The impact of trisomy 21 on epidemiology, management, and outcomes of congenital duodenal obstruction: a population-based study

Abstract: Purpose: Congenital duodenal obstruction (CDO) is associated with trisomy 21 (T21), or Down’s syndrome, in around a third of infants. The aim of this study was to explore the impact of T21 on the epidemiology, management, and outcomes of infants with CDO. Methods: Data were prospectively collected from specialist neonatal surgical centres in the United Kingdom over a 12 month period from March 2016 using established population-based methodology for all babies with CDO. Infants with T21 were compared to those without any chromosomal anomaly. Results: Of 102 infants with CDO that underwent operative repair, T21 was present in 33 [32% (95% CI 23–41%)] babies. Cardiac anomalies were more common in those with T21 compared to those without a chromosomal anomaly (91 vs 17%, p < 0.001), whereas associated gastrointestinal anomalies were less common in infants with T21 (3 vs 12%, p = 0.03). Surgical management was not influenced by T21. Time to achieve full enteral feed, need for repeat related surgery, and mortality were similar between groups. Infants with T21 had a longer median initial inpatient stay (23 vs 16.5 days, p = 0.02). Conclusions: Infants with T21 have a higher incidence of cardiac anomalies and a longer initial inpatient stay; however, it does not change CDO management or outcomes. This information is important for prenatal and postnatal counselling of parents of infants with CDO and T21

The impact of trisomy 21 on epidemiology, management, and outcomes of congenital duodenal obstruction: a population-based study

Abstract: Purpose: Congenital duodenal obstruction (CDO) is associated with trisomy 21 (T21), or Down’s syndrome, in around a third of infants. The aim of this study was to explore the impact of T21 on the epidemiology, management, and outcomes of infants with CDO. Methods: Data were prospectively collected from specialist neonatal surgical centres in the United Kingdom over a 12 month period from March 2016 using established population-based methodology for all babies with CDO. Infants with T21 were compared to those without any chromosomal anomaly. Results: Of 102 infants with CDO that underwent operative repair, T21 was present in 33 [32% (95% CI 23–41%)] babies. Cardiac anomalies were more common in those with T21 compared to those without a chromosomal anomaly (91 vs 17%, p < 0.001), whereas associated gastrointestinal anomalies were less common in infants with T21 (3 vs 12%, p = 0.03). Surgical management was not influenced by T21. Time to achieve full enteral feed, need for repeat related surgery, and mortality were similar between groups. Infants with T21 had a longer median initial inpatient stay (23 vs 16.5 days, p = 0.02). Conclusions: Infants with T21 have a higher incidence of cardiac anomalies and a longer initial inpatient stay; however, it does not change CDO management or outcomes. This information is important for prenatal and postnatal counselling of parents of infants with CDO and T21

Quality of Life after Emergency Hartmann’s Procedure: A Single Centre Retrospective Study

Background: Hartmann?s Procedure (HP) is the resection of the recto-sigmoid colon and creation of an end colostomy. Quality of Life (QoL) is increasingly considered before surgical intervention. This study assesses the patient?s perspective (QoL) before the HP, while the stoma is present and after reversal. Methods: Considered were patients who underwent emergency HP at our institution, between March 2014 and September 2017. The World Health Organization quality of life abbreviated questionnaire (WHOQOL-BREF) was used. An invitation letter and a copy of the questionnaire were sent to the patients, followed by a phone interview. Each patient completed the same questionnaire in relation to three time periods: Before Hartmann?s HP (BH), after Hartmann?s HP with Stoma Present (SP) and after Stoma Reversal (SR). Twice or 3 times, if the stoma was not reversed or was reversed. Results: Eighty-seven patients underwent emergency HP; 38 (43.7%) participated in the study. Fourteen (36.8%) patients had their stomas reversed. No difference in Qol, satisfaction with health, physical health, social relationships and environment between the periods BH vs. SP and BH vs. SR. There was an improvement from SP to SR. Psychological health scores were worse from BH to SP, but better from the SP to SR periods. Complications were the only predictor of outcome, except in psychological health and only after reversal of stoma. Conclusion: Emergency HP does not lower the general QoL but has a negative impact on psychological health. Stoma reversal engenders positive changes in QoL perception, while morbidity after stoma reversal predicts poorer outcomes.The article is available via Open Access. Click on the 'Additional link' above to access the full-text.Unknow

Early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study

Background: Contemporary early outcome data of meconium Ileus (MI) in cystic fibrosis (CF) are lacking on a population level. We describe these and explore factors associated with successful non-operative management. Methods: A prospective population-cohort study using an established surveillance system (BAPS-CASS) was conducted October 2012–September 2014. Live-born infants with bowel-obstruction from inspissated meconium in the terminal ileum and CF were reported. Data are described as median (interquartile range, IQR). Results: 56 infants were identified. 14/56(25%) had primary laparotomy (13/23 complicated MI, 1/33 simple), the remainder underwent contrast enema. Twelve, (12/33 (36%) with simple MI) achieved decompression. 8/12 (67%) who decompressed had &gt;1 enema vs 3/20 (15%) with simple MI who had laparotomy after enema. The number of enemas per infant (1–4), contrast agents and their concentration, were highly variable. Enterostomy was formed at 24/44(55%) of laparotomies. In infants with simple MI, time to full enteral feeds was 6 (2–10) days in those decompressing with enema vs 15 (9–19) days with laparotomy after enema. Case fatality was 4% (95% CI 0.4–12%). Two infants, both preterm died, both in the second month after birth. Conclusions: Infants with simple MI achieving successful enema decompression were more likely to have had repeat enemas than those who proceeded to laparotomy. Successful non-operative management was associated with a shorter time to full feeds. The early management of infants with MI is highly variable and not standardised across the UK and Ireland.</p