Tissue Shrinkage of Resected Specimens in Hirschsprung's Disease: Why Pediatric Surgeons Think the Bowel Specimen was Longer Than Indicated in the Pathology Report.

BACKGROUND: Hirschsprung disease (HD) is an aganglionosis of variable length starting at the rectosigmoid colon with surgery as sole therapeutic option. The length of the resected bowel segment is a crucial information for the treating surgeons and influences the prognosis of the patient. It is often artificially altered due to post operative tissue shrinkage. The objective of this study is to quantify the extent tissue shrinkage of HD specimens. MATERIAL AND METHODS: Colorectal HD specimens were measured at the time of surgery and at the time of cut-up, either fresh or after formalin fixation and statistically analyzed. RESULTS: Sixteen colorectal specimens were included. Following formalin fixation the specimen length decreased by 22.7% (P < .001). Without formalin fixation the specimens shrank by an average of 24.9% (P = .05). There was no significant difference in the extent of tissue shrinkage with or without formalin fixation (P = .76). CONCLUSION: This study showed that there is significant tissue shrinkage in HD specimens. The 2 different cohorts revealed that tissue shrinkage is mostly caused by tissue retraction/alteration after organ removal but also to a lesser extent by fixation with formalin. Surgeons and (neuro-)pathologists should be aware of the sizeable shrinking artifact to avoid unnecessary confusion.

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 >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.

"An invisible map" - maternal perceptions of hunger, satiation and 'enough' in the context of baby led and traditional complementary feeding practices.

Mothers' responsiveness to hunger and fullness cues has been implicated in the development of infant overweight, and baby led weaning (BLW) is argued to be one way to protect against overfeeding. Whilst studies have examined maternal perceptions of hunger, fullness and adequate intake to some degree in traditional weaning (TW) contexts, less is known about this in BLW. This study therefore aimed to understand and compare maternal perceptions of cues and intake in BLW and TW. Eleven mothers of infants (7-24m) participated in semi-structured interviews based on discussions of short videos featuring participants feeding their infants. Interviews were read and transcribed in full. Data were selected for coding which addressed mothers' perceptions of infant hunger, fullness and sufficient consumption and subsequently subjected to template analysis. A sample of data was coded to produce an initial template which was applied to all interviews and revised in an iterative process to produce a final template for interpreting findings. Mothers in the study were adept at recognising fullness cues and gauging feeding state. Both groups perceived similar hunger cues although TW mothers reported a wider range of fullness cues. Both groups used numerous strategies for judging the adequacy of their babies' intake. These included the use of infant cues, however perceived adequacy of intake was also influenced by factors such as infant tiredness and maternal worries about over and under-eating. Findings have implications for the development of responsive feeding interventions while also highlighting the utility of video elicited interviews for understanding feeding interactions.

Reading Appetite Cues in Infancy: A Role for Nutrition Education.

Infants communicate complex aspects of appetite through a variety of behaviors. In newborn babies, mouthing and orientation towards odors from breast milk signal preference, whereas in older infants likes and dislikes are signaled via facial expressions. Infants communicate readiness to eat by sucking their hands and eliciting the hunger cry, and indicate satiation through a wide repertoire of behaviors ranging from subtle facial expressions, shifts in gaze through to changes in gesture and bodily movements including turning their head away. Filming mealtime interactions reveals the dynamic nature of infant communication and caregiver response. Optimal responsiveness to infant cues may be influenced by individual characteristics of the mother and the mode of feeding (breastfed or formula). A series of studies has used video capture to characterize the ways in which infants communicate appetite. We have then translated this into an educational resource for healthcare professionals and caregivers on how to identify, interpret, and respond to these cues. There is a potentially important role for nutrition education in promoting both the principles of good nutrition as well as ways to read then respond sensitively to infant appetite cues.

Pedicled jejunal interposition for long gap esophageal atresia.

BACKGROUND/PURPOSE: Long gap esophageal atresia (LGEA) represents 10% of all esophageal atresias but can be complex to manage. Jejunal interposition (JI) has been recommended as the operative management of choice when esophageal ends cannot be opposed. We report our experience using the pedicled jejunal interposition technique with comparison to patients undergoing primary repair of LGEA. METHODS: This was a retrospective analysis of all patients managed for LGEA at our unit between 2003 and 2017 with comparison between pedicled jejunal interposition and primary repair. RESULTS: 10 patients were treated with pedicled jejunal interposition and 9 patients underwent primary repair (including one Foker procedure performed elsewhere). Patient demographics and short term outcomes were similar between the two groups, but less anastomotic stricturing and gastroesophageal reflux were observed in the JI group. CONCLUSION: This is the largest published series of pedicled jejunal interposition from the UK. Our results support continued use of this procedure with good long term graft function. It is our recommendation that pedicled interposition grafts are used in all patients requiring extensive dissection or tension to achieve opposition for primary repair. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level III.

The eyes have it: Infant gaze as an indicator of hunger and satiation.

Infant gaze serves as a measure of attention to food cues in adults and children and may play a role in signalling infant hunger and satiation. Maternal responsiveness to infant satiation cues, including gaze, supports healthy appetite development and may reduce obesity risk. However, mothers often experience difficulty in interpreting feeding cues, and there have been few attempts to study cues systematically. This study aimed to develop a reliable coding scheme for categorising and tracking infant gaze behaviours during complementary feeding (CF). Twenty infants aged between six and eighteen months were filmed during typical meals on two occasions at home. The Infant Gaze at Mealtime (IGM) coding scheme was devised from the analysis of a sample of videos, a piloting and testing process, and the feeding cues and developmental psychology literature. Inter and intra-rater reliability tests of the scheme with 20% of the study videos revealed high levels of reliability. When applied to the full sample of 225 video clips, the IGM coding scheme revealed a significant decrease over time in the frequency of infants gazing at food and a significant increase in exploratory gaze behaviour within a meal. These changes were consistent across main and dessert courses, suggesting they may be indicative of changes in infant feeding state. The results suggest that infant gaze may offer a means of identifying infant hunger and satiation and, as an easily observed behaviour, an effective tool for mothers and professionals for promoting responsive feeding.

Communicating hunger and satiation in the first 2 years of life: a systematic review.

Responsive feeding has been identified as important in preventing overconsumption by infants. However, this is predicated on an assumption that parents recognise and respond to infant feeding cues. Despite this, relatively little is understood about how infants engage parental feeding responses. Therefore, the aim of this systematic review was to identify what is known about infant communication of hunger and satiation and what issues impact on the expression and perception of these states. A search of Medline, CINAHL, Web of Science, PsycINFO, Science Direct and Maternal and Infant care produced 27 papers. Eligibility criteria included peer reviewed qualitative and/or quantitative publications on feeding behaviours, hunger, and satiation/satiety cues of typically developing children in the first 2 years of life. Papers published between 1966 and 2013 were included in the review. The review revealed that feeding cues and behaviours are shaped by numerous issues, such as infants' physical attributes, individual psychological factors and environmental factors. Meanwhile, infant characteristics, external cues and mothers' own characteristics affect how feeding cues are perceived. The existing literature provides insights into many aspects of hunger and satiation in infancy; however, there are significant gaps in our knowledge. There is a lack of validated tools for measuring hunger and satiation, a need to understand how different infant characteristics impact on feeding behaviour and a need to extricate the respective contributions of infant and maternal characteristics to perceptions of hunger and satiation. Further research is also recommended to differentiate between feeding driven by liking and that driven by hunger.

Parent-mediated intervention versus no intervention for infants at high risk of autism: a parallel, single-blind, randomised trial.

BACKGROUND: Risk markers for later autism identified in the first year of life present plausible intervention targets during early development. We aimed to assess the effect of a parent-mediated intervention for infants at high risk of autism on these markers. METHODS: We did a two-site, two-arm assessor-blinded randomised controlled trial of families with an infant at familial high risk of autism aged 7-10 months, testing the adapted Video Interaction to Promote Positive Parenting (iBASIS-VIPP) versus no intervention. Families were randomly assigned to intervention or no intervention groups using a permuted block approach stratified by centre. Assessors, but not families or therapists, were masked to group assignment. The primary outcome was infant attentiveness to parent. Regression analysis was done on an intention-to-treat basis. This trial is registered with ISCRTN Registry, number ISRCTN87373263. FINDINGS: We randomly assigned 54 families between April 11, 2011, and Dec 4, 2012 (28 to intervention, 26 to no intervention). Although CIs sometimes include the null, point estimates suggest that the intervention increased the primary outcome of infant attentiveness to parent (effect size 0.29, 95% CI -0.26 to 0.86, thus including possibilities ranging from a small negative treatment effect to a strongly positive treatment effect). For secondary outcomes, the intervention reduced autism-risk behaviours (0.50, CI -0.15 to 1.08), increased parental non-directiveness (0.81, 0.28 to 1.52), improved attention disengagement (0.48, -0.01 to 1.02), and improved parent-rated infant adaptive function (χ(2)[2] 15.39, p=0.0005). There was a possibility of nil or negative effect in language and responsivity to vowel change (P1: ES-0.62, CI -2.42 to 0.31; P2: -0.29, -1.55 to 0.71). INTERPRETATION: With the exception of the response to vowel change, our study showed positive estimates across a wide range of behavioural and brain function risk-markers and developmental outcomes that are consistent with a moderate intervention effect to reduce the risk for later autism. However, the estimates have wide CIs that include possible nil or small negative effects. The results are encouraging for development and prevention science, but need larger-scale replication to improve precision. FUNDING: Autistica, Waterloo Foundation, Autism Speaks, and the UK Medical Research Council.

Intervention for infants at risk of developing autism: a case series.

Theory and evidence suggest the potential value of prodromal intervention for infants at risk of developing autism. We report an initial case series (n = 8) of a parent-mediated, video-aided and interaction-focused intervention with infant siblings of autistic probands, beginning at 8-10 months of age. We outline the theory and evidence base behind this model and present data on feasibility, acceptability and measures ranging from parent-infant social interaction, to infant atypical behaviors, attention and cognition. The intervention proves to be both feasible and acceptable to families. Measurement across domains was successful and on larger samples promise to be an effective test of whether such an intervention in infancy will modify emergent atypical developmental trajectories in infants at risk for autism.

Survival in a 2-year-old boy with hemorrhage secondary to an aortoesophageal fistula.

Aortoesophageal fistula (AOF) in children with no history of cardiac disease or trauma is extremely rare, and survival is even rarer. We present a case of a 2-year-old boy who presented with massive hematemesis secondary to a primary aortoesophageal fistula and was found at endoscopy to have a submucosal hematoma. He then suffered a cardiac arrest with further catastrophic bleeding only controlled by a Sengstaken-Blakemore tube. Subsequent cardiopulmonary bypass was instituted and allowed direct repair of the fistula. He was discharged home 4 weeks later. The etiology of the fistula remains unknown.

Bleeding jejunal phlebectasia in an adolescent: case report.

The first case of jejunal phlebectasia in a pediatric patient presenting with life-threatening gastrointestinal bleeding is described. The pathology of vascular anomalies is discussed.