Male Gender Assignment of a Child with Aphallia and Associated Complex Urological Anomaly.

A 2-year-old male child presented to us with aphallia. At birth, he was passing urine from the anus and had undergone emergency colostomy and pyelostomy for urinary sepsis at 1 week of life. After a complete evaluation, the child underwent perineal urethrostomy and scrotal phalloplasty followed by buccal mucosal tube urethroplasty in the second stage, which was completed before the child started schooling.

Multiple failed closure of bladder in children with vesical exstrophy: Safety and efficacy of temporary ileal patch augmentation in assisting bladder closure.

OBJECTIVE: The surgical approach to small bladder template in exstrophy bladder is difficult. Previously, many of these children underwent ureterosigmoidostomy and in recent times, the trend is to do a delayed primary closure. We have used ileal patch as a temporary cover for these small bladders with a view to encourage bladder growth and early results are encouraging. MATERIALS AND METHODS: In five of the 45 children with bladder exstrophy managed by radical soft-tissue mobilization over 10 years, primary bladder closure was not possible due to repeated failed closures. A detubularized ileum was used to patch the bladder initially and after 4 months the patch was excised and bladder closure with sphincter repair was done in second stage. RESULTS: In five children (three girls and two boys) the mean age at initial bladder closure was 14 months and mean age at ileal patch was 22 months. In four patients, the bladder grew facilitating closure and in one patient it failed. There were no complications with the use of gut in patch. CONCLUSION: A temporary ileal patch seems promising in managing failed bladder closure in exstrophy patients. Long-term studies are needed before such a technique can be used in all patients with failed primary bladder closures.

Device-Associated Infection Rates in 20 Cities of India, Data Summary for 2004-2013: Findings of the International Nosocomial Infection Control Consortium.

OBJECTIVE: To report the International Nosocomial Infection Control Consortium surveillance data from 40 hospitals (20 cities) in India 2004-2013. METHODS: Surveillance using US National Healthcare Safety Network's criteria and definitions, and International Nosocomial Infection Control Consortium methodology. RESULTS: We collected data from 236,700 ICU patients for 970,713 bed-days Pooled device-associated healthcare-associated infection rates for adult and pediatric ICUs were 5.1 central line-associated bloodstream infections (CLABSIs)/1,000 central line-days, 9.4 cases of ventilator-associated pneumonia (VAPs)/1,000 mechanical ventilator-days, and 2.1 catheter-associated urinary tract infections/1,000 urinary catheter-days In neonatal ICUs (NICUs) pooled rates were 36.2 CLABSIs/1,000 central line-days and 1.9 VAPs/1,000 mechanical ventilator-days Extra length of stay in adult and pediatric ICUs was 9.5 for CLABSI, 9.1 for VAP, and 10.0 for catheter-associated urinary tract infections. Extra length of stay in NICUs was 14.7 for CLABSI and 38.7 for VAP Crude extra mortality was 16.3% for CLABSI, 22.7% for VAP, and 6.6% for catheter-associated urinary tract infections in adult and pediatric ICUs, and 1.2% for CLABSI and 8.3% for VAP in NICUs Pooled device use ratios were 0.21 for mechanical ventilator, 0.39 for central line, and 0.53 for urinary catheter in adult and pediatric ICUs; and 0.07 for mechanical ventilator and 0.06 for central line in NICUs. CONCLUSIONS: Despite a lower device use ratio in our ICUs, our device-associated healthcare-associated infection rates are higher than National Healthcare Safety Network, but lower than International Nosocomial Infection Control Consortium Report.

Pathogenesis of bladder exstrophy: A new hypothesis.

UNLABELLED: Classical bladder exstrophy affects 1 in 30 000 live births. Results of surgical treatment from different institutions employing various surgical techniques are not uniform, thus there is a need for a consensus on the best technique for bladder exstrophy repair. Surgical correction in bladder exstrophy would be more effective if the exact pathogenetic mechanism was deduced and the procedure was directed to correct the cause, which is responsible for the defect. The anatomy of exstrophy shows that the infraumbilical abdominal wall, the anterior wall of the bladder, and the urethra are split, with splayed out genitalia and musculature along with pubic diastasis. There is no tissue loss and hence embryological defect is unlikely to be the cause of bladder exstrophy. Thus there is a need to examine pathogenesis of bladder exstrophy. METHODS: A literature search was made of the various hypotheses for cause of bladder exstrophy, and attempts were made to propose a new hypothesis. The present hypothesis is also the basis for a technique of mobilization of pelvic musculature, done in two stages. RESULTS: The functional outcomes of 38 children with bladder exstrophy managed over a period of 10 years were reviewed. At a mean follow-up of 4.5 years (range 2.5-8 years), 82% of patients were functionally continent. CONCLUSIONS: The exact embryopathogenesis of bladder exstrophy is unknown. In this study a new hypothesis is proposed, with the aim of tailoring the surgical procedure to correct this defect. Bladder exstrophy epispadias complex (BEEC) is a deformative disruption occurring after embryogenic phase and pubic diastasis, and is central to exstrophy development. A working hypothesis can be formulated in line with our observation so that future experiments based this new hypothesis can aim to elucidate the exact pathogenesis.

Mobilization of pelvic musculature and its effect on continence in classical bladder exstrophy: a single-center experience of 38 exstrophy repairs.

UNLABELLED: Soft tissue mobilization of pelvic musculature in bladder exstrophy repair and its effect on continence. A single-center experience of 38 exstrophy repairs in children. INTRODUCTION/BACKGROUND: Bladder exstrophy is characterized by infra-umbilical abdominal wall defects, evaginated bladder plate of varying size, epispadias, abnormality of genitalia and bony pelvis. The goal of repair is to provide satisfactory continence, which should preferably be done in specialized centers dedicated to exstrophy management. The concept of functional reconstruction rather than urinary diversion is the gold standard worldwide, which can be accomplished by staged or one-stage procedures. Our technique of mobilization of pelvic musculature is based on the concept that continence in bladder exstrophy can be achieved by repairing the disorganized/splayed tissues involved in normal continence (as first advocated by J.H. Kelly) without osteotomy. OBJECTIVES: A systematic review of outcomes of neonatal bladder closure followed by mobilization of pelvic musculature in bladder exstrophy repair in children. STUDY DESIGN: A retrospective chart review of all exstrophy repairs conducted over a 10-year period (between 2001 and 2011). Repairs were done in two stages: bladder closure in the neonatal period (stage 1); and mobilization of pelvic musculature and epispadias repair (stage 2), preferably done between 4 and 6 months of age. The data on complications and continence were evaluated. RESULTS: Thirty-eight patients had completed all the stages of repair. Mean follow-up was 4.5 years (range 2.5-8 years). The following complications were noted: bladder dehiscence in eight patients after stage 1 repair, penopubic fistula occurred in four patients following stage 2 repair. Vulval scarring and vulval dehiscence (2 patients) were complications seen in girls. Twenty-four of the 38 patients (63.5%) achieved complete continence. Functional continence was attained by 31 of the 38 patients (82%). Older age at bladder closure affected continence, while the number of attempts at closure did not affect outcome. The age at pelvic mobilization was not a significant factor in outcome. DISCUSSION: The pelvic floor musculature and urethral sphincters are essential for voluntary control of micturition. In bladder exstrophy, these components are splayed out and our technique is based on reorganizing these components in the second stage. The voluntary urethral sphincter is a delicate complex of musculature located dorsal to the opened urethral plate and spread over the corpora. These are identified using a muscle stimulator and repaired around the tubularized urethra. Normally the levator ani muscle, by its attachment to the pubic bone, forms a loop, by which it compresses the urethra, providing additional aid in continence. In bladder exstrophy with wide pubic diastasis, this loop configuration is lost and becomes a hammock configuration and in fact becomes a pushing force. By mobilizing the pelvic musculature and repairing it in front of the bladder neck, this loop configuration is re-established and further aids in continence. After a successful second stage, patients may have increased frequency and dribbling initially, which improves with age as bladder capacity increases. Perineal exercises aimed at strengthening the pelvic floor musculature are an integral part of our bladder exstrophy management, which begins once the child can understand the technique. The results of our technique are shown in comparison with other series employing the original Kelly's technique (see Table). None of our patients have undergone additional bladder neck repair or permanent augmentation. CONCLUSIONS: Neonatal bladder closure followed by mobilization and repair of the pelvic musculature, produce satisfactory continence in exstrophic children. Proper identification and repair of the external sphincter and levatorplasty correct the altered anatomy by relocating the bladder neck and posterior urethra deep in the pelvis, simulating normal micturition. Early neonatal bladder closure improves outcome. The results are reproducible if basic principles governing continence are followed and when done in a specialized centers.

Posterior urethral valves and Cowper's syringocele: A rare association causing voiding dysfunction.

Urethral syringoceles are cystic dilatations of paired bulbourethral glands of Cowper. They can cause voiding dysfunction in male children and usually occur in isolation. We report a rare association of Cowper's syringocele with posterior urethral valves in a 4-year-old child, which was successfully managed endoscopically.