Bladder and urethral duplication and a bladder exstrophy plate with omphalocele in a female child.

The Exstrophy - Epispadias Complex (EEC) is a spectrum of rare congenital malformations involving the urinary, genital and musculoskeletal systems. We present an atypical or variant case of EEC in which a bladder plate is found involving a small omphalocele, separated pubic bones and bladder and urethral duplication. The treatment had a favorable outcome, with bladder control and the child voiding normally. Perhaps the best accepted embryonic theory to explain exstrophy and its variants should be reviewed as it fails to satisfactorily explain the alterations we found.

Penile necrosis and calciphylaxis.

Penile necrosis is an uncommon condition associated, in most cases, with calciphylaxis, also known as calcific uremic arteriolopathy. It is a progressive disease found in approximately 1%-4% of patients with end stage renal disease. Patients with penile calciphylaxis present a mortality rate of up to 70%, with life expectancy of two-and a half months following the diagnosis. We report a severe calciphylaxis case that had to be submitted to a penectomy but survived the penile event.

Spinal cord tumor leading to urinary retention resulting from Schistosoma mansoni infection in a child.

Schistosomose mansoni is an endemic disease in Brazil. It rarely affects the central nervous system, particularly in children. We report the case of a child that presented with an acute spinal cord compression condition, resulting from a mass that proved to be a granuloma caused by a Schistosoma mansoni infection. Proper treatment had no effect on the regression of urological symptoms and the child had to undergo a Mitrofanoff principle.

The XY female and SWYER syndrome.

SWYER syndrome or pure gonadal dysgenesis is a disease in which individuals with a female phenotype, with female external genital organs, have a 46 XY karyotype and streak gonads that ought to be removed given their high malignization potential. We present the case of a patient with Swyer syndrome, and compare them with other cases of patients with a 46 XY karyotype, phenotypically female, such as in Congenital Adrenal Hyperplasia from deficiency of the 17 α hydroxylase/17-20 Lyase enzyme and in the Congenital Androgenic Insensitivity Syndrome.

Acellular human glans extracellular matrix as a scaffold for tissue engineering: in vitro cell support and biocompatibility

ABSTRACT Objectives: Diseases of the genitourinary tract can lead to significant damage. Current reconstructive techniques are limited by tissue availability and compatibility. This study aims to assess if the decellularized human glans can be used as a biomaterial for penile reconstruction. Materials and Methods: Samples of the glans matrices were descellularized. We evaluate the presence of collagen type I and III, and elastic fibers. Biocompatibility assays were performed to assess the cytotoxic and non-cytotoxic interactions between the acellular matrix and 3T3 cells. The matrices were seeded with mesenchymal stem cells and were assessed for viability and integration of these cells. Biomechanical tests in native tissue, descellularized matrix and seeded matrix were performed to characterize their biomechanical properties. Results: The tissue architecture of the decellularized matrix of human glans was preserved as well as the maintenance of the biomechanical and biological properties. The analyzes of glans seeded with mesenchymal stem cells revealed the integration of these cells to the matrices, and its viability during two weeks "in vitro". Conclusion: The decellularization process did not alter the biological and biomechanical characteristics of the human glans. When these matrices were seeded they were able to maintain the cells integrity and vitality.

The use of mesenchymal stem cells in bladder augmentation.

PURPOSE: To compare integration of bladder acellular matrix (BAM) with the bladder when seeded with mesenchymal stem cells (MSC) and when MSC are injected intravenously (IV). METHODS: MSCs were isolated from bone marrow of EPM-1 Wistar male rats. Female rats were distributed into: Group A-BAM augmentation; Group B-BAM augmentation and MSCs IV administered; Group C-BAM-MSC seeded augmentation. Animals were killed on postoperative days 7, 14 and 28. Morphological analyses were performed using hematoxylin and eosin and Masson's trichrome, in addition to immunohistochemical staining with α-SMA and neurofilament for assessment of tissue repair. RNAm expression of the SRY gene was used to mark MSCs in the rats killed on postoperative day 28. RESULTS: The muscle layer was best repaired in Groups B and C. No difference in the repair of the urothelium in the animals in any of the three groups was found. Group B presented the smallest inflammatory reaction and the best neural repair on postoperative day 28. None of the animals examined had MSCs in their bladder graft. CONCLUSION: The MSCs were able to improve repair of the muscle layer and when injected intravenously, they were noted to initiate the neuronal regeneration process.

Integration of collagen matrices into the urethra when implanted as onlay graft.

OBJECTIVE: To assess the integration of decellularized heterologous collagen matrices into the urethra, when implanted with no cells or when seeded with autologous smooth muscle cells. MATERIALS AND METHODS: Eighteen New Zealand rabbits were randomly assigned to two groups: Group I (n = 9) - animals undergoing urethral segment resection with interposition of a patch of heterologous collagen matrix seeded with autologous smooth muscle cells; Group II (n = 9) - animals undergoing resection of a urethral segment with interposition of a decellularized heterologous collagen matrix patch. Two animals from each group were sacrificed on postoperative days seven, fourteen and twenty-eight; three animals from each group were sacrificed at the end of three postoperative months. At the end of the third month one animal from each group underwent urethroscopy for urethral integrity assessment and one animal from each group had its microcirculation image captured by a SDF device (Side-stream Dark Field - Microscan Analysis Software). One animal from each group in each euthanasia period underwent cystourethrography so as the urethra could be viewed at flow time. The matrices integration was assessed through histological examination using hematoxylin and eosin (H & E), Masson trichrome (MT), Picrosirius red and Von Willebrand staining. In a blind study with two pathologists all the slides were studied. RESULTS: The matrices whether seeded or not with autologous muscle cells were able to restore the architecture of the urethra, but were eliminated from the first week on, before incorporation. Microcirculation of the neourethra, at the end of the third month, showed the same characteristics as a normal urethra in both groups of animals. CONCLUSION: Natural heterologous matrices implanted in the urethra as onlay graft were not incorporated into its walls but were able to fully restore the cell architecture of the organ, regardless of being seeded or not with autologous muscle cells.

Integration of collagen matrices into the urethra when implanted as onlay graft

Objective To assess the integration of decellularized heterologous collagen matrices into the urethra, when implanted with no cells or when seeded with autologous smooth muscle cells. Materials and Methods Eighteen New Zealand rabbits were randomly assigned to two groups: Group I (n = 9) - animals undergoing urethral segment resection with interposition of a patch of heterologous collagen matrix seeded with autologous smooth muscle cells; Group II (n = 9) - animals undergoing resection of a urethral segment with interposition of a decellularized heterologous collagen matrix patch. Two animals from each group were sacrificed on postoperative days seven, fourteen and twenty-eight; three animals from each group were sacrificed at the end of three postoperative months. At the end of the third month one animal from each group underwent urethroscopy for urethral integrity assessment and one animal from each group had its microcirculation image captured by a SDF device (Side-stream Dark Field - Microscan Analysis Software). One animal from each group in each euthanasia period underwent cystourethrography so as the urethra could be viewed at flow time. The matrices integration was assessed through histological examination using hematoxylin and eosin (H&E), Masson trichrome (MT), Picrosirius red and Von Willebrand staining. In a blind study with two pathologists all the slides were studied. Results The matrices whether seeded or not with autologous muscle cells were able to restore the architecture of the urethra, but were eliminated from the first week on, before incorporation. Microcirculation of the neourethra, at the end of the third month, showed the same characteristics as a normal urethra in both groups of animals. Conclusion Natural heterologous matrices implanted in the urethra as onlay graft were not incorporated into its walls but were able to fully restore the ...

Congenital megaprepuce: a new alternative technique for surgical correction.

OBJECTIVE: To present a new alternative technique for surgical treatment of congenital megaprepuce. MATERIALS AND METHODS: From April 2004 to April 2006, five patients aged 2 to 5 years were treated using the new technique. The technique is described and illustrated. It differs from other techniques in that it takes into consideration the constant ballooning of the foreskin, which gives to the external genitalia the aspect of a penoscrotal transposition. Cosmetic and functional success were also assessed by a case review. RESULTS: After a follow-up of 1 to 3 years, all patients have normal voiding and a satisfactory cosmetic aspect. CONCLUSION: This new technique could be a useful alternative in treatment of the congenital megaprepuce.

Congenital megaprepuce: a new alternative technique for surgical correction

OBJECTIVE: To present a new alternative technique for surgical treatment of congenital megaprepuce. MATERIALS AND METHODS: From April 2004 to April 2006, five patients aged 2 to 5 years were treated using the new technique. The technique is described and illustrated. It differs from other techniques in that it takes into consideration the constant ballooning of the foreskin, which gives to the external genitalia the aspect of a penoscrotal transposition. Cosmetic and functional success were also assessed by a case review. RESULTS: After a follow-up of 1 to 3 years, all patients have normal voiding and a satisfactory cosmetic aspect. CONCLUSION: This new technique could be a useful alternative in treatment of the congenital megaprepuce.

Ectopic opening of the vas deferens into a Müllerian duct cyst.

Two consecutive cases of ectopic opening of the vas deferens into Müllerian duct cysts are presented, with descriptions of the presentation, physical examination, radiographic and ultrasonographic images, surgical findings and treatment undertaken. In both cases, ablation of the cyst and ligature of the vas deferens was the technique employed. Both patients are currently well, with normal urinary flow and no urinary tract infections, 3 years after surgery. Ectopic opening of the vas deferens into Müllerian duct cysts is a rare entity but should always be considered when a child presents with purulent urinary discharge and abdominal tumor. Surgery is the only treatment recommended.

Pulse-inversion US imaging of testicular ischemia: quantitative and qualitative analyses in a rabbit model.

PURPOSE: To quantitatively and qualitatively assess perfusion with pulse-inversion (PI) ultrasonography (US) in rabbit model of acute testicular ischemia. MATERIALS AND METHODS: Institutional animal care committee approval was obtained. After 35 rabbits underwent unilateral spermatic cord occlusion, testicular Doppler US and contrast material-enhanced PI imaging were performed. Enhancement data yielded perfusion measurements including mean value during the first 10 seconds, mean value over entire recorded replenishment curve, and curve slope during the first 5 seconds. Calculated perfusion ratios were compared with radiolabeled microsphere-derived perfusion ratios. Two readers assessed testicular perfusion as none, possible, or definite and relative perfusion as greater to the right testis than to the left, greater to the left testis than to the right, or as equal to both testes. With kappa statistics, interobserver agreement for all imaging methods was determined. Association between qualitative perfusion categories and radiolabeled microsphere-based perfusion measurements was assessed. Quantitative and qualitative determinations of relative perfusion were compared with radiolabeled microsphere-based measurements. RESULTS: Correlations between calculated and radiolabeled microsphere-based perfusion ratios were determined (r=0.49-0.64). Interobserver agreement for presence of perfusion was excellent (kappa=0.76), and that for relative perfusion assessment was good (kappa=0.55). Neither kappa value varied significantly with imaging method. The percentage of times a testis classified as having definite perfusion had greater perfusion as measured with radiolabeled microspheres than a testis classified as having no perfusion or possible perfusion was higher with PI imaging than with Doppler US (85%-98% vs 72%-89%). Identification of the testis with less perfusion was better with quantitative methods than with qualitative assessment of images by the readers (75%-79% vs 34%-60%, P<.004). CONCLUSION: PI imaging, compared with conventional Doppler US methods, provides superior assessment of perfusion in the setting of acute testicular ischemia.

Sacral ratio and fecal continence in children with anorectal malformations.

OBJECTIVE: To evaluate the sacral ratio (SR) in patients with an anorectal malformation (ARM) and verify whether it has predictive value for fecal continence. PATIENT AND METHODS: From January 1990 to April 2002, 42 patients (aged 3-14 years) with an ARM and having already been operated on were reassessed and enrolled in the study. Patients with solid or paste-like stools but no soiling were deemed continent, those with similar stool and episodes of soiling partially continent, and those with no sphincter control, incontinent. The fecal continence was always analysed by the same observer. Sacral radiographic images were reviewed and the SR calculated from anteroposterior and images in the lateral position. All images were obtained before surgery and analysed by the same observer, while a third analysed the results. Results were considered statistically significant at P < 0.001. RESULTS: Seventeen patients were deemed continent, seven partially continent and 18 incontinent; there was no significant difference in SR among the three groups. When fecal continence was analysed in relation to changes in the findings on computed tomography of the lumbosacral spine, patients with sacral agenesis had a significantly higher frequency of fecal incontinence than the others, and all had a SR below 'normal'. CONCLUSION: Although the SR was different in patients with sacral agenesis it was no different in continent, partially continent or incontinent patients, and thus it is of no practical value in identifying patients likely to have fecal incontinence.