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PubMedDate not supplied

Uterine prolapse associated with bladder exstrophy: surgical management and subsequent pregnancy.

Congenital bladder exstrophy affects 1 in 125,000 to 250,000 females. Consisting of absence of the anterior abdominal wall with exposure of the ureteral orifices, failure of pubic symphysis fusion, and deficient anterior pelvic diaphragm musculature, bladder exstrophy is frequently associated with genital prolapse. Pregnancy may be complicated by recurrent urinary tract infections, preterm labor, mild procidentia, and malpresentation. Due to the rarity of the condition, there is a corresponding scarcity of obstetric literature regarding management during pregnancy. We report the case of a young woman with surgically repaired bladder exstrophy who developed genital prolapse. The uterus was suspended using a sacral colpopexy utilizing a Gore-Tex graft. Subsequently, the patient became pregnant and delivered a healthy male infant at 35 weeks' gestation via cesarean section (without recurrence of the genital prolapse postpartum). Sacral colpopexy to correct genital prolapse associated with bladder exstrophy may preserve fertility in young patients.

Adolescent↗
PubMed2004

Bladder exstrophy: effects on bone age, bone mineral density, growth, and metabolism.

Bladder exstrophy patients with or without augmentation have not been investigated according to metabolic bone problems, bone ages and growth, and development in details yet. We studied alterations in growth, bone ages, biochemistry of bone, bone mineral densities (BMD) of the forearm, neck of femur and lumbar vertebrae, blood gases, glomerular filtration rates (GFR), and electrolytes of 15 bladder exstrophy patients with augmentation and in those who had no augmentation. In six patients, a sigmoid colon was used for bladder augmentation and one patient underwent a ureterosigmoidostomy. Growth charts of all children were analyzed for determination of the percentiles. The parameters were compared with normal children and a comparison between augmented and nonaugmented patients were made. Growth retardation and decreased bone age were detected in all of the children. Ten patients with bladder exstrophy were below the 10th percentile for height. The mean age/bone age ratio of the patients was 1.59. The mean lumbar and femoral Z scores of the patients were -1.00 and -0.49, respectively. Mean BMD for distal radius was 0.239 g/cm2. Seven patients had a marked BMD decrease, their femoral and/or lumbar Z scores were below -1. Four cases had a pH lower than 7.35. In five patients, a HCO3 level less than 19 mmol/l was detected, four of them had an augmentation. Chloride measurements were slightly increased in six patients and alkaline phosphatase levels in five cases. Reduced GFR values were detected in two patients. There were no significant difference in laboratory values, in percentile height, and weights, in BMDs of femur, vertebra, forearm nor were any differences noted in age/bone age ratios in patients with augmentation when compared with those who had no augmentation. We found varying alteration in bone mineral density and HCO3 levels in patients with bladder exstrophy. Patients with bladder exstrophy, with or without augmentation, may develop serious growth retardation. As much as 45% of them, regardless of presence of augmentation, have an osteopenia or osteoporosis. We found a considerable difference in percentiles of heights as well as bone ages in bladder exstrophy patients when compared with normal population. We recommend close follow up of children with bladder exstrophy for linear growth, development of osteopenia, and bone ages.

Adolescent↗
PubMed2006

An initial evaluation of pelvic floor function and quality of life of bladder exstrophy patients after ureterosigmoidostomy.

Classic bladder exstrophy is characterized by displaced pelvic floor musculature and significant skeletal and genitourinary defects. A paucity of data exist evaluating long-term pelvic floor function in exstrophy patients after ureterosigmoidostomy. This study is an initial attempt to evaluate the prevalence of urofecal incontinence, pelvic organ prolapse, and overall quality of life in patients who have had ureterosigmoidostomies. Fifty-two individuals who underwent ureterosigmoidostomy between 1937 and 1990 were identified through the Ureterosigmoidostomy Association and the Johns Hopkins bladder exstrophy database and mailed questionnaires approved by the Institutional Review Board (Johns Hopkins). Data were analyzed with SigmaStat 3.0 (SPSS, Inc., Chicago, IL). Eighty-three percent of the subjects responded, with a mean age of 44.4 years (range, 14-73 years) and mean of 40.9 years (range, 14-65 years) after ureterosigmoidostomy. Prevalence of daily urinary and fecal incontinence was 48% (n = 20) and 26% (n = 11), respectively, whereas the prevalence of weekly combined urofecal incontinence was 63% (n = 27). The incidence of pelvic organ prolapse in this cohort was 48% (n = 20). In these patients, a significant risk of urofecal incontinence and pelvic organ prolapse exists. Long-term follow-up studies are needed to understand the role of pelvic floor musculature in this complex birth defect.

Adolescent↗
PubMedDate not supplied

Pelvic osteotomy for bladder exstrophy.

Twenty-five patients with bladder exstrophy underwent pelvic osteotomy at or before initial bladder closure and anterior abdominal repair. Ten patients underwent bilateral iliac osteotomies before 1977. An alternative procedure consisting of bilateral superior pubic ramotomies has been used in 15 patients since 1977. In all patients, successful tension-free closure of the abdomen was achieved. Long-term follow-up of these two groups of patients shows no difference in the degree of pubic diastasis, which was asymptomatic. Posterior iliac osteotomy requires two additional incisions, longer operative time, and postoperative immobilization. We therefore recommend bilateral superior pubic ramotomy as an alternative procedure in initial reconstruction of the anterior abdomen in patients with bladder exstrophy.

Adolescent↗
PubMed1999

Bladder exstrophy: staged reconstruction.

The modern staged approach to bladder exstrophy reconstruction has undergone significant changes since it was first advocated by Jeffs and Cendron in the 1970s. Although varied surgical approaches have been tried in the bladder exstrophy condition, the staged approach to bladder exstrophy repair has withstood the test of time. Progress continues to be made in evaluating the outcome of older types of staged reconstruction, with continuing modification and improvements in the modern approach to staged reconstruction. This treatise will update the reader on recent advances in the treatment of bladder and cloacal exstrophy.

Bladder Exstrophy↗
PubMed1989

Anatomical basis of pelvic growth in bladder exstrophy.

Pelvic growth in bladder exstrophy was studied in 10 patients. Growth of their pelves in length as well as in width was similar to that found by Tanner. Meanwhile, the speed at which the diastasis of the pubic symphysis increases is variable. Before 5 years of age and during the end of growth it is fast, whereas it is slow between 5 and 10 years of age. There are no radiological abnormalities of hip joint orientation, and the acetabular angles are normal.

Acetabulum↗
PubMedDate not supplied

[Bladder exstrophy: a neonatal emergency. I: Functional reconstruction].

The goal of the treatment involving the children with exstrophy of the bladder must include urinary continence, protection of renal function, good reconstruction of the penis and a good quality of life. The staged approach (Jeff's protocol) includes bladder closure with penile lengthening, when necessary, in the first week of age, epispadias repair and finally bladder neck reconstruction with bilateral ureteral reimplantation. The authors present their experience on staged functional reconstruction of classical bladder exstrophy (40 patients). They had no dehiscences of the abdominal wall and bladder at the primary bladder closure, at any age, with or without bilateral iliac osteotomy. The final successful rate in the staged functional bladder reconstruction was about 70% and it is continuously improving. In the authors' opinion the successful initial bladder closure is perhaps the most important factor for obtaining a larger bladder more quickly and for achieving a high urinary continence; it must be performed in tre first days of life.

Adolescent↗
PubMed1997

Prostate-specific antigen in men born with bladder exstrophy.

OBJECTIVES: To determine serum levels of prostate-specific antigen (PSA) in men born with bladder exstrophy and to investigate the clinical utility of this test in the bladder exstrophy population. METHODS: Ten men aged 19 to 45 years with a history of bladder exstrophy underwent digital rectal examination and free and total serum PSA determinations. Immunohistochemistry for PSA and prostate-specific acid phosphatase (PSAP) was performed on archival prostate tissue from 1 man with bladder exstrophy. RESULTS: Free and total serum PSA levels for all patients were measurable and below the upper limit for established age-specific reference ranges for normal men. Both PSA and PSAP were detectable by immunohistochemistry in prostate epithelial cells of a man with bladder exstrophy. CONCLUSIONS: Men born with bladder exstrophy have detectable serum PSA levels. However, because prostate cancer in men with bladder exstrophy has not been reported, whether PSA will have a clinical role as a serum tumor marker for prostate cancer in this population remains to be determined. If so, the PSA reference range for men with the exstrophy condition will need to be defined. Because prostate histology in men with exstrophy resembles that of normal men, careful clinical monitoring for benign and malignant tumors should not be overlooked in this particular population.

Adult↗
PubMed1987

Epidemiology of bladder exstrophy and epispadias: a communication from the International Clearinghouse for Birth Defects Monitoring Systems.

A study of infants with bladder exstrophy or epispadias was based on data from ten malformation monitoring systems around the world. The material is derived from nearly 6.3 million births. The recorded prevalence at birth of bladder exstrophy was 3.3 per 100,000 births and of epispadias (without bladder exstrophy) 2.4 per 100,000. The recorded rates of bladder exstrophy did not vary between the monitoring systems, but the rates of epispadias did. Furthermore, nearly all registered infants with epispadias were males. The sex ratio for bladder exstrophy was 1.5:1. Perinatal deaths occurred mainly when other malformations were also present. There was an increased risk in this group of malformations in infants of women aged less than 20 years. At high parity (3+), an increased risk was observed for bladder exstrophy, but there was a decreased risk for epispadias. The birth weight distribution was shifted slightly more to the left in isolated bladder exstrophy than in isolated epispadias, but was considerably shifted to the left when other malformations existed with bladder exstrophy. Most infants with other malformations belonged to the cloacal exstrophy sequence. There was no definite time trend between 1970 and 1985 in the prevalence at birth of bladder exstrophy. The study demonstrates how data from different monitoring registries can be pooled to characterize a rare malformation.

Adult↗
PubMedDate not supplied

[Bladder exstrophy].

OBJECTIVE: To describe the procedures of bladder exstrophy closure, epispadias repair to achieve urinary control. The approaches to the failed exstrophy are also described. METHODS: Bladder closure soon after birth is performed with concomitant pelvic osteotomy. The different techniques are described. The Cantwell-Ransley technique is preferred for epispadias repair and the Young-Dees-Leadbetter procedure for bladder neck reconstruction. RESULTS: A success rate of 92% and a failure rate of 31% have been reported by different authors. CONCLUSION: To date there is no definitive solution in the treatment of bladder exstrophy. Continent urinary diversion may be warranted in some cases.

Bladder Exstrophy↗
PubMed1989

Long-term followup of 207 patients with bladder exstrophy: an evolution in treatment.

Between 1945 and 1985 a total of 207 patients (male-to-female ratio 2:1) was treated at our institution for exstrophy of the bladder. Primary anatomical bladder closure was performed in 137 patients, of whom 42 (31 per cent) eventually required urinary diversion. Primary urinary diversion was the initial treatment in 70 patients, including ureterosigmoidostomy in 40, ileal conduit in 17, colon conduit in 11 and cutaneous ureterostomy in 2. Secondary urinary diversion was necessary in 35 patients and included continent diversion in 7. Primary anatomical bladder closure, done within 72 hours of birth and followed by staged reconstruction of the bladder neck, was the most successful surgical regimen for the treatment of bladder exstrophy. Acceptable urinary continence was achieved in 82 per cent of our patients with this approach. However, multiple procedures often were required to achieve continence.

Adolescent↗
PubMed2001

[Bladder exstrophy in the adult: therapeutic features. Report of 3 cases].

Bladder exstrophy is a malformation which the incidence is estimated at 3.3 per 100,000 births. It is usually treated in the first hours of life. The authors report three cases of bladder exstrophy treated in adult life with continent urinary diversion such as Benchekroun continent ileocaecal bladder (CICB). After a literature review on the management of bladder exstrophy, they studied clinical and therapeutical particularities of the bladder exstrophy in adulthood.

Adult↗
PubMed2001

Gracilis muscle sling for select incontinent "bladder exstrophy cripples".

PURPOSE: Functional reconstruction of children born with bladder exstrophy results in a successful outcome in the majority. However, failed bladder neck repair, especially after multiple surgical attempts, compromises the quality of the tissues and, thus, limits surgical options. In these cases conventional sphincteroplasty and other continence procedures, such as rectus sheath sling or artificial urinary sphincters, are ill-advised due to ischemia and fibrosis of the bladder neck. MATERIALS AND METHODS: We transferred the gracilis muscle as a vascularized pedicle flap around the bladder neck in 5 children born with bladder exstrophy, of whom 3 were "exstrophy cripples" with multiple previous failed attempts at reconstruction, and 2 were older with failed prior attempts at bladder closure. In the latter 2 cases a 1-stage repair was performed, which included bladder augmentation, ureteroneocystotomy, epispadias repair and bladder neck wrap with gracilis muscle. The other 3 children underwent gracilis muscle wrap of the compromised bladder neck. RESULTS: All 5 children were dry between clean intermittent catheterization 2 to 13 years postoperatively. Complications included urinary tract infection in all 5 cases, stomal stenosis in 2, small bowel obstruction in 1 and bladder stones in 2. However, no complication was related to the gracilis muscle transfer. CONCLUSIONS: Vascularized gracilis muscle flap can be used to wrap around the compromised bladder neck of incontinent patients born with bladder exstrophy. The muscle appears to provide a leak proof, cough competent sling that increases bladder outlet resistance and, thus, provides dry intervals between catheterization. The long-term results of up to 13 years have been satisfactory.

Bladder Exstrophy↗