Bladder bowel dysfunction in children with Down's syndrome.

INTRODUCTION: Bladder Bowel Dysfunction (BBD) has been described in patients with Down's Syndrome (DS). Our aim was to report the incidence, demographics, presentation, complications and management of the bladder in DS patients with BBD. METHODS: A systematic review was performed using PRISMA guidelines and search terms "{[(trisomy 21) OR down's syndrome]} AND [("non-neurogenic") OR voiding dysfunction]" in the search engines MEDLINE and SCOPUS. We also include a case series from two paediatric urology centres. RESULTS: A total of 38 patients with BBD and DS were included. Mean age was 12 years (newborn to 21 years), the male:female ratio was 2:1. Functional constipation (90%), recurrent urinary tract infections (38%) and enuresis were common at presentation (56%), while over 56% patients required surgical intervention. Medical treatment and behavioral modification were less successful while intermittent catheterisation did not work. CONCLUSION: This study reviews the largest cohort of patients with BBD in DS. It is common with serious consequences requiring operative intervention. Usual interventions are unreliable due to poor compliance. Early identification and management protect the renal tract. Regular screening for urogenital anomalies in DS is currently not performed. We recommend a thorough history of bladder function in DS patients to identify these cases early.

State of Play: Eight Decades of Surgery for Esophageal Atresia.

AIM: Surgical expertise and advances in technical equipment and perioperative management have led to enormous progress in survival and morbidity of patients with esophageal atresia (EA) in the last decades. We aimed to analyze the available literature on surgical outcome of EA for the past 80 years. MATERIALS AND METHODS: A PubMed literature search was conducted for the years 1944 to 2017 using the keywords "esophageal/oesophageal atresia," "outcome," "experience," "management," and "follow-up/follow up." Reports on long-gap EA only, non-English articles, case reports, and reviews without original patient data were excluded. We focused on mortality and rates of recurrent fistula, leakage, and stricture. RESULTS: Literature search identified 747 articles, 118 manuscripts met the inclusion criteria. The first open end-to-end anastomosis and fistula ligation was reported in 1941. Thoracoscopic fistula ligation and primary anastomosis was performed first in 2000. Reported mortality rate decreased from 100% before 1941 to 54% in 1950 to 1959, 28% in 1970 to 1979, 16% in 1990 to 1999, and 9% nowadays. Rates of recurrent fistula varied over time between 4 and 9%. Leakage rate remained stable between 11 and 16%. However, stricture rate increased from 25 to 38%. CONCLUSION: Including a full range of articles reflecting the heterogeneity of EA, mortality rate significantly decreased during the course of 80 years. Along with the decrease in mortality, there is a shift to the importance of major postoperative complications and long-term morbidity regardless of surgical technique.

A meta-analysis of the risk of boys with isolated cryptorchidism developing testicular cancer in later life.

BACKGROUND: Significant variability exists for the relative risk (RR) of testicular malignancy in isolated cryptorchidism. OBJECTIVE: To perform a meta-analysis to clarify the true magnitude of this risk, allowing clinicians to better counsel patients and their families. SETTING: Secondary research conducted by undergraduate researchers, clinical academics and a clinical statistician. DESIGN, DATA SOURCES, AND METHODS: A search of the English literature was performed for studies relating to testicular cancer and cryptorchidism, published between 1 January 1980 and 31 December 2010, using Embase and Medline databases. 735 papers were identified and analysed by four authors independently in accordance with our inclusion and exclusion criteria. Studies reporting an association between cryptorchidism and subsequent development of testicular malignancy were included. Genetic syndromes or other conditions which predisposed to the development of cryptorchidism were excluded. Pooled estimates and 95% CIs for the RRs were calculated. RESULTS: Nine case-control studies and three cohort studies were selected. The case-control studies included 2281 cases and 4811 controls. Cohort studies included 2 177 941 boys, with a total of 345 boys developing testicular cancer (total length of follow-up was 58 270 679 person-years). The pooled RR was 2.90 (95% CI 2.21 to 3.82) with significant heterogeneity (p<0.00001; I(2)=89%). CONCLUSION: Boys with isolated cryptorchidism are three times more likely to develop testicular cancer. The limitations of this study must be acknowledged, in particular, possible publication bias and the lack of high-quality evidence focusing on the risk of malignancy in boys with isolated cryptorchidism.