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Latex allergy in subjects who had undergone multiple surgical procedures for bladder exstrophy: relationship with clinical intervention and atopic diseases.

OBJECTIVE: To evaluate the prevalence of latex sensitization in a group of patients with bladder exstrophy, and to determine the role of associated risk factors, e.g. atopy, and the number and duration of surgical and anaesthetic procedures. PATIENTS AND METHODS: The study comprised 17 patients (15 children and two young adults) affected by bladder exstrophy who had undergone multiple surgical procedures. Skin-prick tests and specific immunoglobulin-E (IgE) assays against latex, food allergens cross-reacting with latex and inhalant allergens were carried out. RESULTS: Twelve patients showed latex sensitization and five showed symptoms related to latex exposure, the most common of which was contact urticaria (four of the symptomatic patients). Intraoperative anaphylactic reaction had led to life-threatening events in only one child. Latex-specific IgE determined both by the prick test and assay was positive in all those with symptoms. Specific IgEs against inhalant allergens and foods were present in four of five symptomatic patients. In the seven sensitized patients with no clinical symptoms, the assay was positive in all, while the skin-prick test was positive only in four; specific IgEs against inhalant allergens were present in three of the seven. In the five patients not allergic and not latex-sensitized, only one showed allergic sensitization against grass pollen and mite allergen. None of the children without latex antibodies had symptoms of latex allergy. Symptomatic patients had a undergone significantly more hours of surgery, more cystography and had used intermittent catheterization for longer than those with no symptoms. CONCLUSION: A third of patients with bladder exstrophy showed latex symptoms and another third had latex sensitization. Multiple surgical procedures and atopy play a major role in the development of latex hypersensitivity

Adolescent↗

Urinary continence after reconstruction of classical bladder exstrophy (73 cases)

OBJECTIVE: To evaluate urinary continence in children after reconstruction of classical bladder exstrophy. PATIENTS AND METHODS: Seventy-three children were treated between 1966 and 1990 at Debrousse Hospital in Lyon, France. Reconstruction involved a three-stage repair including bladder closure (with posterior iliac osteotomy), bladder neck reconstruction and penile reconstruction. RESULTS: Of 73 children with bladder exstrophy, seven underwent an initial urinary diversion and 66 a successful bladder closure at birth combined with posterior iliac osteotomy, 55 of whom underwent a bladder neck reconstruction and anti-reflux procedures. The Young-Dees procedure was performed in four cases, combined with the Jeff's technique in two cases and the Mollard technique in 49. Fifty-four of 55 (22 girls and 32 boys) were followed up for between 1 and 17 years. Results were classified as excellent (37 cases), good (11 cases) or failed (six cases), following assessment of continence, voiding capabilities, residual urine volume, urinary tract infection and urinary tract dilatation. Of these patients 69% had normal continence with transurethral voiding (girls, 77%; boys, 63%). Persistent incontinence after bladder neck reconstruction was related to insufficient outlet resistance, an abnormal bladder or a combination of the two. Repeated bladder neck reconstruction (seven patients), bladder augmentation (five patients), bladder neck suspension (one patient) and bladder neck reconstruction combined with augmentation (five patients) were performed. These operations were coupled with a Mitrofanoff procedure if further complications occurred. CONCLUSION: The outcome of bladder neck reconstruction was unpredictable. Achievement of a balance between intravesical pressure and outlet resistance was difficult and often required secondary endourethral procedures. Persistent incontinence after bladder neck reconstruction was related to insufficient outlet resistance, an abnormal bladder or a combination of the two. Repeated bladder neck reconstruction, bladder augmentation, bladder neck suspension and reconstruction combined with augmentation were performed. These operations were coupled with a Mitrofanoff procedure, which offered catheterization as a safe alternative if further complications occurred.

Bladder Exstrophy↗

Bladder exstrophy in the newborn: a snapshot of contemporary practice patterns.

OBJECTIVES: To use a large nationwide database to investigate and describe practice patterns in the contemporary management of bladder exstrophy. METHODS: The Healthcare Cost and Utilization Project Nationwide Inpatient Sample (1988 to 2000) was queried to identify infants with bladder exstrophy hospitalized during the first week after birth. Admission and disposition patterns, resource utilization and length of stay, surgical repair trends, and factors associated with in-hospital death were analyzed. RESULTS: We identified 426 hospital admissions of newborns with exstrophy. Most patients (75%) were transferred in from, or out to, other facilities; this was a fundamental feature of early exstrophy care. Racial differences were evident, with Hispanics less likely to be transferred (19% versus 60%, P = 0.001). Among newborns who were not transferred, many (46%) were discharged without bladder surgery. Surgical repair was usually done in a hospital other than the birth hospital; the mean hospital charges for surgery were 75,742 dollars. Of the 5 patients who died after repair, all had undergone surgery at "low-volume" hospitals. The length of stay did not change significantly during the study period, helping to keep resource utilization high in this population. CONCLUSIONS: The results of this study provide a "snapshot" of bladder exstrophy practice patterns during the newborn period between 1988 and 2000. Additional research should investigate whether newborns with exstrophy are receiving optimal care, including appropriate timing of surgery, equitable transfers to tertiary centers, and reconstruction at centers with adequate volume and experience.

Bladder Exstrophy↗

Distraction osteogenesis (callotasis) for pelvic closure in bladder exstrophy.

The success of urogenital reconstruction in bladder exstrophy is dependent on how successfully the pelvic ring can be closed. In patients with this disorder, the pubic bones are short and separated. A two-component plate-rod combination was designed to widen the pelvic ring using distraction osteogenesis (callotasis). After a middiagonal iliac osteotomy, two components of the implant are connected with two bolts passing through the iliac bone. After 10 days, the rods outside the skin are approximated gradually. When the pubic bones get close, they are fixed by heavy nonabsorbable sutures. The current study included 14 patients who were operated on between 1990 and 1996. The mean followup was 6 years. No neurologic or vascular complications developed. Successful tension-free closure of the abdominal wall was achieved in all but one patient. Urogenital reconstruction was done in the second stage. In all but one patient, the bladder was closed in one stage of urogenital reconstruction. In one patient (7%), a deep infection developed for which the patient needed early implant removal resulting in failure of the urogenital reconstruction. The described method addresses the pathoanatomy of the pelvic ring. The diameter of the pelvic ring can be widened and the pubic bones can be approximated without a significant increase in tension.

Abnormalities, Multiple↗

Bladder exstrophy and epispadias complex in sibling: case report and review of literature.

The bladder exstrophy and classic epispadias represent a spectrum of congenital anomalies with different degrees of anterior midline defect. Although the anomaly usually occurs sporadically there are some cases indicative of a strong genetic component. We present the clinical data of two siblings with bladder exstrophy and epispadias complex (BEEC), who were the product of consanguineous union. All previous reports of familial BEEC in the literature have been reviewed.

Abnormalities, Multiple↗

Early prenatal diagnosis of bladder exstrophy: case report and review of the literature.

Exstrophy of the urinary bladder is a rare congenital malformation. The first case of sonographic diagnosis, prior to viability, of bladder exstrophy without associated anomalies is presented. The diagnosis before viability allowed the parents to receive counseling on prognosis as well as possible options. This case is unique in that the bladder anomaly was an isolated defect, not associated with any other structural anomaly. The literature on the subject is also reviewed.

Abortion, Therapeutic↗

Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias.

OBJECTIVE: To review the long-term results of bladder neck reconstruction (BNR) in patients with classical bladder exstrophy or epispadias, and to review the concept of continence surgery in these two groups, stressing the difficulty in finding an adequate balance between urine storage (which implies high outlet resistance and low storage pressure) and complete bladder emptying (which implies low outlet resistance and a transient increase in bladder pressure); surgery cannot achieve 'continence' (which implies active mechanisms) but only 'dryness' (which implies passive mechanisms). PATIENTS AND METHODS: Eighty patients with classical bladder exstrophy (52 male, 28 female) and 25 with incontinent epispadias (17 male, 18 female) had their bladder neck reconstructed after a Young-Dees-Leadbetter procedure, subsequently modified by Mollard. The treatment is detailed and results reviewed after a mean follow-up of 11 years. All patients were treated and followed in the same institution. RESULTS: In the exstrophy group, 36 (45%) patients presented with a dry interval of > 3 h, with urethral emptying after one BNR; 52 (65%) presented with recurrent urinary tract infections, 19 (24%) with urinary stones, 21 (26%) with dilated upper urinary tracts, 13 (16%) with bladder perforations and one with an adenocarcinoma of the bladder. Thirty-eight patients (48%) required further surgery; 51% of all patients required an endoscopic procedure within 3 months after the BNR and 26% had endoscopic procedures for late (> 3 months) urine retention. In the epispadias group, 13 (52%) patients presented with a dry interval of > 3 h with urethral emptying after one BNR; 12 (48%) had recurrent urinary tract infections, five (20%) upper tract dilatation, two (8%) bladder stones, one (4%) bladder perforation and one an adenocarcinoma of the bowels after a ureterosigmoidostomy. Ten (40%) children required further surgery. CONCLUSION: We compared the present results for continence with those in other published series; most complications encountered were related to the obstructive pattern of bladder emptying and the abnormal bladder urodynamic behaviour caused by BNR. We consider that BNR is unpredictable and the roles of the other factors in urinary continence are discussed. Alternative procedures are detailed. The concept of continence surgery in exstrophy and incontinent epispadias is reviewed, stressing the importance of favouring bladder development and limiting obstructive patterns of bladder emptying that cause severe and recurrent complications.

Bladder Exstrophy↗

The outcome of patients with classic bladder exstrophy in adult life.

PURPOSE: We determined the outcome of 20 older adult bladder exstrophy patients regarding urinary continence, sexual function, fertility and psychosocial integration. MATERIALS AND METHODS: A total of 16 men and 4 women completed an anonymous questionnaire and the charts were reviewed for medical history. RESULTS: Of 9 patients who void spontaneously 6 are dry for 4 hours and 3 for 2 hours. Among the remaining 11 patients 5 are dry on clean intermittent catheterization, 3 who had undergone uterosigmoidostomy are dry for more than 4 hours and 3 had an incontinent stoma. Four men and 2 women are married, including 2 men and 1 women who had a total of 7 children. Ten of 16 men (63%) reported that they ejaculate a few cubic centimeters in volume, 3 ejaculate only a few drops and 3 have no ejaculation (2 of whom underwent cystectomy). Semen analyzed in 4 patients (3 with azoospermia and 1 with oligospermia) and average volume of ejaculate was 0.4 cc (range 0.2 to 1). All women reported regular and normal menstrual periods. A total of 15 patients experienced normal erections that were described as satisfactory by 8 and not satisfactory by 6 due to a small penis, with dorsal chordee in 1. Of the 16 men 12 (75%) experienced satisfactory orgasms while 10 had participated in sexual intercourse with complete partner satisfaction in 9. Half of the men and all women describe intimate relationships as serious and longterm. Of the 20 patients 15 (75%) achieved a high level of education. CONCLUSIONS: Bladder exstrophy patients generally achieve good results but the fertility of most men is in doubt.

Adolescent↗

Current management of bladder exstrophy.

This article describes in detail the modern management of the patient born with classic bladder exstrophy. Also, new techniques of prenatal diagnosis and pelvic fixation to secure the initial or secondary closure are discussed. Last, management of the failed closure and the bladder not suitable for closure are described and outlined. A successful initial closure of the infant born with bladder exstrophy is the single most important determinant in the eventual development of continence in the exstrophy patient.

Bladder Exstrophy↗

Growth and stretch response of human exstrophy bladder smooth muscle cells: molecular evidence of normal intrinsic function.

OBJECTIVE: To establish primary cultures of smooth muscle cells (SMC) from human exstrophic bladders (E-SMC), and determine their in vitro growth dynamics and responses to mechanical stretch. MATERIALS AND METHODS: Primary cultures of E-SMC from three patients were established from exstrophic bladder tissue using an explant method. Growth dynamics were assessed using tetrazolium-dye uptake. The DNA synthesis rate in response to cyclic stretch-relaxation was determined with thymidine-incorporation assays. Expression of the SMC mitogen heparin-binding epidermal growth factor-like growth factor (HB-EGF) mRNA in response to mechanical stretch was determined using semiquantitative reverse transcription-polymerase chain reaction. RESULTS: The approximate doubling time of the E-SMC grown in the presence of serum was 4 days, consistent with growth rates of SMC reported previously. E-SMC exposed to stretch had greater DNA synthesis, albeit to a lesser extent than previously seen with non-exstrophic SMC. The expression of HB-EGF was also increased in cells exposed to mechanical stimuli, consistent with our previous finding of stretch-regulated HB-EGF gene expression in bladder SMC. CONCLUSIONS: E-SMC had growth characteristics similar to those previously reported in non-exstrophic cells. E-SMC also had stretch-induced expression of HB-EGF mRNA. These observations provide evidence that despite development in an abnormal defunctionalized state, E-SMC retain the potential for normal growth, and may modulate this response through mechanisms similar to those operating in normal bladder SMC.

Bladder Exstrophy↗

Ureterosigmoidostomy: an outdated approach to bladder exstrophy?

Long-term results among 46 children with ureterosigmoidostomy are presented. The indication for ureterosigmoidostomy had been bladder exstrophy in 40 patients, incontinent epispadias in 5 and neurogenic bladder dysfunction in 1. Of the 40 patients with bladder exstrophy 8 had undergone ureterosigmoidostomy after failure of other types of urinary tract reconstruction (6 had upper tract dilatation before ureterosigmoidostomy). Three patients with previously damaged upper urinary tracts required early postoperative conversion because of severely increasing kidney dilatation. Three other patients required conversion after a mean of 10 years to preserve kidney function. One patient died after 16 years of a cause not related to ureterosigmoidostomy. The remaining 39 patients were alive with a functioning ureterosigmoidostomy after a mean followup of 14.7 years. The daytime continence rate was 97.4% (38 of 39 patients) and the complete continence rate was 92.3% (36 of 39). Except for 1 tubular adenoma that was removed successfully during routine colonoscopy, no bowel neoplasia has been observed. None of the 45 living patients has renal insufficiency.

Adolescent↗