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PubMed1994

[One stage reconstruction of bladder exstrophy and epispadias with abdominal wall skin flap and rectus abdominis muscle flap: report of 5 cases].

5 cases of complete bladder exstrophy were treated by one stage reconstruction with abdominal wall skin flap and rectus abdominis muscle flap. Closure of the abdominal wall and bladder defect were achieved satisfactorily in all the patients. 4 patients have been followed up for 1.5-10.5 years. 3 patients whose bladder neck had been augmented by rectus abdominis muscle flap were continent but 1 patient whose bladder neck had not been, had giggle incontinence. The renal functions were all normal in these patients.

Adult↗
PubMed1988

Primary closure of bladder exstrophy: long-term functional results in 137 patients.

Between 1945 and 1985, 207 patients were treated at our institution for exstrophy of the urinary bladder. Primary anatomical bladder closure was performed in 137 patients. In 97 patients treated prior to 1975, bladder closure was performed at a median age of 1 year and continence in this group was only 16%. Forty patients treated after 1975 underwent closure at a median age of 72 hours followed by staged reconstruction of the bladder neck. Continence in this group was 82%; however, the average number of surgical procedures was five as compared with two in the first group. Early bladder closure and staged reconstruction can achieve acceptable urinary continence, but multiple surgical procedures may be required.

Bladder Exstrophy↗
PubMed1995

Epidermolysis bullosa junctionalis associated with urinary bladder exstrophy: a case report.

We report the second infant of nonconsanguineous parents with epidermolysis bullosa junctionalis associated with urinary bladder exstrophy, epispadias, anteriorized anus, and bilateral inguinal hernias. The family history also included the death of a maternal cousin due to epidermolysis bullosa. Our diagnosis was based on electron microscopy and immunofluorescence evidence. This patient is reported because of the rarity of this constellation of findings.

Abnormalities, Multiple↗
PubMed1986

[Experience with reconstructive-plastic operations in bladder exstrophy in children].

The author analyzes the experience with reconstructive-plastic operations in her modification mad in 22 children with exstrophy of the urinary bladder. Long-term results within 3-17 years were observed in 15 children. Good outcomes (complete retaining of urine, normal capacity of the bladder, good morphofunctional state of the kidneys) were noted in 8 children. Based on the results obtained the author recommends the surgical treatment of exstrophy of the urinary bladder to be started with reconstructive-plastic operations at the age of 1-3 years.

Bladder Exstrophy↗
PubMed2000

Results of umbilicoplasty for bladder exstrophy.

PURPOSE: The umbilicus is an important aesthetic landmark and its absence or deformity may be associated with poor self-image. In patients born with bladder exstrophy the umbilicus is attached to the upper margin of the bladder and reconstructive surgery often removes the navel. The umbilicus marks the waistline and serves to complete the harmony of the curved lines above and below the waist. We present our experience with children born with exstrophic anomalies during the last 2 decades. MATERIALS AND METHODS: Our database included 61 children born with classic bladder and 8 born with cloacal exstrophy treated between 1980 and 1998. We performed primary reconstruction in 35 children, while 34 children and young adults were referred for secondary surgical repair, including bladder augmentation, continent diversion, genitoplasty and so forth. Neoumbilicoplasty was done in all of the former and in 30 of the 34 latter cases. Early in the series a V-shaped flap was raised and buried subcutaneously. The flap eventually became a tube around the cystotomy tube and the cicatrix formed the umbilical dimple. This method necessitated packing with iodoform gauze for 4 weeks with weekly dressing. The technique evolved into a tubularized U-shaped flap. A rubber tube was placed indwelling as a stent to maintain inward projection of the neoumbilicus. RESULTS: In 66 of the 69 cases the early results of umbilicoplasty were described by the surgeon as excellent or satisfactory. In 3 cases the neoumbilicus appeared flat, lost depth and was described as unsatisfactory. Long-term followup of more than 1 year was available in 48 patients, of whom 2 underwent umbilical repositioning for an off center or low umbilicus and 3 underwent repeat umbilicoplasty for a flat umbilicus that had lost depth. The best cosmetic results were achieved in patients with a relatively thick layer of subcutaneous fat, whereas cosmesis was suboptimal in thin children. Nevertheless, the patients and parents were generally pleased with the umbilical appearance even when the surgeon was not. CONCLUSIONS: Although the navel is a functionless depressed scar, it represents an important and pleasing landmark. Umbilical construction should be attempted early during functional closure or urinary diversion.

Adolescent↗
PubMed2002

Complete bladder exstrophy with a normal phallus: A variant of superior vesical fissure.

Variations in the anatomic defects of the bladder exstrophy are well recognized, but their incidence is extremely low. Here the authors describe a rare case of superior vesical fissure in which a relatively large defect caused the whole bladder to prolapse outside. A review of literature found only 2 other cases that resembled our case. This particular variation of exstrophy is not only important owing to its extreme rarity but also raises a question for an embryologic explanation.

Bladder Exstrophy↗
PubMed1992

20 years of functional reconstructive surgery in bladder exstrophy--balance 10 years after a preliminary report.

This is an update of a consecutive series of 19 patients with bladder exstrophy presented as a preliminary report 10 years ago with a follow-up of 6 years. The average time of follow-up now is 14 years. In difference to the former report several additional procedures have been performed in most patients. 3 early "good" patients stay "good and continent". Only 3 out of 5 patients categorized as "fair" are now "good and continent" and only one without further intervention. 3 out of 6 patients who were classified as "poor" are now diverted. The overall rate of secondary diversions is 40% including 2 patients with ureterosigmoidostomies. All patients have normal renal function and normal blood pressures. These results show that an early "good" result generally remains "good" but additional surgery may be warranted. The initially "fair" results usually necessitate a secondary procedure, e.g. augmentation cystoplasty or/and bladder neck tightening to show an improvement. Patients with early "poor" results unfortunately remain "poor" and have a great chance to get diverted.

Bladder Exstrophy↗
PubMed2001

Bladder exstrophy: gynecological and obstetrical characteristics with reference to three cases.

The authors report three cases of pregnancy in women treated for bladder exstrophy. Based on a review of the literature, and the follow-up of these cases, the aim of this study was to determine the prognosis of pregnancy, which is currently possible due to the progress in antibiotherapy and surgery. Nevertheless, these pregnancies need to be carefully followed-up, not only because of the complications that may occur to the mother and the infant, but also because of the type of delivery involved.

Bladder Exstrophy↗
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↗