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PubMedDate not supplied

Hip function and gait in patients treated for bladder exstrophy.

Patients with bladder exstrophy demonstrate pubic diastasis with external rotation of the ilia and posterolateral orientation of the acetabula. Iliac osteotomy is performed to approximate the pubis and thus facilitate the genitourinary repair. The role of iliac osteotomy in the correction of gait abnormalities is controversial. Our goals in this study were to define the presence of hip pathology and gait abnormalities in patients treated for bladder exstrophy and to evaluate the effect of iliac osteotomy. We reviewed pelvic radiographs and gait studies in 15 children. The treatment included bilateral iliac osteotomy in nine of 15 of these patients. We found minor hip dysplasia in two patients, but we saw no evidence of subluxation or dislocation. Pubic diastasis increased with age and was not related to osteotomy. Patients had slightly less passive hip internal rotation than age-matched controls. There was no correlation between the restriction of hip internal rotation and the amount of pubic diastasis. Two thirds (10 of 15) of the patients exhibited increased lower extremity external rotation during gait. None of the subjects demonstrated a waddling gait (gluteus medius lurch) described in the literature. Based on our results, iliac osteotomy does not affect gait patterns in children with bladder exstrophy. However, we believe that it is indicated to improve the results of genitourinary repair.

Adolescent↗
PubMed2001

The Giessen-Mainz-Frankfurt procedure: a new method for complex pelvic reconstruction for bladder exstrophy.

PURPOSE: In bladder exstrophy primary reconstruction remains the gold standard worldwide. Despite various types of osteotomies the permanent correction of pubic diastasis remains a challenge. In maxillofacial surgery callus distraction is a routine treatment for hypoplastic mandibles. Originally described by Ilizarov, this method provides stable and true bone lengthening after gradual distraction of an osteotomy site as long as the periosteum remains intact. In cooperation with the departments of maxillofacial surgery and orthopedics we used this technique to correct pubic diastasis and facilitate phallic reconstruction in a 4 1/2-year-old boy with bladder exstrophy who had previously undergone continent diversion. MATERIALS AND METHODS: Three-dimensional computerized tomography was used to create a stereolithography model and mock surgery was performed. Based on this model bilateral osteotomies of the superior and inferior segments of the pubic bones were done, preserving the periosteum. Pins were inserted and a multidirectional external fixation device was mounted. Distraction was started on day 5 postoperatively. The distraction rate was 1 mm. daily and immobilization time was 28 days. The distraction progress was monitored by sonography. The device was removed 6 weeks postoperatively. RESULTS: Radiography of the pelvis 2 years postoperatively revealed that the distance between the pubic bones had decreased from 6 to 3 cm. (50%). Simultaneously the slanting angle normalized from 24 to 35 degrees due to upward rotation of the inferior pubic rami. Mineralization in the newly formed bones was excellent. Visible penile length had increased significantly. CONCLUSIONS: To our knowledge we describe the first use of the basic Ilizarov principle of callus distraction for permanent complex pelvic reconstruction for bladder exstrophy in a 4 1/2-year-old boy. After subperiostial osteotomies approximation of the symphysis and rotation of the inferior pubic rami were achieved with a device commonly used in maxillofacial surgery. Approximation of 1 mm. daily for 28 days resulted in significant penile lengthening. At a followup of 2 years there were stable pelvic ring reconstruction and normal mineralization of the newly formed bones. In contrast to the standard techniques of osteotomies for correcting pubic diastasis, the Giessen-Mainz-Frankfurt procedure provides true bone growth with a stable decrease in diastasis. Successful penile reconstruction was facilitated 1 year postoperatively. This method may also be useful in primary and secondary bladder reconstruction.

Bladder Exstrophy↗
PubMed2001

Anterior innominate osteotomy in repair of bladder exstrophy.

BACKGROUND: Classic bladder exstrophy is a developmental defect presenting at birth with a wide pubic separation and an exposed bladder; cloacal exstrophy involves, in addition, intestinal prolapse. Reconstruction requires several surgical procedures. The use of anterior iliac osteotomies in this process has not been reviewed in a large series. METHODS: We reviewed the results of eighty-six anterior innominate osteotomies performed in conjunction with genitourinary repair of classic and cloacal bladder exstrophy in eighty-two patients. Clinical outcome measures were successful bladder closure, achievement of continence, and maintenance of a normal gait. Radiographs of the pelvis were reviewed, and the pubic intersymphyseal diastasis (a measure of the reduction in tension on the anterior closure) was measured preoperatively and at three time-points postoperatively. Children with classic exstrophy who had undergone osteotomy and bladder neck reconstruction but not bladder augmentation were divided into four groups on the basis of the degree of continence. In addition, children with classic exstrophy were stratified according to age at the time of the osteotomy. The mean postoperative percent reduction in the amount of the original diastasis was determined for all groups. RESULTS: Children with classic exstrophy and those with cloacal exstrophy had correction of the diastasis after the osteotomy, with greater correction in those with classic exstrophy, presumably because of better bone quality. Daytime continence was achieved with anterior osteotomy and bladder neck reconstruction in 74% of the children for whom continence was a goal. However, no difference in the symphyseal diastasis or in the percentage of pubic reduction was detected among the four continence groups. Children who were older at the time of the osteotomy maintained better correction over time. Wound dehiscence or bladder prolapse occurred in 4% of the patients who had osteotomy and primary closure, and the only important complication of the osteotomies was transient palsy of the left femoral nerve in seven children. CONCLUSIONS: Anterior innominate osteotomy is an effective part of reconstructive repair of bladder exstrophy. The primary goals of the osteotomy are to reduce the tension in the closed bladder and the lower abdominal wall and to promote continence by restoring the sling of the pelvic floor muscles. These goals can be achieved in the majority of patients.

Adolescent↗
PubMed1999

Prenatal diagnosis of bladder exstrophy: what counseling?

PURPOSE: Bladder exstrophy has recently become one of the several congenital malformations detectable prenatally by fetal ultrasonography, which implies the necessity of a correct prognostic evaluation of quality of life on which therapeutic termination of pregnancy may depend within the temporal limits stated by the law. While maintaining a preeminently informative role, prenatal counseling has gained increasing relevance for the quality of life long-term prognosis in adults affected by bladder exstrophy, particularly in regard to urinary continence, and sexual and reproductive problems in male and female individuals. We report a case of bladder exstrophy diagnosed at 22 week of gestation, and comprehensively review the literature on prenatal diagnostic tools and procedures as well as long-term followup in such cases. MATERIALS AND METHODS: At week 22 of gestation a woman underwent prognostic counseling due to the diagnosis of fetal bladder exstrophy made by prenatal ultrasound. After counseling the parents elected pregnancy termination. In addition, literature data were analyzed in regard to quality of life and bladder exstrophy. RESULTS: We believe that the rarity of the prenatal diagnosis of this abnormality has resulted in the lack of a comprehensive review of necessary counseling criteria. CONCLUSIONS: We suggest what we consider to be a valid counseling approach for parents after a prenatal diagnosis of bladder exstrophy.

Adult↗
PubMed1998

Combined bladder closure and epispadias repair in the reconstruction of bladder exstrophy.

PURPOSE: Children with bladder exstrophy in whom initial closure fails are less likely to achieve adequate bladder capacity for later bladder neck reconstruction and continence. Repeat bladder closure may be combined with epispadias repair as initial management, decreasing the need for repeat anesthesia as well as providing increased outlet resistance to permit bladder growth in preparation for later bladder neck reconstruction. We compared results in boys who underwent combined bladder closure and epispadias repair or staged reconstruction. MATERIALS AND METHODS: A total of 16 boys with a mean age of 22 months with bladder exstrophy were referred for bladder closure after previous closure had failed in 15 and for delayed primary closure in 1. Osteotomies were performed in all patients, with bladder closure and epispadias repair as initial treatment. Results were compared to those in 21 boys with a mean age of 26 months in whom exstrophy closure had failed, and who underwent standard staged reconstruction by repeat closure followed by epispadias repair and subsequent bladder neck reconstruction in those who achieved adequate bladder capacity. RESULTS: At a mean followup of 87 months fistula rates were similar in the 2 groups. Eventual bladder neck reconstruction was possible in 69% of the patients in each group. In addition, 2 boys in the combined repair group achieved continence before bladder neck reconstruction was performed. CONCLUSIONS: We recommend epispadias repair combined with bladder exstrophy closure for treating failed exstrophy closure or late initial closure. Operative complications and results are comparable to those in patients in whom previous exstrophy closure failed and who undergo standard staged repair.

Abnormalities, Multiple↗
PubMed2003

New sonographic finding for the prenatal diagnosis of bladder exstrophy: a case report.

Bladder exstrophy is a very rare congenital malformation in which the anterior wall of the bladder is absent, and the posterior wall is exposed externally. Although sonographic findings of bladder exstrophy have been documented, we report a case presenting with a solid mass in the lower fetal abdomen with umbilical arteries running alongside the mass. The relationship between umbilical arteries and bladder exstrophy is a new sonographic finding which may be helpful in the prenatal diagnosis of this condition.

Abnormalities, Multiple↗
PubMed1994

Four cases of bladder exstrophy in two families.

Bladder exstrophy is a rare congenital anomaly, caused by abnormal development of the cloacal membrane. To our knowledge, 18 familial patients with this malformation have been described. Two sets of familial cases with bladder exstrophy are reported here: two cousins and a mother and son and the published reports of the 18 familial cases among 682 index patients with bladder exstrophy are reviewed. Ultrasonography is advocated as the investigation of choice for early prenatal diagnosis.

Bladder Exstrophy↗
PubMed2002

Augmentation colocystoplasty in bladder exstrophy.

A good bladder capacity and adequate outlet resistance determine success after staged reconstruction of bladder exstrophy (BE). Augmentation cystoplasty (AC) is an established salvage procedure to treat the small, noncompliant bladders of some of these children. In a series of 89 patients with BE treated over the last 12 years, 19 underwent detubularized augmentation colocystoplasty (ACC) as an adjunctive procedure. Nine underwent ACC at the time of bladder-neck reconstruction (BNR) for small bladder capacity and poor compliance; 10 underwent ACC as a secondary procedure after BNR for persistent urinary incontinence or poor bladder compliance and upper-tract deterioration. The follow-up period ranged between 6 months and 12 years (mean 41 months). Complications included symptomatic urinary-tract infection in 4 patients, recurrent epididymo-orchitis in 2, calculi in 3, colonic anastomotic dehiscence in 1, bladder-patch fistula in 2, and secondary coloureteric-junction obstruction in 1. There was no postoperative reservoir perforation or mortality. The upper tract remained normal or stable in all patients. Nine of the 19 patients are dry both day and night; 4 others are dry during the day but have occasional nocturnal wetting. Three patients have nocturnal wetting with stress incontinence and 2 remain incontinent. Seven patients can void effectively using abdominal contractions and 12 require clean intermittent catherization to ensure complete voiding. The indications and results of AC in BE from other series are reviewed. AC is an important and safe adjunctive procedure in a subset of BE patients with small and poorly compliant bladders. Despite the known complications, more liberal use of AC in BE is warranted.

Adolescent↗
PubMed2006

Surgery Insight: advantages and pitfalls of surgical techniques for the correction of bladder exstrophy.

Staged reconstruction repair of bladder exstrophy results in hydronephrosis or renal scarring in 15-25% of patients. A cosmetically acceptable and functional phallus can be achieved in 85% of patients, 20-30% of whom will require more than one operation for penile reconstruction. Episodes of penile glans loss or corporal loss are rarely reported with this technique. Widely disparate results relating to complete urinary continence and volitional voiding have been published, with urinary continence reported to occur in 7-85% of patients. The need for bladder augmentation to obtain urinary continence also varies, with reports that somewhere between 10% and 90% of patients require an augmentation procedure to gain urinary continence. Complete primary repair of bladder exstrophy using the penile disassembly technique results in hydronephrosis or renal scarring in 0-30% of patients, and hypospadias, as a consequence of this repair, will occur in 30-70% of patients. Loss of the glans and corpora appear more frequently with penile disassembly than in staged reconstruction of bladder exstrophy, however, the exact incidence of this complication is unknown. Reported complete urinary continence and volitional voiding rates are also varied following penile disassembly, ranging from 25-65%. A modified bladder-neck reconstruction to gain urinary continence is reportedly required in 15-90% of patients, with 5-10% requiring both bladder augmentation and bladder-neck reconstruction. Experience with complete primary repair of bladder exstrophy, using the penile disassembly approach, seems promising but is not a panacea. To outline the risks and benefits regarding the various surgical techniques for bladder exstrophy, we would recommend the establishment of a national registry for patients with this disorder.

Bladder Exstrophy↗
PubMed2004

A combined vertical and horizontal pelvic osteotomy approach for repair of bladder exstrophy: the Dana experience.

BACKGROUND: Bladder exstrophy is a severe congenital defect that requires a multidisciplinary treatment approach. Soft tissue repair may be successful during the first few days after birth, but a combination of pelvic osteotomies and bladder reconstruction is necessary later in life. The combination of externally fixed anterior and posterior osteotomies has biomechanical advantages over previous techniques for achieving primary bladder closure. OBJECTIVES: To describe our experience with a combined vertical and horizontal pelvic osteotomy approach for the repair of bladder exstrophy. METHODS: Four children underwent bladder exstrophy closure; the mean age at surgery was 19 months (range 9-33 months)... We stabilized the osteotomies with a small Synthes AO external fixator, 4.0 mm rod diameter. RESULTS: All four patients had successful bladder repair with no dehiscence; two of them achieved partial continence, and bladder neck reconstruction is planned for the other two. Three of the four patients sustained neurologic injury; two completely recovered, and the third continues to suffer from right drop foot. The average follow-up was 39 months (range 10-60 months). CONCLUSIONS: Vertical and horizontal pelvic osteotomies stabilized by external fixator and bladder repair is an effective treatment for bladder exstrophy.

Bladder Exstrophy↗
PubMed1985

Muscarinic cholinergic receptors in bladder exstrophy: insights into surgical management.

The surgical management of classical bladder exstrophy (functional bladder closure or urinary diversion) should be influenced by the inherent detrusor function of the exstrophied bladder. Cystometrograms performed previously on individuals with successful exstrophy closures demonstrate normal bladder function. The biochemical and neurophysiological properties of the exstrophied bladder have otherwise not been investigated. In this study radioligand receptor binding techniques were used to compare the density and equilibrium dissociation constant of muscarinic cholinergic receptors in control and exstrophy bladders. The density of muscarinic cholinergic receptors in the control and exstrophy groups was 1.97 plus or minus 0.29 and 1.44 plus or minus 0.21 fmol. per microgram deoxyribonucleic acid (mean plus or minus standard error of mean), respectively. The dissociation constant of the control and exstrophy groups was 0.15 plus or minus 0.02 and 0.14 plus or minus 0.02 nM. (mean plus or minus standard error of mean), respectively. These data show that the muscarinic receptor density and binding affinity in control and exstrophy bladders are similar. Therefore, the neurophysiological composition of the exstrophied bladder is not grossly altered during the anomalous development.

Bladder Exstrophy↗
PubMed2002

Connections of the bladder plate and bladder neck with the bony pelvis in a fetus with classic bladder exstrophy.

OBJECTIVES: To demonstrate that the bladder plate and bladder neck in classic bladder exstrophy are laying on smooth muscle fibers that extend laterally to the pubic bones. METHODS: We compared a male fetus of 28 weeks' gestational age with classic bladder exstrophy with a normal fetus of the same age. The specimens were divided into two parts by a midsagittal section, from the bladder neck to the membranous urethra. Thin transverse slices were also obtained on one part, and longitudinal slices on the other part. RESULTS: The smooth musculature of the bladder is normally differentiated cytologically and extends laterally to the bony structures of the pelvis. The musculature of the bladder neck and urethra are normally present compared with the control. CONCLUSIONS: The results of this study demonstrate the musculoskeletal organization of the urogenital system in classic bladder exstrophy. They also indicate that these structures should be clearly individualized and repaired in the reconstruction of classic bladder exstrophy.

Bladder Exstrophy↗
PubMed1999

Duplicate bladder exstrophy: a unique approach to initial repair.

OBJECTIVES: To describe a unique approach to the management of duplicate bladder exstrophy combining initial bladder closure and epispadias repair. Bladder exstrophy has been successfully managed by staged surgical repair with early bladder closure, subsequent epispadias repair, and, finally, bladder neck reconstruction. Duplicate bladder exstrophy is a rare variant of the exstrophy complex with fewer than 20 cases reported. METHODS: A male newborn presented with the appearance of both bladder exstrophy and a ruptured omphalocele. Repair of the omphalocele and bilateral orchiopexy was performed shortly after birth, but bladder closure was delayed until there was complete healing of the omphalocele defect, and the investigators believed the infant was ready for abdominal wall and bladder exstrophy closure. At age 8 months, bladder closure was performed with the intraoperative finding of a duplicate bladder lying posterior to the exstrophied bladder. The left ureter drained on the exstrophic bladder plate, and the right ureter drained into the posterior (internalized) duplicate bladder. After anterior innominate and vertical iliac osteotomies, the left ureter was reimplanted into the posterior bladder. A portion of the exstrophied bladder was then tubularized to construct a neourethra of the epispadic penis. RESULTS: The child has done well with an excellent cosmetic appearance of the abdominal wall and a straight phallus. The bladder subsequently required a Mitrofanoff-type continent stoma along with bladder augmentation, which was performed at the time of his Young-Dees-Leadbetter bladder neck reconstruction (age 3.5). The child is currently continent but requires intermittent clean catheterization through his appendicovesicostomy. CONCLUSIONS: The investigators report a unique and unexpected variant of bladder exstrophy and its successful management.

Bladder Exstrophy↗
PubMed1996

Bladder exstrophy from childhood into adult life.

Exstrophy of the bladder is rare and the incidence of bladder exstrophy is calculated to be from 1 per 30,000 to 50,000 live births with male to female ratio ranging from 1.5-5 to 1(1-4). It was found that persistence or overgrowth of the cloacal membrane on the lower anterior abdominal area, prevents normal mesenchymal ingrowth. This causes divergence of the lower abdominal muscular structures and forces the genital ridges to fuse caudal to the cloacal membrane. The stage of ingrowth of the urorectal septum at the time of rupture determines whether one will produce an exstrophic urinary tract alone (classic bladder exstrophy or epispadias) or cloacal exstrophy with the hindgut interposed between the hemibladders.

Adolescent↗
PubMed2005

Contemporary epidemiology of bladder exstrophy in the United States.

PURPOSE: Although bladder exstrophy is much discussed in the urology literature, there are few population based epidemiological data available for this rare condition. The purpose of this study was to use a large nationwide database to collect contemporary data on the incidence and demographics of bladder exstrophy. MATERIALS AND METHODS: The Healthcare Cost and Utilization Project Nationwide Inpatient Sample is a 20% sample of nonfederal United States hospitals containing data on 5 million to 7 million inpatient stays per year. The sample was limited to newborns, and International Classification of Disease-9 codes were used to identify cases of bladder exstrophy. We then determined nationally weighted incidence through time, and performed multivariate analyses to identify factors associated with exstrophy. RESULTS: We identified 205 patients with exstrophy among 9,452,110 newborns. The overall weighted incidence of exstrophy was 2.15 per 100,000 live births. The male-to-female ratio was almost even (OR 0.989, 95% CI 0.88 to 1.12). White infants were significantly more likely to present with exstrophy than nonwhites (incidence 2.63 vs 1.54 per 100,000, p <0.0001). Exstrophy incidence also varied by geographic region, socioeconomic status (SES) and insurance status. On multivariate analysis the racial variation in exstrophy incidence persisted even after adjustment for geographic region, SES and insurance status. Conditions such as spina bifida, cleft palate, preterm birth and gastrointestinal anomalies were more common in newborns with exstrophy. CONCLUSIONS: Bladder exstrophy is rare, occurs in equal numbers of live male and female newborns, and is associated with certain co-morbid conditions. Incidence appears to be stable through time. Nonwhite race, uninsured status, high or low SES and Western geographic region are associated with lower exstrophy incidence.

Bladder Exstrophy↗
PubMed1992

[Treatment of bladder exstrophy. Reconstruction or urinary diversion].

Bladder exstrophy is seen in 1 of 30,000-40,000 live births, and is seldom treated in many urological departments. Treatment options for children with exstrophy are upper urinary tract diversion or reconstruction of the bladder and plastic surgery of the bladder neck to gain urinary continence by the age of 4-7 years. Historical reviews report continence rates of 10-30% after a staged approach with primary reconstruction and secondary bladder neck repair. This formerly meant upper urinary tract diversion as a third stage in 70-90%. Multiple operative procedures could be avoided when primary diversion was done. The best results were reported following antirefluxive implantation of ureters into the sigmoid colon (ureterosigmoidostomy). In boys, the base of the bladder was removed, leaving a small residual bladder which together with the reconstructed epispadias served as a "seminal tract". Total removal of the bladder was performed in girls. Long-term follow up of upper urinary tract diversion showed disturbances of serum electrolytes, urinary tract infections and stone formation, and after ureterosigmoidostomies an increased rate of colon carcinomas was documented. These results led to renewed interest in reconstruction. The technique of bladder neck reconstruction was changed, resulting in a higher rate of late urinary continence: augmentation cystoplasties, clean intermittent catheterization and the artificial sphincter help to achieve a continence rate of more than 90%. This goal was reached only after multiple operations and without knowledge of the long-term sequelae of augmentation cystoplasties. The years to come will show whether new concepts of ureterosigmoidotomies, such as the sigma-rectum pouch, will be preferable, or a late urinary tract diversion after failed reconstruction. Most centers are now agreed that primary reconstruction of bladder exstrophy should be attempted in the newborn child.

Abdominal Muscles↗
PubMed1995

Criteria for the prenatal diagnosis of classic bladder exstrophy.

OBJECTIVE: To define ultrasonographic criteria for the prenatal diagnosis of classic bladder exstrophy. METHODS: Forty-three prenatal ultrasound scans were studied from 25 pregnancies in which live delivery of an infant with classic bladder exstrophy occurred. The diagnosis of bladder exstrophy could be made retrospectively in 29 prenatal studies from 17 pregnancies. The time of the fetal ultrasound varied from 14-36 weeks' gestation (mean 23). The diagnosis of bladder exstrophy was made before delivery in only three cases. RESULTS: Five factors associated with bladder exstrophy were identified: 1) The bladder was not visualized on ultrasound in 12 of 17 cases (71%); 2) a lower abdominal bulge representing the exstrophied bladder was seen in eight of 17 cases (47%); 3) a small penis with anteriorly displaced scrotum was identified in eight of 14 males (57%); 4) the umbilical insertion was low set in five of 17 cases (29%); and 5) abnormal widening of the iliac crests was seen in three of 17 cases (18%). CONCLUSION: The prenatal diagnosis of bladder exstrophy should be considered any time the bladder is not visualized or any of the aforementioned factors are noted.

Bladder Exstrophy↗