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Ureterosigmoidostomy in bladder exstrophy.

Experience with ureterosigmoidostomy (US) using a reflux prevention technique in 38 of 48 cases of bladder exstrophy is reviewed. During a follow-up period of 14 years, the results were excellent as to morphological changes of the upper urinary tract, incontinence, psychological development and social behavior. Within their families the patients are considered healthy. Even in children with benign disease, US with reflux prevention performed during the second year of life is the operation of choice for urinary diversion. In the postoperative follow-up it is recommended that sigmoidoscopy be performed once a year in addition to routine controls of the upper urinary tract and metabolic balance.

Adolescent↗

Nephrogenic adenoma with bladder exstrophy and immunosuppression.

Nephrogenic adenoma, an unusual proliferative lesion, often occurs in association with chronic inflammation of the bladder and is rarely found in children. Two cases are reported, one occurring in a young child born with bladder exstrophy, and one in a young female, with both interstitial cystitis and chronic use of immunosuppressive therapy. We believe these are the first case reports of nephrogenic adenoma occurring in this specific clinical setting.

Adenoma↗

Pregnancy in women after repair of bladder exstrophy. Two case reports.

Improved techniques in urinary diversion operations have enabled a large number of patients with bladder exstrophy to achieve urinary control. They can live fairly normal lives and some have achieved pregnancy. Case reports of such exist in the literature. Two more cases are reported in this paper, the pregnancies and their outcome are discussed.

Adult↗

[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↗

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↗

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↗

[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↗

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↗

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↗

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↗

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↗

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↗

[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↗

[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↗

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↗

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↗

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↗