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.     

Reconstruction of the umbilicus in exstrophy

Reconstruction of the umbilicus has cosmetic and functional value in the surgical treatment of the child with bladder exstrophy. We report on 12 patients who underwent creation of a neoumbilicus as part of genitourinary reconstruction.     

Covered exstrophy: a rare variant

A case of covered exstrophy without sequestration of a bowel segment is reported. A 4-year-old female presented with dribbling of urine. Treatment to date has been simple excision of the covered membrane with functional closure of the bladder and bilateral posterior iliac osteotomies, with reconstruction of the bladder neck and genitalia to be performed at a later date. The embryogenesis of this rare variant, a review of the reported cases, and management options are discussed. Keyword Covered exstrophy. Exstrophy. Bladder variants     

Bladder hernias

The authors report 5 cases of bladder herniation through inguinal ring. All the patients were males aged between 52 and 72 years with a mean of 63 years. The clinic symptoms were non specific. In four cases the diagnosis was made by IVP. In the fifth case the bladder herniation was discovered after an incidental opening of the bladder during a right inguinal hernia repair. Four patients were operated on for replacement of the bladder and repair of the hernial ring; the procedure followed was Mac Vay technique in two cases, Shouldice in one case and placement of a Mersuture prosthesis in another case. The fifth patient was operated on because of major constraint related to his general conditions. Results was quite satisfactory in all operated cases with a mean control of 24 months.     

Bladder tumors]

Radiographs that must be repeated, which are commonly referred to as "repeats," represent additional, non-billable costs due to increased film, chemistry, and equipment use as well as increased personnel time. Furthermore, patients receive additional radiation exposure from repeats and must remain on the premises until the second exam is completed. Compounding the overt negative financial impact on the department is an increased burden on the waiting room and support staff, and a decrease in patient throughput. A continuous improvement team was assigned to develop an improved technique for monitoring and reducing the number of repeated radiographs in a subset of our radiology department. This paper presents a novel method of accurately measuring the repeat rate through the use of radiographic repeat labels. The labels remove the guesswork from repeat analysis and heighten the technologists' awareness of common problems. Additionally, the labels allow for detailed analysis of the cause of repeated radiographs, which can provide insight for determining remedial actions. Repeat analysis data from our institution acquired using the labels before and after implementing remedial actions are presented.     

Variations in the bladder exstrophy complex associated with large omphalocele

Two newborns with a large omphalocele associated with the bladder exstrophy complex are presented. In 1 case the omphalocele was combined with a musculoskeletal defect and imperforate anus with an anovulvar fistula. The other patient had a musculoskeletal deformity and a duplicated bladder with exstrophy. Surgical treatment was successful in both cases.     

Current urologic management of cloacal exstrophy: experience with 11 patients

PURPOSE: Since 1980 the authors have treated 12 infants with cloacal exstrophy (10 classical and 2 variants). Eleven patients had repair, and are all surviving. The initial phases of management that led to improved survival have previously been reported. Quality of life is now a major focus for the cloacal exstrophy patient. During the past 10 years, nine of the 11 patients had lower urinary tract reconstructive procedures. This review evaluates experience with reconstructive efforts to achieve bowel and bladder control and to improve the quality of life in this complex group of patients. METHODS: Through review of patient charts and by patient interviews, data were collected to evaluate the ability to provide urinary and bowel control. A continence score was applied to provide a measure of success: voluntary control, 3; control with an enema program or intermittent catheterization, 2; incontinence with a well-functioning stoma, 1; and incontinence without a stoma, 0. The best continence score is 6 (genitourinary and gastrointestinal). Surgical complications, urodynamic and metabolic sequelae of continent urinary diversion were reviewed. RESULTS: At the time of the authors' previous report, eight of 11 patients had a continence score of 2 or less. Currently, eight of 11 patients have a score of 3 or better (five with enteric stoma and continent urinary diversion, two with enema program and continent urinary diversion, and one with enema program and continent bladder). Urinary-diversion procedures have included two gastric augmentations and five gastric reservoirs, two of which have required subsequent bowel augmentation. Gastric augmentations carry a definite risk of metabolic problems with three of our patients demonstrating significant episodes of metabolic alkalosis. In addition, results of urodynamic monitoring suggests that gastric reservoirs may be less compliant than reservoirs formed using other bowel segments. CONCLUSIONS: Modern principles of continent urinary diversion have been successfully applied to the cloacal exstrophy patient further improving their quality of life. Use of gastric flaps with preservation of intestinal length has been central to urologic reconstructive efforts. Use of stomach alone for formation of urinary reservoirs may produce suboptimal compliance, and composite ileogastric construction should be considered if the gastric flap is of marginal size.     

Bladder cancer in Sweden

The survival of patients with bladder cancer has not improved significantly during the past decades in spite of new diagnostic methods and treatment modalities. This observation underlines the need for improved routines to ensure earlier detection of the disease by patients and doctors and thereby start the treatment sooner. The common finding of treatment failures in patients who have shown no sign of local recurrence but have undergone radical cystectomy indicates that subclinical metastases are primarily responsible for the poor outcome in most cases. This indicates that, in addition to radical surgery, effective chemotherapy is needed to counteract the systemic spread of the disease.     

Bladder pseudotumor in childhood

The application of frozen and radiation sterilized allogenic bone grafts for reconstructions in orthopaedic operations is described. Analysis of results of treatment of 1125 patients was performed. It was found that use of preserved bone allows to reduce the extend and duration of surgery. Nearly total substitution of grafts may be seen in 3 to 8 months after surgery.     

The choice of the method for the surgical treatment of bladder exstrophy

129 children with exstrophy of the bladder underwent primary surgical reconstruction according to G. A. Bairov. Morphofunctional findings in these children gave grounds for determination of three degrees of the bladder congenital defects. 39 patients had defect of the 1st degree, 53 of the 2nd and 37 of the 3d degree. The results of plastic reconstruction of the bladder with local tissue support the validity of such procedure only for patients with the congenital defect degree I. For degree II the benefit is relative. In degree III plastic surgery in contraindicated.     

Bladder outlet obstruction in women

While voiding dysfunction is relatively common in women, true bladder outlet obstruction is a rare condition and may be present in a misleading manner. To make an accurate diagnosis and tailor appropriate treatment, urodynamic investigation is required. However, even sophisticated urodynamic studies have limitations in predicting obstruction as a complication of anti-incontinence surgery and response to ureterolysis.     

Unusual cloacal exstrophy in a male neonate with a normal penis and urethra

El mouse is a mutant which has epileptic convulsions after tossing-up stimulations and has a hippocampal dysfunction. Platelet-derived growth factor B-chain homodimer has been reported to be a trophic factor of hippocampal neurons. We found that a recombinant PDGF-BB suppressed the convulsions of El mice in a dose-dependent manner. Furthermore, thrombin-treated mice manifested no convulsions, but thrombin receptor activating peptide-treated ones had convulsions. These findings suggest that an abnormality in PDGF-BB release may make El mice susceptible to tonic-clonic convulsions.     

Bladder tumor associated with phenacetin abuse

The association between phenacetin abuse and renal papillary necrosis is widely known. Recently, reports in the European literature indicated a possible correlation between phenacetin abuse and the development of transitional cell carcinoma of the renal pelvis and bladder. A case of transitional cell carcinoma associated with phenacetin abuse is presented. The pharmacology of phenacetin metabolites is discussed, and the world literature is reviewed. The data available warrant careful reappraisal of the ready availability of phenacetin.     

Evaluation of smooth muscle and collagen subtypes in normal newborns and those with bladder exstrophy

PURPOSE: Many patients who undergo bladder exstrophy closure as newborns, subsequent epispadias repair and later bladder neck reconstruction become completely continent yet complications can occur. After successful initial exstrophy closure and later epispadias repair some patients may fail to gain sufficient capacity for bladder neck reconstruction or satisfactory capacity and continence after bladder neck reconstruction. In an attempt to understand the pathogenesis of these failures we compared bladder biopsies from normal neonates and those with exstrophy. MATERIALS AND METHODS: Bladder biopsies obtained from the midline of the bladder wall just above the base of the trigone from 12 newborns with exstrophy were compared to bladder sections from 9 neonatal cadavers. All bladder specimens were stained with monoclonal antibodies against type I, III or IV collagen and a subset was further stained with Masson's trichrome to define the extracellular matrix. All specimens were then analyzed using a color digital image analysis system. RESULTS: At initial examination of the extracellular matrix there was an increase in the collagen-to-smooth muscle ratio from 0.38 in controls to 1.2 in newborns with exstrophy, comprising an increase in collagen and decrease in smooth muscle. The collagen component of the extracellular matrix was then further defined to quantitate the amount of each collagen type (I, III and IV) deposited. We then evaluated the ratio of collagen type-to-total collagen sampled. Compared to control bladders there was no statistical difference in the amount of type I or IV in the bladders of newborns with exstrophy at initial closure. However, there was a 3-fold increase in type III collagen (0.14 +/- 0.05 to 0.46 +/- 0.2%, p < 0.001) in the bladders of neonatal controls versus newborns with exstrophy. CONCLUSIONS: This alteration in collagen makeup may represent an earlier developmental stage of the exstrophy bladder at birth, which then remodels and changes after successful initial closure. Further studies are underway to examine the collagen composition of bladders at bladder neck reconstruction, failed closures and augmentation.