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PubMed2001

Bilateral anterior pubic osteotomy in bladder exstrophy repair: report of increasing success.

Bilateral posterior iliac osteotomy is performed in most patients undergoing primary closure of an exstrophic bladder; the aims are to facilitate abdominal-wall closure, prevent postoperative wound dehiscene, and possibly, to achieve better urinary control in older age. A new technique, anterior pelvic osteotomy of the superior pubic ramus, seems to obtain tension-free symphysis approximation safely and quickly. We report our initial experience with this osteotomy. Five neonates, four males and one female from 1 to 4 days old, all underwent closure surgery for bladder exstrophy (BE) and subsequent bilateral osteotomy of the superior pubic ramus (SPRO). Postoperatively, Bryant's traction was applied. Tension-free, complete approximation of the symphysis and uncomplicated healing were achieved in all five cases without palsy of the obturator nerve or postoperative hemorrhage. Follow-up revealed partial rediastasis with a stable anterior pelvic ring. Tension-free closure and immobilization are important factors in both initial and subsequent closure of BE. Several osteotomy techniques are currently in use. SPRO presents numerous advantages, namely, ease and rapidity, minimal blood loss, and no requirement for an extra skin incision or need to turn the patient on the operating table. A certain degree of rediastasis with growth was subsequently observed: although undesirable, this complication is common to all osteotomy techniques. We believe that SPRO is a valid and uncomplicated method to facilitate BE closure.

Bladder Exstrophy↗
PubMed2002

Twin pregnancy achieved through TESE in an adult male exstrophy.

Bladder exstrophy is a rare anomaly, it compromises bladder functions, and in males it occurs with an impairment of reproductive functions, because of erectile and ejaculatory deficit. Advancements in the surgical treatment of bladder exstrophy have allowed an improvement of the bladder functions while spontaneous conception is still impaired. This is a case report of a pregnancy and subsequent birth of twins following testicular sperm extraction, on a man born with classical bladder exstrophy with infertility due to an ejaculation.

Adult↗
PubMed1993

Flaps for the repair of bladder exstrophy.

From April 1986 to March 1992, flaps were used in the surgical treatment of 17 patients with congenital bladder exstrophy. Eleven of these patients had had surgery in other hospitals but all failed. Using the flap method for the repair all of them had procured a functional bladder except one who could not have a complete control of urination. The longest follow-up period was 3 years, and the renal and bladder functions were completely normal. The immediate result in 14 patients was good in the control of urination right after the removal of the catheter. The follow-up results were good in 16 cases. The flaps used included 8 types of skin flaps, fasciae and vascular pedicles. Provided with the central vascular supply, the new series of procedures were suitable for the treatment of a variety of exstrophy deformities.

Adolescent↗
PubMed1997

[Surgical approach on rabbit embryos. A model trial of bladder exstrophy].

We describe herein a new model of surgical approach of an early mammalian embryo. Indeed the developmental mechanisms of numerous congenital anomalies, such as bladder exstrophy, remain obscure, and progresses in their knowledge must be achieved to propose better treatments. But up to now all the successful reports in experimental models of early produced malformations concerned birds or batracians, whose development is very different from human. We used the rabbit. Twenty-five time matted does were operated at 12.5 days of gestation. Out of their 247 embryos, 99 underwent a surgical procedure. Forty-eight were injured in order to produce an exstrophy. In 18 cases, the embryo extruded from the uterine cavity and could not be reintegrated, and 2 sacs were found empty. The remaining 31 were only exteriorized. Nine additional does received intravenous teratogen at 12.5 days of gestation. In the group of operated embryos we obtained 6 full-term fetuses, one of whom had a cloacal exstrophy. No exstrophy was noted among the intact embryos, neither in the 87 fetuses submitted to the teratogen. We conclude that: it is possible to operate on an early mammalian embryo, and to obtain further growth until term and that the exstrophy we observed in one case resulted from the surgical specific procedure.

Animals↗
PubMed1997

A comparison of methods of repairing the symphysis pubis in bladder exstrophy by tensile testing.

OBJECTIVE: To compare the efficacy of several fixation techniques in the reconstruction of diastasis of the symphysis pubis in bladder exstrophy. MATERIALS AND METHODS: The symphysis of 32 pelves removed from piglets about 1 month old were disrupted and repaired using one of eight methods. After repair, each pelvis was tested biomechanically for load-to-failure, stiffness and energy-to-failure. The various repair techniques were compared with one another and to a group of six pelves tested intact. RESULTS: Four of the methods tested, including a #2 nylon suture placed through bone in a horizontal mattress arrangement, several loops of #2 nylon suture tied around the pubes, Mersilene tape tied around the pubes, and Mitek G-II suture anchors placed into the superior pubic rami, showed the highest stiffness and load-to-failure. All methods were very weak compared with the intact symphysis; the best load-to-failure (#2 nylon horizontal mattress suture) was less than half of that for intact bone, and the best stiffness (mersilene tape) was less than one-third that for the intact symphysis. CONCLUSION: The repairs varied greatly in the variables tested and those which are most promising merit further investigation to assess methods of improving their performance.

Animals↗
PubMed1998

Pubic symphysis repair strength in simulated bladder exstrophy using a sheep model.

OBJECTIVES: To investigate the mechanical strength of various pubic symphysis suture material in a simulated animal model of neonatal bladder exstrophy. METHODS: Neonatal lamb pelves, which are the approximate size of neonatal human pelves, were used. Twenty-four neonatal lamb pelves were divided into four equal groups. A midline symphysotomy was made through the cartilaginous pubic symphysis in three groups and repaired using two figure-of-eight sutures (size 0) placed through the cartilaginous pubis using polypropylene, braided polyester, or polyglactin. The fourth group served as the control. The pelves were then tested to ultimate load in pure tension at a strain rate of 0.25 mm/s until failure. RESULTS: There was a highly significant difference between the intact specimens and the repaired specimens (P = 0.0008). For the repaired specimens, there was a significant difference in the ultimate load normalized by pubic height between those repaired with polypropylene and polyglactin (P = 0.025), but not for those repaired with polypropylene and braided polyester (P = 0.11) or braided polyester and polyglactin (P = 0.11). CONCLUSIONS: Braided resorbable sutures are recommended for pubic symphysis repair, because they have a lower tendency to cut out of the cartilage.

Animals↗
PubMed1990

[A rare case of bladder exstrophy with a completely malformed complex in a girl].

Report of the observation of a female newborn presenting a complex malformative set: distal anorectal malformation without fistula; bladder exstrophy without epispadias, with cutaneous bridge; congenital urethrovaginal fistula; malformative sexual ambiguity with bulky genital tubercle, whole urethra, urethral meatus on the apex of the genital tubercle, associated with a mid labial genital joint and a high genital orifice; unilateral multicystic kidney; vertebral abnormalities with supernumerary thoracolumbar vertebrae; no karyotype abnormalities and no familial pathological history; no endocrine abnormalities. The therapeutic multistage program permitted a good reconstruction of the different lesion, particularly with bladder functioning witch in hope a good continence with two years of follow-up. No similar case is reviewed in the literature.

Abnormalities, Multiple↗
PubMed1989

Current management of bladder exstrophy: a BAPS collective review from eight centres of 81 patients born between 1975 and 1985.

The British Association of Paediatric Surgeons (BAPS) agreed to collect information on bladder exstrophy from its members. Data were obtained on 81 patients from eight centres. Twenty-one patients had other congenital abnormalities. Three died within the first year of life, and one was lost to follow-up in the early neonatal period--leaving 77 for further analysis. Forty patients had bladder closure without permanent urinary diversion. Ten were able to stay dry for two hours or longer, and 16 were constantly wet. Of the remaining 14, some were too young to assess, while others were dry for from 30 minutes to two hours. Thirty-seven had undergone diversions; fourteen had ureterosigmoidostomy, nine underwent ileal loop conduit, nine had colon loop conduit, and five had cutaneous ureterostomy. Seventeen of these diversions had been carried out before the patients were 2 years of age.

Adolescent↗
PubMed1995

Prevalence and repair of inguinal hernias in children with bladder exstrophy.

PURPOSE: We delineated the prevalence, recurrence rates and optimal treatment of inguinal hernia in the exstrophy population. MATERIALS AND METHODS: Of 181 children with exstrophy followed at our hospital inguinal hernias developed in 121 (66.8%). RESULTS: In a 12-year period inguinal hernias developed in 81.8% of the boys and 10.5% of the girls. In 18.2% of the cases the hernia was repaired via a preperitoneal approach at the same time as exstrophy closure. The remaining patients underwent an inguinal operation. Most patients had a wide defect at the internal ring in addition to a patent processus vaginalis. The overall recurrence rate was 8.3%. The incidence of synchronous or asynchronous bilaterality was 81.8%. CONCLUSIONS: Children with bladder exstrophy should be carefully examined for inguinal hernias before bladder closure. If a unilateral hernia is present, the contralateral side should be explored. Careful preperitoneal repair should emphasize repair of the internal ring.

Adolescent↗
PubMed1998

Further delineation of the Opitz G/BBB syndrome: report of an infant with complex congenital heart disease and bladder exstrophy, and review of the literature.

The combination of complex congenital heart disease (double outlet right ventricle with pulmonary atresia, malalignment ventriculoseptal defect, right-sided aortic arch with left ductus arteriosus) and bladder exstrophy occurred in an infant with Opitz syndrome. Neither of these defects has previously been reported in association with Opitz syndrome. These malformations, which are midline defects, further characterize this syndrome as an impairment in midline development. The spectrum of congenital heart disease and genitourinary anomalies seen in Opitz syndrome is reviewed.

Abnormalities, Multiple↗
PubMedDate not supplied

Association of bladder exstrophy with congenital pathology of the hip and lumbosacral spine: a long-term follow-up study of 13 patients.

The authors evaluated, clinically and radiographically, 13 of 28 patients with bladder exstrophy treated at their institution between 1964 and 1982. All had been treated with bilateral iliac osteotomies and pelvic rami reapproximation to assist in urologic repair. At skeletal maturity, the diastasis had partially recurred and the patients had short stature and were living normal lives. Mild acetabular dysplasia and other pelvic abnormalities were common, as were abnormal radiographic findings in the lumbosacral spine. Despite these findings, most did not affect function. These results support the need for pelvic reconstruction for urologic repair, as well as the need for periodic radiographic evaluation of the pelvis and lumbosacral spine. If present, these have the potential to adversely affect function as an adult.

Abnormalities, Multiple↗
PubMed1993

Cosmetic reconstruction of the mons veneris and lower abdominal wall by skin expansion as the last stage of the surgical treatment of bladder exstrophy: a report of three cases.

The results of the correction of the medial scar depression and diastasis of the pubic hair of the mons veneris in three postpuberal female patients operated on for bladder exstrophy are reported. The reconstructive surgery is carried out using skin expanders. The expanded skin is utilized to create two dermoadipose flaps to fill the medial depression, to outline again the public hair areas, and to allow sutures without tension.

Abdominal Muscles↗
PubMed1980

[Bladder exstrophy. Transformation from an external diversion of the urine to an internal diversion].

It is possible to transform an external diversion (transcolic cutaneous ureterostomy) into an internal one (uretero-sigmoido-sigmoidostomy) in cases of bladder exstrophy which have not been reconstructed. This must not be done at too early an age (not before 7) and only under certain conditions : good renal function, normal upper urinary tract, no sigmoido-ureteral reflux good anal sphincter. Such an operation radically changes the life of the exstrophic who was previously handicapped by a collecting apparatus. One must assure a careful long term follow-up. The major details of technique and the results of 20 cases are described.

Adolescent↗
PubMed1999

Normal urodynamics in patients with bladder exstrophy: are they achievable?

PURPOSE: Urodynamic study was performed in patients with exstrophy to determine the effect of bladder neck reconstruction and the ability to achieve normal urodynamics following surgery. MATERIALS AND METHODS: A total of 30 exstrophy cases at different stages of reconstruction were retrospectively reviewed in terms of bladder capacity, compliance, stability and presence of detrusor contractions following urodynamic study. RESULTS: Bladder capacity increased from a third predicted volume for age to half after reconstruction. Approximately 80% of patients had compliant and stable bladders before bladder neck reconstruction. Following bladder neck reconstruction approximately half of the patients maintained normal compliance with a smaller number maintaining normal stability. A quarter of patients maintained normal filling dynamics following bladder neck reconstruction, and 19% maintained normal filling and voiding dynamics after reconstruction. CONCLUSIONS: The majority of closed exstrophy bladders have normal filling dynamics before bladder neck reconstruction. Compliance and stability are impaired following bladder neck reconstruction. Approximately 25% of patients with exstrophy may maintain normal detrusor function following reconstruction. However, less invasive alternatives to the Young-Dees-Leadbetter bladder neck reconstruction should be sought.

Adolescent↗
PubMed1985

Family-focused services for children with rare disorders, exemplified by bladder exstrophy. A Norwegian national health program.

Families of children with rare disorders have many experiences in common, as well as special problems related to the specific disorder. This paper presents Frambu Health Centre and the family-focused services offered to patients with bladder exstrophy during the first information and treatment course arranged for this group at the Centre. Main areas of concern as reported by the families are presented.

Adolescent↗
PubMed1988

Anatomic basis for the orthopedic treatment of bladder exstrophy: anatomic study of the sacrosciatic ligaments in the newborn.

Anatomic study of the pelvis of 30 fetuses and newborn children, yielded a precise relationship between the sacrotuberous ligaments and size of the gluteal region. At birth, the distance between the posterior superior iliac spine and the ischium is 40 mm. Closure of the anterior defect in bladder exstrophy sometimes requires an orthopedic surgical procedure. Bilateral posterior iliac osteotomy and section of these ligaments can be easily done by a single medial posterior approach herein described.

Bladder Exstrophy↗