Development of a gastroschisis core outcome set

Objective Outcome reporting heterogeneity impedes identification of gold standard treatments for children born with gastroschisis. Use of core outcome sets (COSs) in research reduces outcome reporting heterogeneity and ensures that studies are relevant to patients. The aim of this study was to develop a gastroschisis COS. Design and setting Systematic reviews and stakeholder nomination were used to identify candidate outcomes that were subsequently prioritised by key stakeholders in a three-phase online Delphi process and face-to-face consensus meeting using a 9-point Likert scale. In phases two and three of the Delphi process, participants were shown graphical and numerical representations of their own, and all panels scores for each outcome respectively and asked to review their previous score in light of this information. Outcomes were carried forward to the consensus meeting if prioritised by two or three stakeholder panels in the third phase of the Delphi process. The COS was formed from outcomes where ≥70% of consensus meeting participants scored the outcome 7–9 and <15% of participants scored it 1–3. Results 71 participants (84%) completed all phases of the Delphi process, during which 87 outcomes were assessed. Eight outcomes, mortality, sepsis, growth, number of operations, severe gastrointestinal complication, time on parenteral nutrition, liver disease and quality of life for the child, met criteria for inclusion in the COS. Conclusions Eight outcomes have been included in the gastroschisis COS as a result of their importance to key stakeholders. Implementing use of the COS will increase the potential for identification of gold standard treatments for the management of children born with gastroschisis

Oesophageal atresia with no distal tracheoesophageal fistula: Management and outcomes from a population-based cohort.

Purpose To describe the incidence and outcomes to one-year in infants born with oesophageal atresia (OA) with no distal tracheoesophageal fistula within a population cohort. Method A prospective multicentre population cohort study was undertaken of all infants born with OA investigating clinical outcomes up to one year following initial surgery. Outcomes of infants with OA and a lower pouch fistula have previously been reported. A subgroup analysis describing the outcomes of infants with OA and no tracheoesophageal fistula, (Type A) and those with only an upper pouch fistula, (Type B) was performed. Main Results Twenty-one of 151 infants were diagnosed with Type A or B oesophageal atresia (14%). Fifteen were Type A (71%) and 6 Type B (29%). With the exception of an infant with Type A, who died before reconstruction; all but four infants (all Type B) underwent more than one operation. Median time to delayed primary anastomosis in infants with Type A and Type B was 82 days (75-89 days) (n=7). The median time to oesophageal replacement was 94 days (89-147 days) (n=8). Median length of stay for infants with Type A or B OA from first operation to first discharge was 101 days (31-123 days). Conclusions Oesophageal atresia with no distal tracheoesophageal fistula is uncommon. Infants with</p

One-year outcomes following surgery for necrotising enterocolitis: a UK-wide cohort study

Objective: The objective was to describe outcomes and investigate factors affecting prognosis at one-year post-intervention for infants with surgical NEC. Design: Utilising the British Association of Paediatric Surgeons Congenital Anomalies Surveillance System, we conducted a prospective, multi-centre cohort study of every infant reported to require surgical intervention for NEC in the UK and Ireland between 01/03/2013 and 28/02/2014. Association of independent variables with one-year mortality was investigated using multivariable logistic regression analysis. Setting: All 28 paediatric surgical centres in the UK and Ireland. Patients: Infants were eligible for inclusion if they were diagnosed with NEC and deemed to require surgical intervention, regardless of whether that intervention was delivered. Outcomes: Primary outcome was mortality within one-year of the decision to intervene surgically. Results: 236 infants were included in the study. 208(88%) had one-year follow-up. 59 of the 203 infants with known survival status(29%,95%CI 23%-36%) died within one year of the decision to intervene surgically. Following adjustment, key factors associated with reduced one-year mortality included older gestational age at birth(aOR0.87,95%CI 0.78-0.96). Being small for gestational age (SGA) (aOR3.6,95%CI 1.4-9.5), and requiring parenteral nutrition at 28-days post-decision to intervene surgically (aOR3.5,95%CI 1.1-11.03) were associated with increased one-year mortality. Conclusions: Parents of infants undergoing surgery for NEC should be counselled that there is approximately a 1:3 risk of death in the first post-operative year, but that the risk is lower for infants who are of greater gestational age at birth, who are not SGA, and who do not require parenteral nutrition at 28-days post-intervention. </p

Oesophageal atresia with no distal tracheoesophageal fistula: Management and outcomes from a population-based cohort.

Purpose To describe the incidence and outcomes to one-year in infants born with oesophageal atresia (OA) with no distal tracheoesophageal fistula within a population cohort. Method A prospective multicentre population cohort study was undertaken of all infants born with OA investigating clinical outcomes up to one year following initial surgery. Outcomes of infants with OA and a lower pouch fistula have previously been reported. A subgroup analysis describing the outcomes of infants with OA and no tracheoesophageal fistula, (Type A) and those with only an upper pouch fistula, (Type B) was performed. Main Results Twenty-one of 151 infants were diagnosed with Type A or B oesophageal atresia (14%). Fifteen were Type A (71%) and 6 Type B (29%). With the exception of an infant with Type A, who died before reconstruction; all but four infants (all Type B) underwent more than one operation. Median time to delayed primary anastomosis in infants with Type A and Type B was 82 days (75-89 days) (n=7). The median time to oesophageal replacement was 94 days (89-147 days) (n=8). Median length of stay for infants with Type A or B OA from first operation to first discharge was 101 days (31-123 days). Conclusions Oesophageal atresia with no distal tracheoesophageal fistula is uncommon. Infants with</p

Systematic review and meta-analysis comparing outcomes following orchidopexy for cryptorchidism before or after 1 year of age

Background Current guidelines recommend orchidopexy for cryptorchidism by 12 months of age, yet this is not universally adhered to. The aim of this systematic review and meta-analysis was to compare outcomes between orchidopexies performed before and after 1 year of age. Methods MEDLINE and Embase were searched (September 2015) using terms relating to cryptorchidism, orchidopexy and the outcomes of interest. Studies were eligible for inclusion if they compared orchidopexy at less than 1 year of age (early) with orchidopexy at 1 year or more of age (delayed) and reported the primary outcome (testicular atrophy) or one of the secondary outcomes (fertility potential, postoperative complication, malignancy). Studies were excluded when more than 50 per cent of infants had intra-abdominal testes, or the population included infants with disorders of sexual differentiation. Additional studies were identified through reference list searching. Unpublished data were sought from the ORCHESTRA study investigators. Results Fifteen eligible studies were identified from 1387 titles. There was no difference in atrophy rate between early orchidopexy and delayed orchidopexy (risk ratio 0⋅64, 95 per cent c.i. 0⋅25 to 1⋅66; 912 testes). Testicular volume was greater (mean difference 0⋅06 (95 per cent c.i. 0⋅01 to 0⋅10) ml; 346 testes) and there were more spermatogonia per tubule (mean difference 0⋅47 (0⋅31 to 0⋅64); 382 testes) in infants undergoing early orchidopexy, with no difference in complication rate (risk ratio 0⋅68, 0⋅27 to 1⋅68; 426 testes). No study reported malignancy rate. Conclusions Atrophy and complication rates do not appear different between early and delayed orchidopexy, and fertility potential may be better with early orchidopexy. Imprecision of the available data limits the robustness of these conclusions.</p

One-year outcomes following surgery for necrotising enterocolitis: a UK-wide cohort study

Objective: The objective was to describe outcomes and investigate factors affecting prognosis at one-year post-intervention for infants with surgical NEC. Design: Utilising the British Association of Paediatric Surgeons Congenital Anomalies Surveillance System, we conducted a prospective, multi-centre cohort study of every infant reported to require surgical intervention for NEC in the UK and Ireland between 01/03/2013 and 28/02/2014. Association of independent variables with one-year mortality was investigated using multivariable logistic regression analysis. Setting: All 28 paediatric surgical centres in the UK and Ireland. Patients: Infants were eligible for inclusion if they were diagnosed with NEC and deemed to require surgical intervention, regardless of whether that intervention was delivered. Outcomes: Primary outcome was mortality within one-year of the decision to intervene surgically. Results: 236 infants were included in the study. 208(88%) had one-year follow-up. 59 of the 203 infants with known survival status(29%,95%CI 23%-36%) died within one year of the decision to intervene surgically. Following adjustment, key factors associated with reduced one-year mortality included older gestational age at birth(aOR0.87,95%CI 0.78-0.96). Being small for gestational age (SGA) (aOR3.6,95%CI 1.4-9.5), and requiring parenteral nutrition at 28-days post-decision to intervene surgically (aOR3.5,95%CI 1.1-11.03) were associated with increased one-year mortality. Conclusions: Parents of infants undergoing surgery for NEC should be counselled that there is approximately a 1:3 risk of death in the first post-operative year, but that the risk is lower for infants who are of greater gestational age at birth, who are not SGA, and who do not require parenteral nutrition at 28-days post-intervention. </p

Development of a gastroschisis core outcome set

Objective Outcome reporting heterogeneity impedes identification of gold-standard treatments for children born with gastroschisis. Use of core outcome sets (COS) in research reduces outcome reporting heterogeneity and ensures that studies are relevant to patients. The aim of this study was to develop a gastroschisis COS. Design and Setting Systematic reviews and stakeholder nomination were used to identify candidate outcomes that were subsequently prioritised by key stakeholders in a three-phase online Delphi process and face-face consensus meeting using a nine-point Likert scale. In phases two and three of the Delphi process, participants were shown graphical and numerical representations of their own, and all panels scores for each outcome respectively and asked to review their previous score in light of this information. Outcomes were carried forward to the consensus meeting if prioritised by two or three stakeholder panels in the third phase of the Delphi process. The COS was formed from outcomes with consensus meeting scores ≥70% 7–9 and Results 71 participants (84%) completed all phases of the Delphi process, during which, 87 outcomes were assessed. Eight outcomes, mortality, sepsis, growth, number of operations, severe gastrointestinal complication, time on parenteral nutrition, liver disease and quality of life for the child met criteria for inclusion in the COS. Conclusions Eight outcomes have been included in the gastroschisis COS as a result of their importance to key stakeholders. Implementing use of the COS will increase the potential for identification of gold standard treatments for the management of children born with gastroschisis.</p

Development of a gastroschisis core outcome set

Objective Outcome reporting heterogeneity impedes identification of gold-standard treatments for children born with gastroschisis. Use of core outcome sets (COS) in research reduces outcome reporting heterogeneity and ensures that studies are relevant to patients. The aim of this study was to develop a gastroschisis COS. Design and Setting Systematic reviews and stakeholder nomination were used to identify candidate outcomes that were subsequently prioritised by key stakeholders in a three-phase online Delphi process and face-face consensus meeting using a nine-point Likert scale. In phases two and three of the Delphi process, participants were shown graphical and numerical representations of their own, and all panels scores for each outcome respectively and asked to review their previous score in light of this information. Outcomes were carried forward to the consensus meeting if prioritised by two or three stakeholder panels in the third phase of the Delphi process. The COS was formed from outcomes with consensus meeting scores ≥70% 7–9 and &lt;15% 1-3. Results 71 participants (84%) completed all phases of the Delphi process, during which, 87 outcomes were assessed. Eight outcomes, mortality, sepsis, growth, number of operations, severe gastrointestinal complication, time on parenteral nutrition, liver disease and quality of life for the child met criteria for inclusion in the COS. Conclusions Eight outcomes have been included in the gastroschisis COS as a result of their importance to key stakeholders. Implementing use of the COS will increase the potential for identification of gold standard treatments for the management of children born with gastroschisis.</p

Outcomes at five to eight years of age for children with Hirschsprung’s disease

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 NETS1HD 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

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