowing regular intercourse and reproduction, to severe forms with significant cosmetic deformity and failure to reproduce spontaneously. This is a case report of a patient with severe congenital bladder exstrophy and failure to reproduce spontaneously due to microphallism and anejaculation, treated with testicular sperm aspiration and intracytoplasmic sperm injection (ICSI), resulting in the conception and birth of a healthy unaffected singleton male infant. This the first case report of bladder exstrophy of such a severe degree with a successful reproductive outcome. It illustrates that artificial reproductive techniques can be successful in achieving conception and ongoing pregnancy, including birth of a healthy infant, in patients where intercourse is impossible due to specific genital defects.

Adult↗
PubMed1997

Female bladder exstrophy.

Bladder exstrophy is one of the most challenging congenital urinary tract abnormalities. Apart from the open bladder the patient also has various other abnormalities, including urogenital, musculoskeletal and anorectal defects. The size of the exstrophic bladder varies from patient to patient. In the female the clitoris to bifid and the vagina is anteriorly placed. In this paper various aspects of female bladder exstrophy are reviewed in detail, including incidence, etiology, anatomy and clinical features, together with early, medium-term and long-term surgical management. The aim is to achieve a functional bladder closure, although some patients are better off with a urinary diversion. Considering the complexity of the urogenital problems and the surgical management thereof, all patients require lifelong follow-up.

Abnormalities, Multiple↗
PubMed1998

Successful pregnancy in a patient with previous bladder exstrophy.

Bladder exstrophy is an unusual congenital anomaly. Patients becoming pregnant with such anomaly after surgical repair are even rarer. The present case reports a lower segment Caesarean section delivery of a living healthy baby in a woman who had been operated on for bladder exstrophy at the age of 18 years. This is the first case of this type seen in our institution in 35 years.

Adult↗
PubMed2004

Initial management of complex urological disorders: bladder exstrophy.

Bladder exstrophy is a rare condition associated with lifelong issues including infection,renal insufficiency, incontinence, and fertility. Despite its seriousness, many affected children lead normal lives. With new operative techniques, children require fewer surgeries and have more hope for continence. With close follow-up, no child should develop renal insufficiency. Families should be reassured that a normal life and parenthood with normal sexual function are possible.

Animals↗
PubMed2002

The role of imipramine therapy in bladder exstrophy after bladder neck reconstruction.

OBJECTIVE: To assess the role of imipramine therapy after bladder neck reconstruction in patients with bladder exstrophy. PATIENTS AND METHODS: Seventeen children with bladder exstrophy who achieved partial continence after bladder neck reconstruction received imipramine (1.5-2 mg/kg body weight) for a mean duration of 9.5 months. Assessment before and after therapy included an objective classification of the continence status and artificial slow-fill cystometry. RESULTS: Eleven of the 17 patients had a good clinical response to imipramine, with an increase in the continent period to > 2 h, and in nocturnal continence. The urodynamic findings showed a significant improvement in the capacity, end-fill pressure, uninhibited contractions and '20 below' capacity. Only minor side-effects of the drug were reported. CONCLUSION: Imipramine has a role in patients who achieve partial continence after reconstruction, and who have a moderately small capacity bladder with poor compliance and uninhibited contractions.

Antidepressive Agents, Tricyclic↗
PubMed2006

Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder.

The surgical management of classic bladder exstrophy has evolved over time. Different techniques are used to address the challenge of reconstructing these patients. We review the long-term outcomes of bladder exstrophy treatment from the published literature with regard to urinary continence, voiding and secondary complications. Continence now can be achieved in up to 80% of children in specialist centres. Whether such success can sustained into adult life is uncertain. About 40% of adults are dry in the best hands. Up to 84% of children can void, but there is some evidence that this function is lost with time in 70%. The need for bladder augmentation is widely variable between series, reported in 0-70% of children. This reduces the ability to void spontaneously to about 50% of children. It brings with it the later risk of metabolic disturbance and stone formation. Adults with exstrophy have a 694-fold increase in the risk of bladder cancer by the age of 40 years.

Adult↗
PubMed1999

A large animal model of bladder exstrophy: observations of bladder smooth muscle and collagen content.

PURPOSE: The development of adequate bladder capacity is not ensured in all patients with bladder exstrophy despite successful bladder closure and reconstruction. To determine the factors leading to the development and maturation of the exstrophic bladder we created a large animal model of exstrophy. We compared biopsies obtained from a cohort of experimentally induced exstrophic neonatal sheep bladders to those of normal control bladders and related the findings to a previously reported comparison of human neonatal normal and exstrophic bladders. MATERIALS AND METHODS: Bladder specimens of 7 newborn lambs with experimentally induced exstrophy were compared to specimens of 10 newborn control lamb bladders. All specimens were stained with Masson's trichrome as well as with specific monoclonal antibodies to types I and III collagen. Stained sections were then analyzed using a morphometric image analysis system to quantify the amounts of smooth muscle and collagen present. RESULTS: A significant increase in the ratio of collagen-to-smooth muscle was noted in exstrophic versus normal control bladders (p <0.05). This difference was similar to that in the previous study of neonatal human bladders. There was no significant difference in the ratios of types I and III collagen in the 2 groups of sheep bladders. This finding is different from that reported in the previous human studies. CONCLUSIONS: Overall changes in the ratio of smooth muscle-to-collagen in the sheep exstrophy model are similar to those in humans. However, the differences in collagen types I and III do not seem to be present.

Animals↗
PubMed2002

Spontaneous twin gestation after vaginal dilation in a woman with uterus didelphys and bladder exstrophy.

BACKGROUND: Bladder exstrophy is an uncommon anomaly rarely associated with uterus didelphys. Fertility is limited by associated vaginal malformations, which have traditionally required surgical reconstruction. CASE: A woman with a history of bladder exstrophy and hypoplastic vagina presented complaining of painful intercourse. The patient underwent vaginal dilator treatment after corrective surgery for the bladder defect. Without any further fertility therapy she subsequently conceived twins, with one implantation in each horn of a didelphic uterus. CONCLUSION: Bladder exstrophy, uterus didelphys, and vaginal hypoplasia share a common embryological devel-opment, and the finding of one anomaly can alert to the presence of another. Vaginal dilators may be used in place of surgical reconstruction to allow conception.

Abnormalities, Multiple↗
PubMed1982

One stage functional reconstruction of exstrophied bladder: report of two cases with six-year follow-up.

Two female babies aged 8 and 13 mo., affected with bladder exstrophy, were submitted to a single-stage functional reconstruction of the bladder. Innominate osteotomy, bladder closure, anti-reflux procedure and narrowing of the bladder outlet followed by pubic reapproximation and easy closure of the abdominal wall were performed in one session. Bladder enlargment and a steadily improving continence occurred in the first case with no reflux and no urinary infection. Further surgery may not be needed if full urinary control can be achieved through active cooperation by the patient. Postoperative continence was achieved in the second patient with a secondary operation on the bladder outlet and pedicled muscle transplant. The absence of reflux, infection and pyelonephritis dis warrant a safe and definite bladder enlargement, with a very satisfactory functional result. An anti-reflux procedure should always be performed at the time of bladder reconstruction in babies older than 3 or 4 months.

Bladder Exstrophy↗
PubMed2002

Growth and metabolic consequences of bladder augmentation in children with myelomeningocele and bladder exstrophy.

OBJECTIVE: Bladder augmentation using intestinal segments is reported to cause decreased linear growth in bladder exstrophy and myelomeningocele patients. We studied changes in calcium metabolism, height, bone chemistry, and bone density in exstrophy and myelomeningocele patients after bladder augmentation. METHODS: Thirty-three patients were prospectively admitted to the Pediatric Clinical Research Center at the University of California San Francisco for 24 hours. Blood and urine were analyzed for electrolytes, and serum was obtained for markers of calcium metabolism. Dual radiograph bone densitometry of the forearm was performed. Myelomeningocele patients were compared with nonaugmented myelomeningocele patients matched by age, gender, level of defect, and ambulatory status. Exstrophy augmented patients were compared with nonaugmented exstrophy patients. The bone densities in both groups were compared with normal children. Laboratory values and percentile heights were statistically analyzed using the Student t test; bone densitometry was analyzed using the Tukey test. RESULTS: Twenty-two patients with myelomeningocele and 11 with bladder exstrophy were studied. Mean follow-up was 3.7 years postaugmentation (range: 1-13 years). The results indicate a significant difference in serum bicarbonate and chloride levels between myelomeningocele patients who underwent ileal augmentation and those who did not. Although this may be indicative of chronic metabolic acidosis, there was no affect on growth or bone density when compared with controls. There were no other significant differences in laboratory values, or percentile heights, nor were any differences noted in patients who underwent gastrocystoplasty. In the exstrophy group, there were no observable differences in percentile height or laboratory values between the augmented and nonaugmented group. There were no significant differences in bone density between these 2 groups when matched for age and gender. No significant difference was seen in bone density when these groups were compared with normal children. CONCLUSION: Bladder augmentation is safe and does not impact negatively on the linear growth or bone densities of patients with myelomeningocele or bladder exstrophy.

Adolescent↗
PubMed2004

Anatomical repair of exstrophy bladder.

An anatomical repair of bladder exstrophy was done in ten patients (six females and four males, aged 1.5-6 months. We describe our method of exstrophy with epispadias repair done in a single stage achieving anatomical closure including relocating skin from an infraphallic to a supraphallic position. The cosmetic and functional results are evaluated. The advantages of this technique are described.

Bladder Exstrophy↗
PubMed2000

[Selected secondary reconstructive procedures for improvement of urinary incontinence in bladder exstrophy and neurogenic bladder dysfunction in childhood].

Partial or complete urinary and stool incontinence due to malformation of the genito-urinary tract and the pelvic floor despite of several operative reconstructions is the most important handicap in the patients life. Often this problems seems to be unsoluble. In recent time we secondarily reconstructed 7 patients suffering from urinary incontinence: 1 girl and 4 boys with bladder ekstrophy; 1 boy with a complex anomaly with menigomyelocele, sinus urogenitalis, single kidney with vesicorenal reflux and neurogene bladder; 1 boy with complex anomaly of the pelvis and the lower limbs with duplication of the bladder with an ekstrophic left part. All patients underwent multiple operative trials of reconstruction. Until that time all patients suffered from complete urinary incontinence. At the age of 5 to 14 years we performed the secondary reconstruction: bladder-neck-plasty and ileumaugmentation (3 patients), closure of the bladder-neck, ileumaugmentation and a continent appendicostoma (Mitrofanoff's method)), Mainz-I-pouch and a continent appendicostoma respectively ileostoma (Monti's technique) (2 patients), Mainz-II-pouch. With a normal bladder-capacity all patients are completely continent postoperatively; one patient has regained partial continence. The emptying of the bladder is carried out by clean intermittent catheterism (CIC) with the exception of the one patient with the Mainz-II-pouch. Even in patients with complex anomalies of the pelvic floor and the genitourinary tract complete urinary continence is possible in consequence of recently developed operative techniques. Because of a high rate of complications we reject the primary use of artificial sphincter systems for children.

Abnormalities, Multiple↗