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Adenocarcinoma at ureterosigmoidostomy junction in a renal transplant recipient 15 years after conversion to ileal conduit.

In recent years, adenocarcinoma of the colon mucosa has become a recognized complication of ureterosigmoidostomy and in most cases the tumor arises at the site of ureterocolonic anastomosis. We report a case of a 29-year-old renal transplant recipient who developed two colonic carcinomas at the site of ureterosigmoidostomy 25 years after the urinary diversion and 15 years after conversion to an ileal conduit. This case emphasizes the need for a careful life-long follow-up of all patients who undergo ureterosigmoidostomy.

Adenocarcinoma↗

Long-term results of ureterosigmoidostomy for ectopia vesicae.

Ureterosigmoidostomy was performed on 31 children with ectopia vesicae between 1941 and 1964. Two of the children died as a result of the operation and 25 of the remaining 29 were followed up in detail. Contrary to what is sometimes believed, the long-term results leave much to be desired, whether a Coffey or a Mathisen technique is used. Only four of the patients are perfectly normal, but here the end result is very good indeed. The remaining patients suffer from moderate to severe complications or are incontinent or both. It is essential that after ureterosigmoidostomy the patients are carefully followed up for the rest of their lives.

Adolescent↗

[Development of sigmoid carcinomas after ureterosigmoidostomy].

It is reported on a case of sigma carcinoma 15 years after ureterosigmoidostomy. A survey table contains the cases of sigma tumours after passing of the urine into the sigma hitherto reported in the literature of the world. Short discussion of frequency, latency period and diagnostic and therapeutic measures after ureterosigmoidostomy.

Adolescent↗

Prenatal diagnosis and management of abdominal wall defects.

The widespread use of fetal ultrasonography in routine antenatal care now allows the majority of abdominal wall defects to be identified prenatally, with subsequent opportunities for parental counseling, fetal intervention, and optimal perinatal management. Outcome is significantly affected by the presence of additional structural or chromosomal malformations; appropriate multidisciplinary counseling and management is dependent on the early identification of such anomalies in addition to accurate delineation of the abdominal wall defect itself. In cases with associated lethal or multiple severe abnormalities, parents may opt for elective termination of the pregnancy. Serial sonography is of particular value in gastroschisis, but there is little evidence that fetal manipulation or premature delivery confers any significant benefit. For all types of abdominal wall defect, optimal perinatal management is achieved in centers where neonatal medical, surgical, and anesthetic expertise is immediately available; cases diagnosed in units without these services should be considered for in utero transport to the nearest perinatal center.

Abdominal Muscles↗

Association of imperforate anus with short colon: a report of eight cases.

Eight cases of imperforate anus with short colon were seen in our hospital from April 1982 to December 1987. Five were boys, three were girls, and their ages ranged from 2 days to 2 years. The international literature about this kind of disease was reviewed. The disease's name, embryology, diagnoses, differential diagnosis, treatment, and prognosis are discussed herein. We suggest that this case, which was not combined with exstrophy of the bladder and/or intestine, be called association of imperforate anus with short colon (AIASC). Other cases, combined with exstrophy of the bladder and/or intestine could be called, association of imperforate anus with exstrophy splanchnica (AIAES). This distinction is necessary because each group differs in symptoms, signs, diagnosis, treatment, and prognosis.

Anal Canal↗

The place of Mitrofanoff neourethra in the repair of exstrophy-epispadias complex.

The authors present their experience with seven patients having an exstrophy-epispadias complex who had undergone Young-Dees-Leadbetter bladder neck reconstruction with an adjuvant Mitrofanoff neourethra. All the patients, but one, were male with a mean age of 7.4 +/- 3.9 years. Six of them had had previous primary closure of their exstrophied bladders accompanied by anterior diagonal iliac osteostomies, and one having pure epispadias had an incompetent bladder neck and a very low bladder capacity. Four of these patients had breakdown of their reconstructed bladder necks due to clean intermittent catheterization (CIC) via urethra. Ileocystoplasties were performed in all of the patients for their unacceptably low bladder capacities (mean 20.9 +/- 12.9 ml) with an adjuvant Mitrofanoff neourethra. The patients were put on CIC every 3-4 hours via Mitrofanoff channel. Six were totally continent, and one had to use a pad during daytime. There were no complications related to the Mitrofanoff stoma except two patients complained about temporary difficulty on catheterization. The authors conclude that incorporating a Mitrofanoff stoma to bladder neck reconstruction procedures in exstrophy-epispadias complex, prevents injury to the bladder neck during CIC, and thus lowers the risk and incidence of failure.

Bladder Exstrophy↗

Endourethral injection of bulking agents for urinary incontinence in children.

OBJECTIVE: To assess the early and late outcome of endourethral injection with bulking agents in children with urinary incontinence (a neuropathic bladder or exstrophy-epispadias complex), by reviewing our experience over a 5-year period. PATIENTS AND METHODS: The records of 15 children (10 boys) were reviewed retrospectively; 10 had spina bifida and a neurogenic bladder, four had a neurogenic bladder from other causes and one had epispadias. All children had a stable low-pressure detrusor and a compliant bladder with sphincteric weakness on preoperative urodynamic testing. Four children had undergone previous enterocystoplasty with a Mitrofanoff stoma, with concomitant urethral lengthening in two and a Goretex trade mark bladder neck sling in two. Three children voided spontaneously while 12 depended on intermittent catheterization. The agent was injected under general anaesthesia in all patients but one, with an endourethral submucosal injection of the bulking agent into four or more points at the junction of the bladder neck and proximal urethra, aiming to obtain visual occlusion of the urethra. The median (range) number of injections was 2 (1-3); five children had one injection, seven had two and three had three. There were no procedure-related complications and most were day-case procedures. Initially PTFE paste was used as the bulking agent, being replaced by bovine collagen or polydimethylsiloxane in the latter half of the series. RESULTS: At a median (range) follow-up of 28 (11-65) months three children were completely dry after a single injection; there was no change in four and a short-term improvement (median 25 months, range 4 days to 37 months) in eight. After this period all children deteriorated to their original incontinence grade; hence the overall cure rate was three of 15. CONCLUSION: This experience with a long-term follow-up differs from previously reported high success rates for the endourethral injection of bulking agents for urinary incontinence in children. Despite a short-term benefit, in the long-term this technique was unreliable and often ineffective. Patients and their carers should be given a realistic and guarded prognosis.

Bladder Exstrophy↗

Complete primary repair of exstrophy. Surgical technique.

The single-stage reconstructive approach to exstrophy evolved out of changes in the management of exstrophy. The success of Jeffs and others that functionally reconstructed the bladder of patients with exstrophy demonstrated that the approach was feasible and acceptable. Increased understanding of the anatomic pathology associated with exstrophy and epispadias resulted in the development of complete penile disassembly for epispadias and the extension of this technique to exstrophy as the complete primary repair technique described herein. The results using this technique are encouraging, leading to the recommendation for the procedure by other surgeons committed to the care of patients with exstrophy.

Bladder Exstrophy↗

Stones in the urinary bladder in children and young adults.

Bladder stones were diagnosed in 22 children and young adults at The Children's Hospital, Boston, from 1969 to 1985. One half of the patients were less than 12 years old (mean, 11.9 years). One or more lithogenic factors were implicated in all but two. The most common causes were the presence of an intravesical foreign body, infection with Proteus (a urea-splitting organism), exstrophy of the bladder, and the presence of intestinal mucosa in the urinary tract. The radiologic features of the stones were not specific, and no special techniques were needed to identify them. Heightened awareness of patients at risk will lead to prompt diagnosis and treatment.

Adult↗