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PubMed1980

Renal function in continent patients after surgical closure of bladder exstrophy.

We assessed the upper urinary tracts and renal function in 22 children who had achieved continence after staged correction of bladder exstrophy. All patients had been followed for an average of 8 years after completion of the reconstructive operation. In 15 patients the upper urinary tract was normal and only 1 of the remaining 7 required surgical correction (transureteroureterostomy) of the residual anomaly. Renal function was normal in all children. We attribute these satisfactory results to careful selection of patients, improved surgical techniques and good postoperative care.

Adolescent↗
PubMed1989

[Bladder exstrophy and quality of life].

The primary objectives of operative management of exstrophy of the urinary bladder are to obtain secure closure of pelvic girdle and abdominal wall, unimpeded voiding, urinary continence, absence of reflux, preservation of renal function, functional and cosmetically acceptable external genitalia, and the absence of malignancy. These objectives cannot be fully achieved by only one of the established procedures such as functional bladder closure, internal or external urinary diversion and epispadias repair. Early individual and creative surgical management including changing concepts when needed and careful life-long followup are thought to provide the basis for a satisfying social adjustment and quality of life.

Adolescent↗
PubMed1993

[Functional reconstruction of urinary bladder exstrophy: long-term follow-up of urinary continence].

The author followed up a group of seven patients 5 to 29 years after functional reconstruction without augmentation on account of classical exstrophy of the urinary bladder. The plastic operation to ensure continence was performed in 5 boys and 2 girls at the age of two and a half to 23 months, in six cases by the Young-Dees' method and in one patient by Leadbetter's method. Posterior iliac osteotomy was performed in two and an antireflux plastic operation was performed in four patients. In one patient a disruption of the plastic operation of the abdominal wall occurred on the fourth day after reconstruction. Continence was excellent in five patients and satisfactory in two. Among the many factors for urinary continence a plastic operation of the posterior urethra and neck of the bladder is most important as it creates the appropriate length of the continence zone and the adequate pressure on closure. In the discussion the author emphasizes the advantage to start with the reconstruction of the abdominal wall and bladder during the neonatal period and to proceed in stages. The functional closure of the bladder and its neck with the posterior urethra should be implemented after a minimal capacity of 60 ml has been attained. Augmentation enterocystoplasty is indicated on account of urinary incontinence as a secondary operation if the function of the urethra and neck of the bladder is satisfactory and achieved capacity is less than 100 ml.(ABSTRACT TRUNCATED AT 250 WORDS)

Bladder Exstrophy↗
PubMed1999

Mental health, psychosocial functioning, and quality of life in patients with bladder exstrophy and epispadias - an overview.

Although there has been only limited clinical research on mental or psychosocial implications in patients with bladder exstrophy and epispadias, questions have been raised as to whether their life is of such questionable quality that a termination of pregnancy should be considered. A systematic overview of outcome studies published over the past three decades was carried out. In all, 1208 abstracts and 52 papers were read; only 10 (0.8%) papers focused on the mental or psychosocial outcome, but with diverse findings. However, most of the studies suffered from serious methodological deficiencies. Physical, mental, and psychosocial problems revealed in studies with reliable and valid instruments have clinical implications and underline the need for the further development of surgical and psychosocial interventions. Multicenter studies with a multimodal, prospective, and longitudinal design, based on semistructured interviews and specific questionnaires related to the disorder, are appropriate.

Abortion, Therapeutic↗
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↗
PubMed2004

A case of bladder exstrophy and thoracal intramedullary teratoma.

Exstrophic bladder and intramedullary teratomas are rare congenital anomalies. To the authors' knowledge, the coexistence of these 2 anomalies has not been reported previously. The authors report on a newborn with thoracal intramedullary teratoma and exstrophic bladder. The possible embryogenetic background also is discussed.

Abnormalities, Multiple↗
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↗