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At least 523 records · Page 29Linked to original sources
PubMed1980

Duplicate exstrophy of the bladder.

Duplicate exstrophy is a very rare variant of the exstrophy complex. Treatment is gratifying, because the patients have normal internal urogenital structures. Two different cases of duplicate exstrophy are presented. One is completely similar to the type, described by Marshall and Muecke, of which now 9 cases have been reported. It is suggested that the other, together with 4 earlier reported cases, belongs to a different type of duplicate exstrophy. The embryological mechanism responsible for this type is probably a coincidence of 1) duplication of cloacal structures and 2) exstrophy of one of the bladders.

Abdominal Muscles↗
PubMedDate not supplied

[Plastic repair of the isolated bladder in exstrophy in children].

Sigmoid colon segment was used to pass the urine through the intestine in 14 children with exstrophy of the bladder. Preoperative management of large intestine helped to reduce the number of operative stages from two to one. 12 patients were followed up from 1 to 3 yrs. Right ureterohydronephrosis of the upper urinary tract was observed in 1 patient. Urodynamic investigation of isolated sigmoid colon segment evidenced of its adequate contractile and peristaltic activity preventing the contact of feces with entero-ureteral anastomoses. To improve the closing ability of rectal sphincter in the above patients anal electrostimulation with diadynamic current can be recommended.

Anastomosis, Surgical↗
PubMed1999

Duplicate exstrophy of the bladder.

Duplicate exstrophy of the bladder is a rare, distinct congenital anomaly without additional major anomalies of the urinary tract. A 15-year-old boy with duplicate exstrophy is reported on, and the possible role of bladder sequestration in the development of classical musculoskeletal deformities in this anomaly is discussed.

Adolescent↗
PubMed2002

Influence of enterocystoplasty on linear growth in children with exstrophy.

PURPOSE: Many children with bladder exstrophy undergo reconstruction incorporating bowel into the lower urinary tract, which may result in metabolic changes affecting height. Linear growth in children with exstrophy who underwent enterocystoplasty was studied. MATERIALS AND METHODS: We retrospectively reviewed the charts of children with classic bladder exstrophy who underwent bladder augmentation with small or large bowel, or creation of a bowel neobladder before age 11 years. Mean followup plus or minus standard error was 9.4 +/- 0.9 years. Most patients were assessed yearly with measurement of height and serum electrolytes. Metabolic acidosis was treated with oral bicarbonate. Preoperative height percentiles at enterocystoplasty were compared to height percentiles at the most recent visit. In addition, height percentiles in a control group of individuals with exstrophy who did not undergo enterocystoplasty were compared to postoperative height percentiles in the enterocystoplasty group. The t test was used for statistical analysis. RESULTS: Data on 18 children who underwent bladder reconstruction with bowel were compared with those on a control group of 18 with exstrophy. The mean preoperative height percentile at a mean age of 5.2 years was 35.6 +/- 4.5 and the postoperative height percentile at a mean age of 14.6 years was 20.3 +/- 5.7 (p <0.01). The mean height percentile in the control group at a mean age of 15.2 years was 30.6 +/- 7.8 (p <0.01 versus the postoperative height percentile in the study group. In the enterocystoplasty group 2 patients were receiving oral bicarbonate for metabolic acidosis. Five patients who underwent enterocystoplasty and 6 controls were below the third percentile for height. CONCLUSIONS: In children with bladder exstrophy bladder augmentation or neobladder creation may have an adverse effect on linear growth. The height of children with bladder exstrophy is less than average compared with standard growth charts.

Adolescent↗
PubMed1980

Variants of the exstrophic complex.

In the split symphysis variant, a rare anomaly of bladder exstrophy, the bladder is closed with varying degrees of skin covering it. A good prognosis does exist for surgically produced continence in patients with a single bladder, although patients with bladder duplication have a greater risk of renal damage.

Bladder Exstrophy↗
PubMed2001

Pseudoexstrophy of the bladder: a rare variant.

A 15-day-old female presented with a healed omphalocele and a triangular musculoskeletal defect in the hypogastric area similar to the defect seen in classic bladder exstrophy. The bladder was normal on exploration. The defect was successfully closed using a bilateral anterior pubic ramotomy. Only ten cases of pseudoexstrophy have been reported in the world literature.

Abdominal Muscles↗
PubMed2004

Clinical and molecular characterization of the bladder exstrophy-epispadias complex: analysis of 232 families.

OBJECTIVE: To identify genetic and nongenetic factors contributing to the risk of bladder exstrophy-epispadias complex (BEEC). PATIENTS AND METHODS: In all, 285 families with BEEC were invited to participate in the study, and 232 of them were recruited. Epidemiological information was obtained from 151 of the consenting families, with a detailed clinical genetic examination of 94 probands. In all, 440 DNA samples were collected from 163 families for molecular analysis. RESULTS: Most of the cases were sporadic and had no family history of BEEC. Among patients, 95% were Caucasian, and males were more common in both the epispadias group (M/F, 2.2, 29 patients) and the classic bladder-exstrophy group (M/F 1.8, 164), but in the cloacal exstrophy group the sex ratio was close to unity (1.1, 15). There was a statistically significant association with advanced parental age (P < 0.001). Birth weight, gestational age and maternal reproductive history did not appear to be significantly different from those in the general population. Information on exposures to tobacco, alcohol and drugs was collected but none appeared to act as a risk factor. Karyotype analysis on 37 cases detected two chromosomal abnormalities, i.e. 46XY t(8;9)(p11.2; q13) and 47XYY. Molecular analysis of the HLXB9 gene, which causes Currarino syndrome, did not detect mutations in the blood or bladder DNA of 10 patients with bladder or cloacal exstrophy. CONCLUSIONS: BEEC most commonly occurs as an isolated sporadic birth defect with a recurrence risk of << 1%. There was no evidence of a single-gene effect or common environmental factor in this study population. In addition to race and advanced parental age, birth order may be a risk factor for BEEC. We suggest somatic mutations in a gene(s) within the pathway regulating bladder development may be the cause of BEEC.

Adolescent↗
PubMed2000

Total urogenital complex mobilization in female patients with exstrophy.

PURPOSE: Female bladder exstrophy/epispadias has traditionally been approached in a staged fashion. This approach results in a vagina that remains in an abnormal position on the anterior abdominal wall. We present a surgical correction of the female exstrophy/epispadias urogenital complex with total mobilization that returns the vagina to its proper anatomical position. MATERIALS AND METHODS: Since 1997, 7 female patients presenting with variants of the exstrophy/epispadias complex have undergone surgical repair using total urogenital complex mobilization. Of the patients 1 newborn and 2 school-age children had classic bladder exstrophy, 2 school-age children had cloacal exstrophy and 2 school-age children had primary epispadias. Total urogenital complex mobilization involved treatment of the urethra and vagina as a single unit. Complete disassembly of the pelvic diaphragm or floor anterior to the rectum was required to reposition the urethra and vagina to their proper anatomical positions in the perineum. The pelvic diaphragm was then reconstructed anterior to the urogenital complex to recapitulate the normal female pelvic floor anatomy. RESULTS: All patients have an anatomically correct position of the urogenital complex. All the vaginas reached the perineum without the need for skin flaps. All patients have adequate vaginal caliber without evidence of stenosis. CONCLUSIONS: The female with exstrophy/epispadias has unique anatomical defects in the urogenital complex that require special attention. Anterior displacement of the bladder, urethra and vagina with concomitant lack of development of the anterior pelvic floor musculature make a single stage, total urogenital complex mobilization repair ideal for this population. The results of this technique have been functionally and cosmetically pleasing. Whether repositioning the urogenital complex into the normal anatomical position will improve bladder dysfunction and urinary continence rates, and decrease or eliminate the need for future surgery will only be known after further long-term followup has been completed.

Bladder Exstrophy↗