BACKGROUND: Fluorescence-guided surgery (FGS) with Indocyanine Green (ICG) dye enhances intra-operative visualisation of anatomical structures, tissue perfusion, and lymphatic drainage. To inform future practice and collaborative research, we aim to determine current FGS use in paediatric surgical centres in the UK and Ireland (UK&I). METHODS: In November 2023, an online survey of 11 to 21 questions was disseminated to one consultant surgeon at each of the 27 UK&I paediatric surgical centres. RESULTS: Twenty-four of 27 centres (88.9%) responded. Fourteen centres (58.3%) never used FGS, with 7 lacking the appropriate camera. The 10 centres (41.7%) with experience reported recent, limited use, with half performing their first case less than 1 year ago; half used fluorescence on fewer than 5 occasions. The top three indications were oncological procedures, lymphatic assessment, and biliary tree visualisation. A range of ICG doses were reported. Half of the centres had no ICG protocol. Only one centre used FGS in premature neonates. 19/24 (79.2%) deemed FGS helpful or acknowledged future potential; 13/24 (54.2%) believe an FGS registry would be valuable. CONCLUSIONS: Current use of FGS in children is limited and non-standardised. An FGS collaborative interest group or UK&I registry could support experience-sharing and standardisation.

Background: A primary pull-through for Hirschsprung's disease (HD) requires confirmation of normal ganglionic bowel by intraoperative biopsies to determine the level of resection. Despite this, aganglionic bowel that is not fully resected (so-called "transition zone pull-throughs") is reported in 15%-19% of patients. We hypothesize that this may result from insufficient biopsies sent for intraoperative diagnosis. Methods: A new biopsy protocol has been developed in our institution for patients undergoing a laparoscopic-assisted endorectal pull-through for HD. Laparoscopic seromuscular biopsies are taken as per standard practice and are reported intraoperatively to identify the most distal site of ganglionic bowel. A 5 mm laparoscopic stapling device is used to divide the bowel at the proposed proximal resection margin and 2 cm distally. If there is any evidence of abnormality in the first doughnut, a second, more proximal doughnut is taken. Results: Between 2015 and 2020, 21 patients underwent a primary laparoscopic endorectal pull-through for HD using the doughnut biopsy protocol. Sixteen patients were male. The mean patient age at the time of surgery was 3 months (range 1-6 months), and the mean weight at the time of surgery was 6.5 kg (range 4.1-8.54 kg). In all 21 cases, initial laparoscopic biopsies were reported showing normal ganglionic bowel; in two cases (10%), the laparoscopic doughnut influenced the proximal resection margin. In both cases, aganglionic tissue was identified intraoperatively in the doughnuts, and a second, more proximal doughnut was sent. No patients had transition zone resections on final histology (mean clear margin 45.55 mm, range 11-72 mm). Conclusions: In conclusion, intraoperative frozen sections taken from doughnuts of bowel retrieved using 5 mm laparoscopic stapling devices are safe and have resulted in a 0% rate of transition zone pull-throughs while reducing the potential of spillage of enteric contents. We would recommend this protocol for all patients undergoing primary endorectal pull-throughs.

Background: Anterior anus is considered part of the spectrum of anorectal malformations in girls and has been associated with childhood constipation. However, limited literature exists on outcomes and associated malformations. Methods: All girls <6 months referred to our centre with suspected anterior anus between January 2015 and December 2022 were identified. Data collected included patient demographics, presence of anomalies, operative management, laxative use and continence. Results are described using descriptive statistics and percentages. Results: 147 girls were referred with suspected anterior anus of which 95 were confirmed to have an anterior anus. Median age of first assessment was 3 (0-13) months. Median follow up was 24 (1-94) months. 21 (22.1 %) had clinical concern of partial absence of normal circumferential anal corrugation. 10 (10.5%) girls underwent examination under anaesthesia; 4 patients underwent anoplasty with covering colostomy. Laxatives were prescribed in 31 (32.6%) girls. Of the 53 patients followed up to age 4 years and older, 51 (96.2%) achieved continence. On renal ultrasonography significant hydroureteronephrosis was detected in one patient. No significant spinal anomalies were detected on imaging. Two patients had ventricular-septal defects were identified. All significant anomalies were in patients with an ectopic anus/ perineal fistula. Conclusion: This represents the largest reported series of girls with anterior anus. The incidence of identified associated malformations was low. Furthermore, laxative use and continence outcomes are similar to the general infant/childhood population. Screening and routine follow-up should be reserved for individual cases where there is clinical concern.

Children and young people with severe neurodisabling conditions (CYPSND)experience severe functional gastrointestinal symptoms and dependence on artificial nutrition. 'Gastrointestinal dystonia' (GID) has been applied by clinicians when symptoms become debilitating and potentially life-limiting. Evidence is lacking regarding the definition and appropriate management of GID. We therefore assembled a RAND appropriateness panel. We performed a systematic review, created an online survey and distributed this to a panel of 27 experts from five stakeholder groups from 13 UK specialist centres across the British Isles (gastroenterology, neurology/neurodisability, surgery, palliative care and allied health professionals). A Disagreement Index ≥1 indicated disagreement. The panel rated the appropriateness of 250 statements covering the following in GID: definition, clinical evaluation, nutritional assessment/feeding strategies, investigations, medications and prescribing, surgical interventions, safeguarding, palliative care and ethics. Agreement was reached except in selected statements regarding uncommon diagnostic features. There was uncertainty in specific clinical scenarios regarding: investigation, the use of blenderised diet, certain pharmacological agents and surgical interventions. The only intervention deemed inappropriate was antireflux surgery in the context of GID and gastrointestinal dysmotility without reflux disease. The remaining statements (198) were considered appropriate. We present a comprehensive review, agreement on the definition of GID and recommendations on management pathways agreed by a selected panel of multidisciplinary experts. Clear diagnostic criteria will enable important epidemiological work to record outcomes for this complex patient group. Identifying the associated morbidity, burden of care and mortality will help advocate for appropriate health resources and support to carers and families.

Background For children and young people with severe neurodisabling conditions (CYPSND), the clinical constellation of pain behaviour, retching, bloating, abdominal distension and constipation/pseudo-obstruction can be referred to as gastrointestinal dystonia (GID). Nabilone, a synthetic analogue of the active component tetrahydrocannabinol found in cannabis, has a licence in paediatrics for the treatment of severe chemotherapy-induced nausea. We aim to describe our experience of using nabilone for CYPSND with GID. Methods Approval was sought on a named patient basis from the clinical directorate and lead pharmacist on the basis that; patients fulfilled our criteria for GID, patients had been trialled on medical therapies, jejunal feeding and blended diet, concomitant medication reviewed in multidisciplinary team including tone management and to review potential exacerbants to GID. Patients were admitted for 48 hours for monitoring of temperature, pulse, respiratory rate, oxygen saturations and symptoms using a modified paediatric pain score. Nabilone was commenced at 250 mu g daily and then incremented in 250 mu g doses to a dosage of <18 kg: 500 g two times per day and 18-27 kg: 500 mu g three times a day. Efficacy was assessed by parents' and clinicians' perception, PedsQL V.3.0 (gastrointestinal subset questions) and sleep diary, prior to treatment, 1 month and 6 months after stable dose, and median scores were analysed by paired Student's t-test (p<0.05). Results In four of five suitable patients, the PedsQL score was higher after commencing treatment and there was a sustained improvement at 6 months. Patient median total PedsQL Quality of Life (QOL) scores were 46 (28-50) prior to treatment and 68.5 (58-74) 1 month after commencing treatment (p=0.032.) After 6 months, the median PedsQL QOL score was 73 (51-82) (p=0.017.) Conclusions Nabilone shows promise in treating GID, a poorly understood debilitating complication of severe neurodisability. In particular, symptoms of nausea, retching and pain on feeding were all reduced in our cohort.

Anorectal malformations (ARMs) are a spectrum of congenital anomalies where there is abnormal development of the anus and rectum. With an incidence of 1:5000 live births and affecting both males and females, these anomalies vary in their appearance and presentation, lack features enabling antenatal detection and should be detected at birth by the examining midwife or within 72 hours through the newborn and infant physical examination (NIPE) screening programme. However, it is recognised that the diagnosis of ARMs can be missed or delayed leading to morbidity and mortality. In the UK, despite the existence of the NIPE screening programme and NICE guidelines, published literature shows that nearly a quarter of ARMs are not diagnosed at birth. This review takes a critical look at the frequency of missed/delayed diagnosis of ARMs at birth, the implications of delayed diagnosis, and the possible reasons for this related to education and training of healthcare professionals involved in newborn examination, focusing on the UK national screening programme for NIPE. We propose a strategy for enhancing detection of ARMs in a timely manner through the existing framework of the NIPE screening programme.

Introduction/Background Chronic constipation is a common condition in childhood, with an estimated prevalence of 3% worldwide.1 Commonly no cause is found (idiopathic constipation) although there are a number of important aetiologies to rule out during the evaluation of children with constipation. Pharmacological management is the mainstay of treatment, current NICE recommendations for refractory cases is for referral to surgical services for potentially more invasive therapies.2 Prucalopride is a 5-HT4 receptor agonist, it produces an enterokinetic effect to increase colonic motility. It is recommended as a treatment of refractory constipation in adult but, despite promise, there is little data supporting its use in paediatrics.4 Aim To provide a single tertiary centre experience of using Prucalopride in paediatric patients with treatment refractory constipation. Subjects and Methods 5 female teenage patients (12–17yrs) average age at initiation 15yrs with treatment refractory constipation in our centre. Patients received a dose of 2mg once daily on an initial 1 month trial of treatment. Table 1 summarises the patient demographics and treatment outcomes. All patients had idiopathic constipation. 4 out of 5 (80%) had rectal biopsies that were normal. 3 out of 5 (60%) had previous transit studies with 2 out of 3 (66%) showing delayed elimination of markers. 1 out of 5 (20%) underwent manometry assessment and this was normal. 2 out of 5 (40%) patients had previous surgical therapy for their constipation with both patients undergoing an ACE procedure and 1 patient additionally receiving anal botox therapy. Results There was a wide variation in reported stooling prior to commencing Prucalopride and 3 out of 5 (60%) of patients reported an improvement in stooling pattern following the commencement of Prucalopride. In patients where there was an improvement in stooling frequency, all were able to deescalate other medical therapies including reductions in rectal therapies. With regards to adverse side effects: 2 (40%) patients reported pain initially on commencing treatment, 1 (20%) patient reported headache, 1 (20%) patient reported a worsening of existing bladder instability and 1 (20%) patient reported reduction of appetite and weight loss. No patient had adverse side effects that led to the cessation of treatment. Summary We present our unit’s experience in using Prucalopride, a novel agent for use in the management of paediatric constipation. 3 out of 5 patients receiving Prucalopride had an improvement in their stooling pattern with acceptable side effect profiles. Prucalopride should be considered in management of paediatric chronic constipation not responding to conventional medical therapy. References Tabbers MM, et al. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr 2014 Feb;58(2):258–74. Constipation in children and young people: diagnosis and management Clinical guideline [CG99]Published: 26 May 2010 Last updated: 13 July 2017 NICE. Prucalopride for the treatment of chronic constipation in women Technology appraisal guidance [TA211]Published: 15 December 2010 NICE. Mugie SM, et al. Prucalopride is no more effective than placebo for children with functional constipation. Gastroenterology 2014 Dec;147(6):1285–95.e1.

Background: This prospective cohort study compared primary-school-aged outcomes between children with Hirschsprung disease (HD) following Soave, Duhamel or Swenson procedures.Methods: Children with histologically proven HD were identified in British/Irish paediatric surgical cen-ters (01/10/2010-30/09/2012). Parent/clinician outcomes were collected when children were 5-8 years old and combined with management/early outcomes data. Propensity score/covariate adjusted multiple -event-Cox and multivariable logistic regression analyses were used.Results: 277 (91%) of 305 children underwent a pull-through (53% Soave, 37% Duhamel, 9% Swen-son). Based upon 259 children (94%) with complete operative data, unplanned reoperation rates (95% CI) per-person year of follow-up were 0.11 (0.08-0.13), 0.34 (0.29-0.40) and 1.06 (0.86-1.31) in the Soave/Duhamel/Swenson groups respectively. Adjusted Hazard Ratios for unplanned reoperation com-pared with the Soave were 1.50 (95% CI 0.66-3.4 4, p = 0.335) and 7.57 (95% CI 3.39-16.93, p < 0.001) for the Duhamel/Swenson respectively. Of 217 post-pull-through children with 5-8 year follow-up, 62%, 55%, and 62% in Soave/Duhamel/Swenson groups reported faecal incontinence. In comparison to Soave, Duhamel was associated with lower risk of faecal incontinence (aOR 0.34,95%CI 0.13-0.89, p = 0.028). Of 191 children without a stoma, 42%, 59% and 30% in Soave/Duhamel/Swenson groups required assistance to maintain bowel movements; compared to Soave, the Duhamel group were more likely to require as-sistance (aOR 2.61,95% CI 1.03-6.60, p = 0.043).Conclusions: Compared with Soave, Swenson was associated with increased risk of unplanned reopera-tion, whilst Duhamel was associated with reduced risk of faecal incontinence, but increased risk of consti-pation at 5-8 years of age. The risk profiles described can be used to inform consent discussions between surgeons and parents.Level of evidence: Level II (c) 2022 The Author(s). Published by Elsevier Inc. This is an open access article under the CC BY license ( http://creativecommons.org/licenses/by/4.0/ )

Background and Aims Children and young people with severe neurosdisabling conditions (CYPWSND) experience an array of serious gastrointestinal symptoms beyond gastro-oesophageal reflux, constipation or dependence on artificial nutrition. When enteral feeds leads to disabling dystonia the term 'gastrointestinal dystonia of severe neurodisability' (GID) has been applied by clinicians. However a clear definition with criteria for entry point is lacking in the literature. We describe the methods for formal establishment of an agreed definition of GID. Methods After commissioning by BSPGHAN, systematic review1 and consultation with public bodies it was agreed, due to paucity of evidence that an appropriateness panel should be the forum for formulation of output on GID. A writers group structured the questions for the survey definition, based on the limited written evidence and added professional experience. A panel of 27 experts in their field were assembled from 5 stakeholder groups including: Gastroenterology, Neurology/Neurodisability, Surgery, Palliative Care and Allied Health Professionals. Geographic representation was from 13 UK specialist centres (including all 4 nations) and 1 centre from Republic of Ireland. The panel rated the appropriateness of definition, investigations and management of GID. A scale of 1–9 enabled scoring of 1–3 to indicate inappropriate, 4–6 uncertain, 7–9 appropriate as criteria for recommendation. Panel agreement index was calculated using a continuous likelihood ratio, with <1 indicated 'general agreement' and >1 'no agreement'. Results were discussed at a moderated. Results All of the panel completed all questions on 'common' (table 1) and 'uncommon' features of GID. The panel had strong concurrence that GID definition required patients have GMFCS 4–5 cerebral palsy or equivalent and that a temporal relationship between symptoms and enteral feeding had to be present (although this relationship may lessen or cease during progressive disease). Pain, distress, retching, autonomic activation and hypertonicity were seen as common features. Temporal relationship with bowel habit, involuntary movements were considered less common. The diagnosis should be a positive clinical diagnosis (not of exclusion) made by a specialist multi-disciplinary team with experience of feeding disorders in severe neuro-disability. Features suggesting patients feed intolerance has reached the threshold for GID would include malnutrition primarily due to feed cessation and GI symptoms being the greatest burden on QOL for patient/family on appropriate survey. Conclusions We present a coherent first definition for GID by consensus of a panel of identified experts drawn from 5 invested stakeholder groups. Clear entry point for diagnosing GID will allow for important epidemiological work to report investigations, interventions and outcomes for this complex group of patients. Identifying significant morbidity care burden and mortality in this patient group will help advocate for appropriate health resources, support to carers and families. The ongoing development of a management framework through completion of the RAND2 process in 2022 should assist navigation of the complex medical and ethical challenges of management of distressing and debilitating symptoms for patients with this condition. Reference McConnell N, Beattie LM, Richards CA Protheroe S, Barclay AR. JPGN; 2018: 1002 https://www.rand.org/pubs/monograph_reports/MR1269.html Acknowledgement BSPGHAN BiG funding 2020.

Introduction Children with severe neurodisability and tone disorders can present with debilitating symptoms and declining growth. Gastrointestinal dystonia (GID) describes the clinical manifestations of: pain behaviour; hypertonicity; retching; vomiting; vagal phenomena; abdominal distension and straining attributable to the GI tract in the context of severe neurodisability.1Blended diet (BD) has an emerging role in the nutritional management of patients with neurodisability, however we are unaware of data pertaining to BD specifically in patients with GID. Aim A tertiary centre review of outcomes of patients with GID receiving BD. Method Patients who commenced BD between 07/2017 and 02/2020 were identified from prospectively gathered complex enteral nutrition (CEN) and specialist dietetic databases. BD was initiated with specialist paediatric dietetic support within the CEN clinic or in other clinics. Data gathered included: demographics; primary diagnosis; enteral feeding plan; fundoplication; weight standard deviation z score at 0, 6, 12 and 18 months prior to and from commencing BD; medications at 0 and 6, 12, 18 months; reasons if BD discontinued; parenteral nutrition (PN) requirement. Results 29 children met criteria for GID and commenced BD.1 18 were male. 14 had a fundoplication. Feeding method prior to BD: gastric bolus 17; gastric continuous 8; jejunal 4. Mean age BD commenced was 7 years. Follow up ranged 8 to 40 months. 25 patients continue BD to date. 4 discontinued BD within 1–2 months citing increased GI symptoms or device blockage. Of the 25 who continue BD, median weight z scores declined from -1.77 at 12 months prior to BD, -1.86 at 6 months prior, -1.94 at 0 months, then rose to -1.54 at 12 months, and -1.40 at 18 months. Mean weight z scores were maintained at -1.97 (CI -0.85 to -3.09) at 0 months, -1.97 (CI -0.27 to -3.02) at 12 months and -1.79 (CI -0.34 to -3.25) at 18 months [figure 1]. This trend was more significant in a subset of 6 patients, z score -2.06 (CI -1.46 to -2.67) at 0 months, -1.13 (CI -0.59 to -1.67) at 12 months (p = 0.018) and -0.84 (CI -0.46 to -1.21) at 18 months (p = 0.003) [figure 2]. 9 of the 25 were able to discontinue one GI medication, 4 discontinued two GI medications. Tone medications: 3 reduced; 20 unchanged; 2 increased. No patients received PN during the study. Conclusion GID represents the severest end point of gastrointestinal symptoms in neurodisability, with progressive decline often a feature. Our data show children with GID receiving BD continue to track their weight trajectory, whilst some experience significantly improved growth. No patients required trial on PN during the study. The authors advocate for BD's role in minimizing the need for invasive treatments in GID whilst addressing symptoms and maintaining nutritional status2 and growth superiorly to formula feeds alone. Improvements in symptomatology and quality of life will be better described by prospective survey of patients commencing BD for GID. The authors are currently gathering this data. References McConnell N, et al. JPGN;66(supp2):1002; 2018 Hay J, et al. (presented BSPGHAN 2020)

ABSTRACTExclusive enteral nutrition (EEN) is effective in inducing remission in paediatric Crohn Disease (CD) and has been shown to reduce inflammation and improve outcomes in adult CD patients when used before resectional surgery. This retrospective study demonstrates that preoperative EEN is achievable in paediatric CD patients undergoing right hemicolectomy and is associated with positive peri‐operative outcomes. Seventeen patients (8 who received preoperative EEN and 9 who did not) were included in the study. Six of 8 (75.0%) managed EEN orally; 1 via nasogastric tube and another via a previously sited gastrostomy. Use of preoperative EEN was associated with a decreased rate of moderate/severe disease on resection pathology (5/8 [62.5%] vs 9/9 [100%]; P = 0.04). Larger studies are required to determine the wider potential benefits of preoperative EEN on postoperative outcomes within paediatric practice.

Objective This study describes core outcomes of Hirschsprung’s disease (HD) in a UK-wide cohort of primary school-aged children. Design A prospective cohort study conducted from 1 October 2010 to 30 September 2012. Outcomes data were collected from parents and clinicians when children were 5–8 years of age, and combined with data collected at birth, and 28 days and 1 year post diagnosis. Setting All 28 UK and Irish paediatric surgical centres. Participants Children with histologically proven HD diagnosed at <6 months of age. Main outcome measures NETS 1HD core outcomes. Results Data were returned for 239 (78%) of 305 children. Twelve children (5%) died prior to 5 years of age. Of the 227 surviving children, 30 (13%) had a stoma and 21 (9%) were incontinent of urine. Of the 197 children without a stoma, 155 (79%) maintained bowel movements without enemas/washouts, while 124 (63%) reported faecal incontinence. Of the 214 surviving children who had undergone a pull-through operation, 95 (44%) underwent ≥1 unplanned reoperation. 89 unplanned reoperations (27%) were major/complex. Of the 83 children with returned PedsQL scores, 37 (49%) had quality of life scores, and 31 (42%) had psychological well-being scores, that were ≥1 SD lower than the reference population mean for children without HD. Conclusion This study gives a realistic picture of population outcomes of HD in primary school-aged children in the UK/Ireland. The high rates of faecal incontinence, unplanned procedures and low quality of life scores are sobering. Ensuring clinicians address the bladder, bowel and psychological problems experienced by children should be a priority.

Objectives: A minimally invasive biomarker to monitor disease activity is one of the greatest unmet clinical needs of the pediatric eosinophilic esophagitis (EoE) population. We aimed to determine whether circulating eosinophil progenitors (EoPs) could be used as a biomarker to identify pediatric patients with active EoE. Methods: In a prospective observational study, peripheral blood samples, symptom history, and laboratory data were collected from pediatric patients undergoing endoscopy for evaluation of EoE on dietary therapy at Cincinnati Children's Hospital. Peripheral blood EoP level was determined by flow cytometry. Results: Thirty-four children with active (n = 16) and inactive (n = 18) EoE were included in the analysis. EoP levels in the peripheral blood were 3-fold higher in patients with active EoE than inactive EoE (P < 0.0025). Blood absolute eosinophil count did not distinguish between active and inactive EoE (P = 0.16). A cut-off EoP level >= 17 accurately detected active disease in 79% of patients with 94.4% specificity and 62.5% sensitivity (area under the curve 0.81;P ).Antihistamine use lowered the threshold EoP level to detect active EoE. Conclusions: This study suggests that blood EoP levels may be used as a biomarker to detect active EoE disease in patients undergoing food trials and potentially reduce the need for repeated endoscopies. Larger prospective studies are needed to investigate the effects of antihistamines and swallowed steroids on EoP mobilization into the peripheral blood and longitudinal studies to assess the performance of the assay in individual patients over time.

Exclusive enteral nutrition (EEN) is effective in inducing remission in paediatric Crohn Disease (CD) and has been shown to reduce inflammation and improve outcomes in adult CD patients when used before resectional surgery. This retrospective study demonstrates that preoperative EEN is achievable in paediatric CD patients undergoing right hemicolectomy and is associated with positive peri-operative outcomes. Seventeen patients (8 who received preoperative EEN and 9 who did not) were included in the study. Six of 8 (75.0%) managed EEN orally; 1 via nasogastric tube and another via a previously sited gastrostomy. Use of preoperative EEN was associated with a decreased rate of moderate/severe disease on resection pathology (5/8 [62.5%] vs 9/9 [100%]; P=0.04). Larger studies are required to determine the wider potential benefits of preoperative EEN on postoperative outcomes within paediatric practice.

Background Infants with gastrointestinal conditions and poor weight gain are administered sodium supplementation based on urinary sodium concentrations. However, the reference range of urinary electrolytes is unknown. The aim of this study was to ascertain the normal values of urinary electrolytes in healthy, term infants. Secondary aims were to establish the relationship between urinary electrolytes with weight velocity and feeding practices. Methods Healthy, term (≥37 weeks’ gestation) infants were recruited. Parental questionnaires were completed before discharge and at six weeks. Electrolytes were quantified from a urine sample at six weeks. t-Tests and Mann-Whitney U tests were conducted for parametric and non-parametric electrolytes, respectively. Results A total of 200 infants were recruited before discharge. Twenty-nine follow-up questionnaires and urine samples were returned (nine female; mean gestational age 39 + 6 weeks [SD 9.9 days]; mean birthweight 3350 g [SD 483 g]; 17 breastfed, nine formula and three mixed; mean change in Z score for weight –0.914 [SD 0.814]). Majority (25/29) of infants had urinary sodium <20 mmo/L. Change in Z score for weight was similar between infants with sodium <20 mmol/L and >20 mmol/L (P = 0.78). All exclusively breastfed infants had sodium <20 mmol/L, however, not statistically dissimilar to formula-fed infants (P = 0.27). Conclusion Most term infants in this study had urinary sodium values <20 mmol/L with no identified relationship to weight velocity. Lower concentrations of sodium could be not quantified reliably because of the limitations of the analytical method that were used. More evidence is required to identify candidates for sodium supplementation.

Congenital diaphragmatic hernia (CDH) remains one of the most challenging conditions to manage in neonatology and neonatal surgery. Despite significant advances in antenatal detection, antenatal therapy, neonatal intensive care and neonatal surgery, there is a significant mortality rate due to the combination of pulmonary hypoplasia, cardiac dysfunction and pulmonary hypertension. One of the major deficiencies in the literature has been a lack of high quality, multicentre data with a complete population denominator to accurately reflect the prognosis of this condition. Such studies would aid parental counselling, guide therapy and provide benchmarking data for clinicians and centres. The paper by Long et al 1 is a valuable contribution to the current evidence base, but we must be cautious in interpreting the presented data as true population outcomes.This paper is a prospective study of a cohort of infants who presented to paediatric surgical units in the UK and Ireland with diaphragmatic defects over an 18-month period during 2009–2010. The authors used robust methodology of case ascertainment, supported by the National Perinatal Epidemiology Unit, where local clinicians returned cards on a monthly basis. The data describe 219 live-born infants with an antenatal detection rate of 61%. This reflects 134 antenatally diagnosed CDH cases in 18 months. For the first 12 months of this study, the UK Obstetric Surveillance System (UKOSS) was conducting a study for pregnancies affected by CDH. Although these data have not yet been published, they identified 179 pregnancies in this period (https://www.npeu.ox.ac.uk/research/ukoss-cdh-174). Before discussing the outcome of postnatal surgical management, we should question why only approximately 50% of antenatally diagnosed cases have presented to surgical units postnatally to be included in the presented paper. Possible explanations include terminations of pregnancy and antenatally diagnosed newborns not reaching surgical centres.Modern management of CDH starts …

BACKGROUND:Between 20% and 50% neonates with bilious vomiting are diagnosed with surgical pathology. Distinguishing neonates requiring surgery remains challenging. Our aim was to conduct an audit of term neonates with bilious vomiting referred for assessment to identify characteristics of this cohort and management. Secondary aims were to identify factors predictive of surgical pathology. METHODS:Infants <28 days referred for bilious vomiting from 2011 to 2015 were identified through cross-referencing multiple patient databases. Data obtained included clinical features, laboratory, radiological investigations and management. The sensitivity and specificity of tests were calculated and regression analyses were conducted to identify predictors of surgical pathology. RESULTS:351 eligible neonates were referred [46% female; mean gestation 39 + 6 weeks (SD 9.2 days); mean birthweight 3469 g (SD 558 g)]. Laboratory results were available for 68.7% patients, 88.9% underwent X-ray and 96.6% contrast studies. 11.7% had a surgical diagnosis [malrotation 4.6% (1.7% with volvulus)]. No single test available in peripheral centers could exclude a surgical diagnosis. In regression analyses, age > 72 h, presence of abdominal distension, raised CRP and abnormal X-ray were statistically significant predictors of surgical pathology, while only the former two were predictive of time-critical surgical pathology. CONCLUSION:11.7% neonates had surgical pathology, fewer than in previous studies. Only contrast fluoroscopy could exclude surgical pathology and therefore prevent transfer. A more sensitive, widely available test would be required to reduce unnecessary neonatal transfers. TYPE OF STUDY:Prognosis study. LEVEL OF EVIDENCE:Level III.

Objective The objective of this study was to develop a Hirschsprung's disease (HD) core outcome set (COS). Methods Candidate outcomes were identified from a systematic review and stakeholder nomination. A three-phase Delphi process and consensus meeting were used to prioritise candidate outcomes based on scores assigned by stakeholder participants using a nine-point scale. In phases two and three, participants were shown graphical representations of their panel's scores and all panels' scores respectively for each outcome from the previous phase. After the third phase, outcomes prioritised by two or three panels were taken forward to the consensus meeting. The COS was formed from the 10 highest scoring outcomes meeting the threshold for inclusion (≥70% 7–9 and <15% 1–3). Results Eighty-nine stakeholders (82%) completed all three phases of the Delphi process. Seventy-four outcomes were assessed in phase one of the Delphi process, the following 10 of which met criteria for inclusion in the COS: (1) death with cause specified, (2) long-term faecal incontinence, (3) long-term voluntary bowel movements without need for enemas, or rectal or colonic irrigation, (4) long-term psychological stress for the individual with Hirschsprung's disease, (5) long-term urinary incontinence, (6) objective score of quality of life, (7) objective score of bowel function, (8) unplanned reoperation, (9) >need for a permanent stoma, (10) enterocolitis. Conclusions This HD COS is formed of 10 outcomes deemed important by key stakeholders. Use of this COS in research will reduce outcome reporting heterogeneity and increase our ability to identify gold standard treatments for HD.

Background/purpose: Primary pull-through without a stoma has become preferred practice in managing Hirschsprung disease (HD). The aims of this study were to establish stoma rate and identify factors associated with stoma formation in a population-based cohort in the UK and Ireland.Methods: Live-born infants with HD were prospectively identified in all 28 specialist pediatric surgical units in the UK and Ireland between October 2010 to September 2012. Method of colonic decompression was recorded and multivariable logistic regression was used to identify factors associated with stoma formation.Results: 305 infants with HD were identified. Rectal washouts were initially used in 86% (263) with a defunctioning stoma formed as the primary management in 13% (39). Ultimately, 36% (111) required a stoma prior to definitive surgery. Compared to infants managed with rectal washouts alone; infants managed with a stoma were more likely to have a transition zone proximal to the splenic flexure, Down (or another) syndrome, and HD diagnosis established more than 28 days after presentation.Conclusions: Although rectal washouts are commonly employed, a stoma prior to definitive surgery was required in 36% of infants in a national cohort. Delayed diagnosis, aganglionosis proximal to the splenic flexure and presence of other anomalies are associated with stoma formation. (C) 2017 Elsevier Inc. All rights reserved.