Impact of rectal dissection technique on primary-school-age outcomes for a British and Irish cohort of children with Hirschsprung disease.

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 centers (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% Swenson). 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 compared with the Soave were 1.50 (95% CI 0.66-3.44, 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 assistance (aOR 2.61,95% CI 1.03-6.60,p = 0.043). CONCLUSIONS: Compared with Soave, Swenson was associated with increased risk of unplanned reoperation, whilst Duhamel was associated with reduced risk of faecal incontinence, but increased risk of constipation 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.

Thromboprophylaxis Use in Paediatric Inflammatory Bowel Disease: An International RAND Appropriateness Panel.

BACKGROUND AND AIMS: Thromboprophylaxis use in paediatric inflammatory bowel disease [IBD] is inconsistent. Current guidelines only support treating children with acute severe colitis with risk factors. We convened an international RAND panel to explore thromboprophylaxis in paediatric IBD inpatients in the context of new evidence. METHODS: We convened a geographically diverse 14-person panel of paediatric gastroenterologists alongside supporting experts. An online survey was sent before an online meeting. Panellists were asked to rate the appropriateness of thromboprophylaxis in hospitalised paediatric IBD patients via 27 scenarios of varying ages, gender, and phenotype, with and without thrombotic risk factors. Anonymised results were presented at the meeting. A second modified survey was distributed to all panellists present at the meeting. Results from the second survey constitute the RAND panel results. The validated RAND disagreement index defined disagreement when ≥ 1. RESULTS: The combined outcome of thromboprophylaxis being considered appropriate until discharge and inappropriate to withhold was seen in 20 of 27 scenarios, including: all patients with new-onset acute severe colitis; all flares of known ulcerative colitis, irrespective of risk factors except in pre-pubescent patients with limited disease and no risk factors; and all Crohn's patients with risk factors. Disagreement was seen in five scenarios regarding Crohn's without risk factors, where outcomes were already uncertain. CONCLUSIONS: RAND panels are an established method to assess expert opinion in areas of limited evidence. This work therefore constitutes neither a guideline nor a consensus; however, the findings suggest a need to re-evaluate the role of thromboprophylaxis in future guidelines.

Prehabilitation: The Impact of Preoperative Exclusive Enteral Nutrition on Paediatric Patients With Crohn Disease.

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.

An audit of bilious vomiting in term neonates referred for pediatric surgical assessment: can we reduce unnecessary transfers?

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.

NETS1HD study: development of a Hirschsprung's disease core outcome set.

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.

NETS1HD: study protocol for development of a core outcome set for use in determining the overall success of Hirschsprung's disease treatment.

BACKGROUND: Use of core outcome sets in research has been proposed as a method for countering the problems caused by heterogeneity of outcome measure reporting. Heterogeneity of outcome measure reporting occurs in Hirschsprung's disease (HD) research and is limiting the development of a robust evidence base to support clinical practice. METHODS: Candidate outcome measures have been identified through a systematic review. These outcome measures will form the starting point for a three-phase online Delphi process to be carried out in parallel by three panels of experts. Panel 1 is a neonatal panel; panel 2 is a non-neonatal panel; and panel 3 is a lay panel. In round 1, experts will be asked to score the previously identified outcome measures from 1 to 9 based on how important they think the measures are in determining the overall success of their/their child's/their patient's HD. In round 2, experts will be presented with the same list of outcome measures and graphical representations of how their panel scored that outcome in round 1. They will be asked to re-score the outcome measure, taking into account how important other members of their panel felt it to be. In round 3, experts will again be asked to re-score each outcome measure, but this time they will receive a graphical representation of the distribution of scores from all three panels, which they should take into account when re-scoring. Following round 3 of the Delphi process, 40 experts will be invited to attend a face-to-face consensus meeting. Participants will be invited in a purposive manner to obtain balance between the different panels. Results of the Delphi process will be discussed, and outcomes will be re-scored. Outcome measures where >70% of participants at the meeting scored it 7-9 and <15% scored it 1-3 will form the core outcome set. DISCUSSION: Development of a core outcome set will help to reduce heterogeneity of outcome measure reporting in HD. This will increase the quality of research taking place and ultimately improve care provided to infants with HD.

Patients' experience of portacaths in cystic fibrosis: questionnaire-based study.

BACKGROUNDS AND AIMS: Portacaths are regularly used in children with cystic fibrosis (CF). We aimed to assess patient satisfaction with lateral chest wall portacaths in children with CF. METHODS: All children in a geographical region with CF and portacath in situ were identified. Site of chest wall placement was identified on X-ray; only children with lateral chest wall portacaths were sent questionnaires. Data collected included preoperative information, cosmesis and interference with activities. RESULTS: Of the 46 patients identified, 42 had lateral chest wall ports. 25 of this 42(60%) submitted their questionnaires. 22(88%) were happy with preoperative information although only 8(32%) recall being offered choice of position. 23(92%) were satisfied with cosmesis. 2 patients reported problems with physiotherapy only with indwelling needles. 6(24%) patients had problems with clothing, 7(32%) with sports and 3(12%) with seatbelts. CONCLUSIONS: Lateral chest wall portacaths are cosmetically acceptable. Impact on daily activities is less common than that reported with anterior chest wall placement.

Laparoscopic assistance for primary transanal pull-through in Hirschsprung's disease: a systematic review and meta-analysis.

OBJECTIVE: To compare outcomes following totally transanal endorectal pull-through (TTERPT) versus pull-through with any form of laparoscopic assistance (LAPT) for infants with uncomplicated Hirschsprung's disease. DESIGN: Systematic review and meta-analysis. SETTING: Five hospitals with a paediatric surgical service. PARTICIPANTS: 405 infants with uncomplicated Hirschsprung's disease. INTERVENTIONS: TTERPT versus LAPT. PRIMARY OUTCOMES: mortality, postoperative enterocolitis, faecal incontinence, constipation, unplanned laparotomy or stoma formation, and injury to abdominal viscera. SECONDARY OUTCOMES: Haemorrhage requiring transfusion of blood products, abscess formation, intestinal obstruction, intestinal ischaemia, enteric fistula formation, urinary incontinence or retention, impotency and duration of procedure. RESULTS: Five eligible studies comprising 405 patients were identified from 2107 studies. All studies were retrospective case series, with variability in outcome assessment quality and length of follow-up. Operative duration was 50.29 min shorter with TTERPT (95% CI 39.83 to 60.74, p<0.00001). There were no significant differences identified between TTERPT and LAPT for incidence of postoperative enterocolitis (OR=0.78, 95% CI 0.44 to 1.38, p=0.39), faecal incontinence (OR=0.44, 95% CI 0.09 to 2.20, p=0.32) or constipation (OR=0.84, 95% CI 0.32 to 2.17, p=0.71). CONCLUSIONS: This meta-analysis did not find any evidence to suggest a higher rate of enterocolitis, incontinence or constipation following TTERPT compared with LAPT. Further long-term comparative studies and multicentre data pooling are needed to determine whether a purely transanal approach offers any advantages over a laparoscopically assisted approach to rectosigmoid Hirschsprung's disease. TRIAL REGISTRATION NUMBER: PROSPERO registry- CRD42013005698.

The long-term outcome of patients diagnosed with sacrococcygeal teratoma in childhood. A study of a national cohort.

BACKGROUND/PURPOSE: The improved survival of sacrococcygeal teratoma (SCT) has led to increased awareness of its long-term sequelae. Our aim was to assess the long-term outcome of a national cohort using detailed questionnaires. METHODS: The three paediatric surgery centres in Scotland were contacted to identify all SCT patients ≥5 years of age. Case notes were reviewed. Detailed separate questionnaires were used to assess long-term bowel, urinary and obstetric outcomes and were completed during an arranged interview. Groups were statistically compared using Z-tests or Fisher's exact test. RESULTS: Overall, 48 patients were identified but only 31 were available for follow-up. Age ranged from 5-35 years (median 12 years and 8 months). There were 25 (81%) females and 5 (16%) patients had malignant disease. Abnormal bowel function was noted in 42% of patients, with constipation being the commonest complaint (39%) with no obvious predictive features at presentation. Urinary symptoms were reported in 55% of the patients. A total of nine (29%) patients suffered from urgency and/or wetting. Confirmed urinary tract infections (UTIs) were reported by nine patients. Successful pregnancies were reported by two females and neither of their children had SCT. CONCLUSIONS: This is one of the largest national studies assessing the long-term outcome of patients with SCT. It highlights the significant gastrointestinal and urological long-term morbidities of SCT patients, which is useful for counselling families.

Neonatal stomas: does a separate incision avoid complications and a full laparotomy at closure?

PURPOSE: Enterostomy formation is a common outcome in emergency neonatal laparotomy. No consensus exists regarding optimal stoma site. This study aims to identify incidence of complications and closure details related to position of stomas. METHODS: This study is a retrospective case note review of emergency neonatal enterostomy formation over 11 years at a single institution. Patients were separated into two groups: stomas created through the laparotomy wound and stomas created through a separate incision. Demographic details, complications and closure details were ascertained. Differences between groups were analysed (Mann-Whitney test for continuous variables, Chi-squared test or Fisher's exact test for categorical variables). RESULTS: One hundred and thirteen stoma formations were examined in 106 patients (71 within laparotomy wound, 42 through a separate incision). Age, gestation, weight, wound-related and stoma-related complications were not significantly different between the groups. A trend towards a higher rate of full laparotomy at closure with stomas through the wound (p = 0.09) was seen. If stomas were sited adjacently, there was no difference in avoidance of full laparotomy at closure (p = 0.97). CONCLUSION: Stomas sited adjacently within the laparotomy wound are not related to increased complications and offer the same advantage of circumexcision at closure as stomas sited through a separate wound, without an additional abdominal wound.

Screening of testicular descent in older boys is worthwhile: an observational study.

BACKGROUND: Testicular descent in boys is now routinely screened only once, at 6-8 weeks of age. Early surgery for undescended testes is recommended. AIM: To assess the value of screening for testicular descent at 6-8 weeks, 8-9 months, and 39-42 months of age. DESIGN OF STUDY: Observational study. SETTING: Royal Hospital for Sick Children, Glasgow and the Scottish community-based Child Health Surveillance Programme. METHOD: Screening data for boys undergoing surgery for abnormal testicular descent between April 2006 and September 2007 was reviewed. The main outcome measure was median age at first operation for abnormal testicular descent comparing attendance at screening with non-attendance. RESULTS: Boys who attended screening underwent surgery at a significantly younger median age than boys who did not attend screening at 6-8 weeks (2.7 versus 7.7 years; P<0.001); 8-9 months (4.5 versus 9.7 years; P<0.001); and 39-42 months (7.8 versus 10.8 years; P = 0.014). A new diagnosis was made in 33% (42 of 128 boys) at 6-8 weeks, 28% (21/74) at 8-9 months, and 39% (15/38) at 39-42 months. Detection on screening did not always trigger referral. Referral was triggered by screening in 48% (62/128) of cases, and by incidental examinations in 27% (34/128). CONCLUSION: The previous screening regimen was effective, but checks at 8-9 months and 39-42 months have recently been abolished. Reinstatement of screening for testicular descent in older boys is advocated because screened boys underwent surgery at a younger age. Doctors should be encouraged to check testicular descent in boys throughout childhood, and refer promptly when there is any concern.

Bilious vomiting in the newborn: how often is further investigation undertaken?

BACKGROUND/PURPOSE: Pediatric surgeons consider bile vomiting in the neonate a potential surgical emergency. The reported rate of surgical intervention is 30% to 40%, but as most neonates are born outwith pediatric surgical centers, referral of these babies is at the neonatologists' discretion. The aim of this study was to determine the referral policy of neonatologists in the West of Scotland for a neonate with bile vomiting. METHODS: Questionnaires were sent to all neonatologists in the West of Scotland to determine the management plan for a neonate with a single bile vomit or repeated bile vomits. Respondents were asked to indicate whether they would advocate postnatal ward observation, admission to the special care baby unit, abdominal x-ray, or upper gastrointestinal contrast study, or refer to pediatric surgeons. Respondents were asked to prioritize these options numerically. RESULTS: A return rate of 81% was achieved. Most neonatologists (80%) would admit a neonate with a single bile vomit to the special care baby unit, but more than 50% did not consider an upper gastrointestinal contrast study appropriate. One third felt that pediatric surgical referral is not appropriate for a single bile vomit. In a neonate with persistent bile vomiting, pediatric surgical referral was considered the highest priority. CONCLUSION: Neonatologists use a policy of observation for neonates with a single bile vomit. Those neonates with no further bile vomiting are unlikely to be referred. Pediatric surgeons are not referred a significant proportion of neonates that vomit bile.

Extracorporeal life support - state of the art.

Extracorporeal life support (ECLS) has become an accepted therapeutic measure in the treatment of infants, children and adults with reversible respiratory or cardiac failure. The principle behind ECLS involves obtaining access to drain blood from the venous circulation into the extracorporeal circuit where it is oxygenated and cleansed of carbon dioxide before being returned to the circulation. The UK Collaborative ECMO Trial showed that an ECLS policy was clinically effective in terms of improved survival without a rise in severe disability at age 1 year. Long-term follow-up has confirmed these benefits. The value of ECLS in paediatric and, more recently, adult respiratory failure is becoming clearer. ECLS has a vital role to play in the support of paediatric cardiac surgery programmes. Recent advances include newer oxygenators, greater use of less invasive veno-venous support and the use of ECLS to support novel therapies used to treat severe congenital diaphragmatic hernia.