Search

Objective: To compare validity indices of the King-Devick (KD) test and Sport Concussion Assessment Tool 5 (SCAT5) for traumatic events in MMA, and to determine if perfusion events (alterations in consciousness as the result of choke holds) cause similar changes in KD/SCAT5 scores. Design: A prospective cohort study in MMA fighters who completed KD and SCAT5 assessments before and after a match. Outcomes were categorized as non-event, traumatic event, or perfusion event. KD/SCAT5 changes were compared between all athletes. Participants: One hundred forty MMA athletes (7 women, 133 men), mean age=27.1 ± 4.9 years. Intervention: N/A Main outcome measures: King-Devick (KD) test and Sport Concussion Assessment Tool 5 (SCAT5) Results: Among the 140 athletes, 19 sustained traumatic and 15 perfusion events. Testing provided sensitivities/specificities of 21.05%/93.39% (KD) and 77.78%/52.99% (SCAT5) in detecting a traumatic event. KD and SCAT5 Symptom Severity scores differed between athletes with and without traumatic events (P=.041 and .014). KD and SCAT5 Symptoms Score changes were observed between athletes with and without traumatic events (P=.023 and .042). Neither KD nor SCAT5 differed significantly between athletes with and without perfusion events. Conclusions: The KD test provides high specificity and the SCAT5 demonstrates reasonable sensitivity when detecting a traumatic event. Of the SCAT5, symptoms-related scores may most effectively identify a traumatic event. A traumatic event may cause KD/SCAT5 changes similar to a concussion, while perfusion events did not.

ڐ: This survey study was conducted to better consider ways that the Society of Maternal-Fetal Medicine (SMFM) can increase their international outreach. Most survey respondents indicated that international representation could be improved and cited barriers of cost and physical distance to annual meetings. This study highlights support for continuation of virtual conferencing to improve international representation and proposes a hybrid model of scientific engagement moving forward.

Aims: To undertake a prospective, single blind, randomised trial comparing the efficacy and tolerance of two outpatient colonoscopy bowel preparation regimens.Methods: Patients aged between 18 months and 16 years being admitted for day case colonoscopy were allocated randomly to receive either Picolax (an oral, sugar free powder containing sodium picosulphate 10 mg/sachet with magnesium citrate) and clear fluids or bisacodyl tablets with an unrestricted diet and a phosphate enema just before colonoscopy. Patient compliance, bowel frequency, and associated symptoms were recorded, and the adequacy of the bowel preparation was assessed in a blinded manner.Results: 63 of 66 patients completed the trial. Mean age, mean weight, extent of colonoscopy, and distribution of underlying pathology were similar in both groups. Bowel preparation was good or excellent in all of the patients in the Picolax group (n = 32) compared with 22 patients in the bisacodyl/phosphate enema group (n = 31). The latter group experienced more abdominal discomfort during bowel preparation but three of the Picolax group vomited and the lack of solid food distressed some children.Conclusions: All bowel preparation methods have limitations and unpleasant side effects but the use of Picolax and clear fluids proved superior to bisacodyl tablets and a phosphate enema in children undergoing day case colonoscopy. [ABSTRACT FROM AUTHOR]

Aims: To assess the long term morbidity and quality of life in survivors of gastroschisis.Design: All babies born with gastroschisis between 1972 and 1984 and who survived more than one year were identified. Those who could be traced were questioned about their general health, growth, abdominal symptoms, cosmetic concerns, education, employment, and fertility.Results: Of the 35 patients, two have died, seven could not be traced, and three declined to be interviewed. Twenty three subjects (70% of survivors) with a median age of 16 years (range 12-23 years) responded. Twenty two (96%) were in good health and overall growth was within normal limits. Eight subjects (35%) have had further surgery related to gastroschisis, including two for adhesive small bowel obstruction and three for scar complications. In 13 (57%), absence of an umbilicus caused distress during childhood.Conclusion: Most gastroschisis survivors can eventually expect normal growth and good health. Adhesive bowel obstruction is an uncommon, but potentially late, complication. The umbilicus should be conserved during gastroschisis repair. [ABSTRACT FROM AUTHOR]

STUDY DESIGN.: A qualitative and semiquantitative study of the morphology of the human thoracolumbar transversospinal (TSP) muscles. OBJECTIVE.: To further define the functional morphology of the thoracolumbar TSP muscles. SUMMARY OF BACKGROUND DATA.: The TSP muscle group plays an important role in vertebral function but few studies have rigorously investigated their morphology throughout the thoracolumbar region and details on the location of motor endplates (MEPs) and fiber types are sparse. METHODS.: Thoracolumbar TSP muscles were examined by microdissection in five cadavers (seven sides). MEPs were identified using acetylcholinesterase histochemistry in muscles between T5 and S4 unilaterally in two cadavers. The relative proportions of type I and type II skeletal muscle fibers were determined using immunohistochemistry on whole cross sections of every TSP muscle from one side of one cadaver (T5-S4). RESULTS.: TSP morphology was homogeneous and consistent throughout the thoracolumbar region. Notable differences to standard descriptions included: (1) consistent attachments between muscles; (2) no discrete cleavage planes between muscles; and (3) attachment sites over the sacrum and to lumbar zygapophysial joints. Previously undescribed small muscles were found attaching to the medial sacrum. All TSP muscles were multipennate, with fibers arranged in parallel having one MEP per muscle fiber. Muscles were highly aerobic (mean proportion of type I fibers 89%), with the proportion of type I fibers decreasing caudally. A significantly greater proportion of type I fibers were found in the midthoracic compared to the low lumbar regions. CONCLUSION.: The complex morphology of the TSP muscles indicates that they would be better classified as spinotransverse muscles. They are multipennate, highly aerobic, with fibers organized in parallel, an arrangement lending itself to 'fine-tuning' of vertebral movements. Understanding their morphology has implications for investigation, treatment, motor control, and biomechanics. [ABSTRACT FROM AUTHOR]

Objective: To develop a list of tests or treatments frequently used in pediatric rheumatology practice that may be unnecessary based on existing evidence., Methods: A Choosing Wisely (CW) working group composed of 16 pediatric rheumatologists, 1 allied health professional, 1 parent, and 1 patient used the Delphi method to generate, rank, and refine a list of tests and treatments that may be unnecessary or harmful. The items with the highest content agreement and perceived impact were presented in a survey to all Canadian Rheumatology Association (CRA) physicians who practice pediatric rheumatology. Respondents were asked to rate their agreement and impact, and to rank the items. Five items with the highest composite scores and 2 additional items selected by the CW working group were put forward for literature review., Results: The initial Delphi procedure generated 80 items. After 3 rounds, the list was narrowed to 13 items. The survey was completed by 41/81 (51%) CRA pediatric members across Canada. Respondent characteristics were similar to those of the CRA pediatric membership for self-reported gender, geographical location, and career stage. The highest composite score items were antinuclear antibody testing, drug toxicity monitoring, HLA-B27 testing, rheumatoid factor/anticyclic citrullinated peptide testing, and Lyme serology testing. Two additional items (numerous or repeated intraarticular corticosteroid injections, and autoinflammatory diseases genetic testing) were also selected. Literature review was performed for these 7 highest priority items., Conclusion: We have identified areas for quality improvement in the evaluation and treatment of rheumatic diseases in Canadian children.

Objective: We aimed to evaluate the rate of depressive and/or anxiety symptoms in adolescents with juvenile idiopathic arthritis (JIA) and to explore the association with demographic and disease activity measures., Methods: Depressive and anxiety symptoms were assessed in adolescents with JIA aged 12 to 18 years at a Canadian tertiary care hospital, using the Revised Child Anxiety and Depression Scale (RCADS). The RCADS includes 6 subscales: separation anxiety, social phobia, generalized anxiety, panic disorder, obsessive-compulsive, and major depressive disorder. Scores above clinical threshold on the RCADS subscales indicate that an individual's responses reflect symptoms similar to those diagnosed with the corresponding mental health disorder. Fisher exact test and Mann-Whitney U test were used to compare demographic and disease-related variables between participants who scored above and below clinical threshold on each of the subscales., Results: There were 32/80 (40%) of participants who scored above clinical threshold on at least 1 subscale. Scores above clinical threshold were most frequent for major depressive disorder (23.8%) and panic disorder (22.5%) subscales. Social phobia and separation anxiety followed with 16.3% and 13.8%, respectively. Females were more likely to have scores above clinical threshold on the panic disorder subscale. Participants with higher self-reported disease activity were more likely to have scores above clinical threshold for all anxiety subscales except separation anxiety., Conclusion: We report high rates of symptoms of depression and anxiety (panic in particular) in adolescents with JIA. This highlights the ongoing need for mental health screening protocols and services. The relationships between concomitant mental health disorders, disease activity, and patient-reported outcomes requires further research., (Copyright © 2023 by the Journal of Rheumatology.)

Objective: To evaluate microRNA expression in synovial fluid (SF), plasma, and leukocytes from patients with juvenile idiopathic arthritis (JIA)., Methods: MicroRNA expression in pooled JIA plasma and SF was assessed by absolute quantitative droplet digital PCR array. The results were validated in individual patient samples. MicroRNA content in leukocytes and extracellular vesicles was evaluated by real-time PCR in JIA blood and SF. Blood microRNA expression was compared with healthy controls (HCs). Principal component analysis was used to profile JIA plasma and SF microRNAs, and the potential biological consequences of microRNA dysregulation were investigated by pathway analysis., Results: MiR-15a-5p and miR-409-3p levels were higher in JIA plasma than in HC plasma. JIA SF contained elevated levels of miR-21-5p, miR-27a-3p, miR-146b-5p, miR-155-5p, and miR-423-5p, and decreased miR-192-5p and miR-451a, compared to JIA plasma. Extracellular vesicle analysis demonstrated variable encapsulation among selected microRNAs, with only miR-155-5p being represented substantially in extracellular vesicles. SF leukocytes also had higher expression of miR-21-5p, miR-27a-3p, miR-146b-5p, and miR-155-5p, and lower expression of miR-409-3p and miR-451a, relative to blood. No differences were observed between JIA and HC blood leukocytes. Clusters of microRNAs were commonly altered in JIA joint fluid and leukocytes compared to JIA blood samples. In silico analysis predicted that differentially expressed microRNAs in JIA target the transforming growth factor (TGF)-β pathway., Conclusion: The expression of multiple microRNAs is dysregulated in JIA both locally and systemically, which may inhibit the TGF-β pathway. These findings advance our knowledge of JIA immunopathogenesis and may lead to the development of targeted therapies., (Copyright © 2023 by the Journal of Rheumatology.)

The transition from pediatric to adult care is the focus of growing research. It is important to identify how to direct future research efforts for maximum effect. Our goals were to perform a scoping review of the transition literature, highlight gaps in transition research, and offer stakeholder guidance on the importance and feasibility of research questions designed to fill identified gaps. The transition literature on rheumatic diseases and other common pediatric-onset chronic diseases was grouped and summarized. Based on the findings, a survey was developed and disseminated to pediatric rheumatologists and young adults with rheumatic diseases as well as their caregivers. The transitional care needs of patients, healthcare teams, and caregivers is well described in the literature. While various transition readiness scales exist, no longitudinal posttransfer study confirms their predictive validity. Multiple outcome measures are used alone or in combination to define a successful transition or intervention. Multimodal interventions are most effective at improving transition-related outcomes. How broader health policy affects transition is poorly studied. Research questions that ranked highest for importance and feasibility included those related to identifying and tracking persons with psychosocial vulnerabilities or other risk factors for poor outcomes. Interventions surrounding improving self-efficacy and health literacy were also ranked highly. In contrast to healthcare teams (n = 107), young adults/caregivers (n = 23) prioritized research surrounding improved work, school, or social function. The relevant transition literature is summarized and future research questions prioritized, including the creation of processes to identify and support young adults vulnerable to poor outcomes., (Copyright © 2022 by the Journal of Rheumatology.)

Aim: To investigate the frequency and modality of pre-operative imaging in children with an external angular dermoid cyst and whether this influenced subsequent management., Methods: A retrospective study was undertaken to assess the presentation, investigation, treatment and outcomes in children aged less than 16 years with an external angular dermoid cyst. All children who underwent external angular dermoid cyst excision between January 2008 and April 2021 at a regional children's hospital were analysed., Results: Sixty-one patients (32 boys) were identified. Fifty-four were managed by paediatric surgeons and seven by ophthalmic surgeons. Pre-operative imaging was performed in 19 (31%) patients, including plain radiographs (3), ultrasound scan (14), computerised tomography scan (1), and magnetic resonance imaging scan (2). None of these investigations showed evidence of an intracranial or orbital extension of the cyst or changed management. All cysts were excised under day-case general anaesthesia. There was one minor postoperative complication and two recurrences., Conclusions: External angular dermoid cysts rarely communicate through the bone with the orbit or anterior cranial fossa. Routine pre-operative imaging is unnecessary unless there are clinical features such as an atypical location (e.g. temporal), eye signs (e.g. strabismus, proptosis and globe displacement) or a draining sinus., (© 2022 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Minimal information exists on the length of the child's esophagus, namely the distance from the cricopharyngeus to the esophagogastric junction (EGJ). We aimed to investigate the relationship between esophageal length (EL) and the age, height, and weight of the child. Children undergoing upper gastrointestinal endoscopy between February 2019 and May 2021 at our institution were prospectively audited. Children with anatomical esophageal disorders were excluded. Endoscopic distances from the incisors to the cricopharyngeus and EGJ were obtained, and novel predictive equations derived to predict EL. Intra-observer agreement for endoscopic measurements showed an intra-class correlation coefficient of 0.99. A total of 290 children aged 0.4-17.3 years were included in the analysis; they were divided into a model development cohort (n = 261) and a model validation cohort (n = 29). Measured EL correlated best with height (r = 0.92) as compared to age (r = 0.90) or weight (r = 0.83). The optimal equation for predicting EL was 0.156 × height (cm) - 1.336 (adjusted R 2  = 0.841); this had a success rate of 76% in the validation cohort. The optimal equation for predicting distance from incisors to EGJ was 0.199 × height (cm) + 6.470 (adjusted R 2  = 0.889); this had a success rate of 69% in the validation cohort. This is the first study to report a predictive equation for estimating esophageal length in children. Accurate prediction of esophageal length may assist with clinical esophageal procedures in children such as nasogastric and pH probe placement., (© 2021 American Association of Clinical Anatomists.)

Objective: To develop best practice statements for the provision of virtual care in adult and pediatric rheumatology for the Canadian Rheumatology Association's (CRA) Telehealth Working Group (TWG)., Methods: Four members of the TWG representing adult, pediatric, university-based, and community rheumatology practices defined the scope of the project. A rapid literature review of existing systematic reviews, policy documents, and published literature and abstracts on the topic was conducted between April and May 2021. The review informed a candidate set of 7 statements and a supporting document. The statements were submitted to a 3-round (R) modified Delphi process with 22 panelists recruited through the CRA and patient advocacy organizations. Panelists rated the importance and feasibility of the statements on a Likert scale of 1-9. Statements with final median ratings between 7-9 with no disagreement were retained in the final set., Results: Twenty-one (95%) panelists participated in R1, 15 (71%) in R2, and 18 (82%) in R3. All but 1 statement met inclusion criteria during R1. Revisions were made to 5/7 statements following R2 and an additional statement was added. All statements met inclusion criteria following R3. The statements addressed the following themes in the provision of virtual care: adherence to existing standards and regulations, appropriateness, consent, physical examination, patient-reported outcomes, use in addition to in-person visits, and complex comanagement of disease., Conclusion: The best practice statements represent a starting point for advancing virtual care in rheumatology. Future educational efforts to help implement these best practices and research to address identified knowledge gaps are planned., (Copyright © 2022 by the Journal of Rheumatology.)

Epigastric hernias are relatively uncommon in children, and there is a paucity of literature on their incidence, presenting features, natural history, and surgical outcomes. A systematic review was conducted according to PRISMA guidelines. Articles describing the incidence, outcome, and interventions for pediatric epigastric hernias, both open and laparoscopic, were analyzed. Eight relevant articles published between 1975 and 2019 were included in the analysis. Of 81 children, 58% were females, 35% were symptomatic and 8% were multiple. All hernias contained preperitoneal fat only and were repaired using standard open surgery or laparoscopic techniques. No recurrences were recorded. In a personal series of 37 hernias in 36 children of median age 4 years, there were no recurrences; however, this series included two children with a recurrent or persistent epigastric hernia after surgery by others. Epigastric hernias in children are relatively uncommon. They typically contain only preperitoneal fat but more than a third are symptomatic. Standard open repair can be undertaken with minimal morbidity. Laparoscopic repair takes longer and provides a marginal cosmetic benefit., Competing Interests: None declared., (Thieme. All rights reserved.)

Introduction: Intestinal malrotation is life-threatening and often presents during infancy with bilious vomiting. The prevalence and presentation among extremely premature infants are unknown., Methods: We retrospectively reviewed all infants born at less than 28 weeks' gestation diagnosed with symptomatic intestinal malrotation in a tertiary neonatal intensive care unit over a 10-year period (2010-2020)., Results: Seven of 514 (1.4%) extremely premature infants developed symptomatic intestinal malrotation during this period. All were non-syndromic. In comparison, the prevalence of symptomatic intestinal malrotation in 7382 infants ≥ 28 weeks' gestation admitted during the same period was 0.2%. Intestinal malrotation was confirmed at laparotomy in all extremely premature infants and six of seven had midgut volvulus. All but one presented with marked abdominal distension; none had bilious vomiting and only three had bilious gastric aspirates. A subacute onset with non-specific features such as recurrent apnoea and bradycardia, feed intolerance, and intermittent abdominal distension was common. All infants underwent a Ladd procedure. Two required extensive bowel resection resulting in short gut syndrome and three underwent further surgery for adhesive small bowel obstruction. One patient died at 10 months of age from respiratory failure but the others were well 1-3 years later., Conclusions: Symptomatic intestinal malrotation in extremely premature infants has a relatively high prevalence. It may present with marked abdominal distension without bilious vomiting, demanding a high index of suspicion. An atypical presentation, potential alternative abdominal pathologies, coexisting comorbidities, and concerns about survival in these fragile babies may deter the surgeon despite the opportunity of a good outcome., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: This study aimed to expand knowledge about soluble low-density lipoprotein receptor-related protein 1 (sLRP1) in juvenile idiopathic arthritis (JIA) by determining associations of sLRP1 levels in nonsystemic JIA patients with clinical and inflammatory biomarker indicators of disease activity., Methods: Plasma sLRP1 and 44 inflammation-related biomarkers were measured at enrollment and 6 months later in a cohort of 96 newly diagnosed Canadian patients with nonsystemic JIA. Relationships between sLRP1 levels and indicators of disease activity and biomarker levels were analyzed at both visits., Results: At enrollment, sLRP1 levels correlated negatively with age and active joint counts. Children showed significantly higher levels of sLRP1 than adolescents (mean ranks: 55.4 and 41.9, respectively; P = 0.02). Participants with 4 or fewer active joints, compared to those with 5 or more active joints, had significantly higher sLRP1 levels (mean ranks: 56.2 and 40.7, respectively; P = 0.006). At enrollment, considering the entire cohort, sLRP1 correlated negatively with the number of active joints (r = -0.235, P = 0.017). In the entire cohort, sLRP1 levels at enrollment and 6 months later correlated with 13 and 6 pro- and antiinflammatory biomarkers, respectively. In JIA categories, sLRP1 correlations with inflammatory markers were significant in rheumatoid factor-negative polyarticular JIA, oligoarticular JIA, enthesitis-related arthritis, and psoriatic arthritis at enrollment. Higher sLRP1 levels at enrollment increased the likelihood of absence of active joints 6 months later., Conclusion: Plasma sLRP1 levels correlate with clinical and biomarker indicators of short-term improvement in JIA disease activity, supporting sLRP1 as an upstream biomarker of potential utility for assessing JIA disease activity and outcome prediction., (Copyright © 2021 by the Journal of Rheumatology.)

Objective: To conduct a needs assessment and environmental scan to support optimal transition from pediatric to adult rheumatology care in Canada., Methods: This initiative involved 3 phases: (1) a survey-based needs assessment of adult and pediatric rheumatologist members of the Canadian Rheumatology Association to identify perceived infrastructure, educational needs, and national resources to support transition care; (2) an environmental scan, through semistructured interviews, of existing rheumatology transition service care models and challenges in care delivery; and (3) a focus group to prioritize national activities., Results: The needs assessment survey was completed by 65 members, with 66% agreeing that a national approach to transition care was needed. Semistructured interviews reflecting activities at 9 transition care sites were conducted, and they identified candidate models of care, including direct transfer, progressive transfer, and shared care models. Challenges and needs experienced in these care models reflected resource and infrastructure needs, poor availability of mechanisms to support parents and youth through the transition process, and the need for evaluation to support quality improvement. The focus group and prioritization activity was attended by 26 participants, with each having the ability to cast 3 votes. "Supporting patient education for transition to adult rheumatology health care system" (n = 17 votes) and "advocacy activities to access allied health support, including funding" (n = 10 votes) emerged as the top priorities for national initiatives., Conclusion: We have identified priorities in education and advocacy for advancing transition care in Canada that require participation of pediatric and adult rheumatology providers, patients, and arthritis stakeholders in the interest of advancing transition care outcomes., (Copyright © 2021 by the Journal of Rheumatology.)

Purpose: To document the prevalence and variable appearance of gastric heterotopic pancreas (HP) in children undergoing upper gastrointestinal (GI) endoscopy., Methods: A prospective 4-year study of children undergoing flexible upper GI endoscopy in a single institution., Results: A total of 607 upper GI endoscopies were performed in 478 pediatric patients during the 4-year period. Eleven children (6 girls, 5 boys) aged 2.9 to 16.9 years had endoscopic features typical of gastric HP. All but one lesion was located in the gastric antrum and most appeared as an umbilicated submucosal nodule measuring 1-2 cm in diameter. Five of 13 children with repaired esophageal atresia (EA) and two of nine children with trisomy 21 had gastric HP. The prevalence of endoscopically visualized gastric HP in children without a history of EA or trisomy 21 was 1.1%., Conclusions: Gastric HP is present in about 1% of pediatric upper GI endoscopies. It is significantly more common in patients with EA and may also be associated with trisomy 21. Gastric HP typically appears as a single 1-2 cm antral submucosal nodule, usually with a central pit. Recognition of this lesion is important to avoid misdiagnosis and inappropriate treatment., Level of Evidence: Level II (diagnostic)., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Symphyseal pain (SP) experienced during pregnancy is a common condition that can negatively influence function and wellbeing. Despite its adverse impact on quality of life, standardised diagnostic criteria for SP as a distinct type of pelvic girdle pain (PGP) are lacking., Objectives: To develop a reliable self-administered instrument that could differentiate SP from posterior PGP in pregnant women, and ultimately be used for epidemiological or clinical purposes., Method: Qualitative data from 17 women (four focus groups) were used to develop a questionnaire. The questionnaire was tested against physical therapy diagnoses based on clinical assessment in 122 pregnant women with SP (n = 41), posterior PGP (n = 41) or no PGP (n = 40); 30 women repeated the questionnaire a day later to assess reliability. Multinomial logistic regression models were used to assess the performance of candidate items in distinguishing between the groups., Results/findings: The single questionnaire item relating to location of worst pain (diagrammatic form) is useful for differentiating SP from posterior PGP and individuals with no PGP. The worst pain location question with the addition of the Pelvic Girdle Questionnaire provides a measure of "SP with impact", and is the best combination for distinguishing SP and posterior PGP. Test-retest reliability scores were excellent., Conclusion: These findings provide new opportunities for diagnosing pregnancy-related SP, and highlight questionnaire items which best differentiate SP from posterior PGP. These items could be used in future epidemiological research, and in clinical settings as a quick, effective screening tool., Competing Interests: Declaration of competing interest None to declare., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Recent reports have recommended laparoscopic diverticulectomy for symptomatic Meckel diverticulum (MD) rather than laparoscopic-assisted extracorporeal resection. This technique may risk leaving residual ectopic mucosa leading to complications. This systematic review attempts to quantify the relative risks of both approaches. A systematic review was conducted according to PRISMA guidelines. Articles were eligible for inclusion if they reported data on the laparoscopic management of symptomatic MD in children. Eleven reports were identified, all of which were institutional retrospective studies. Pooled outcome data on 248 children showed no statistically significant difference in complications between laparoscopic diverticulectomy (n = 133) and laparoscopic-assisted segmental resection (n = 115) (3% vs. 6.1%, p = 0.39). One patient from the diverticulectomy group re-presented with recurrent bleeding necessitating segmental small bowel resection. Conclusions are limited by the number of patients and variable follow up. Short, wide MD with a height:base ratio of < 2; diverticula with thickening or ischemia at the base and those complicated by volvulus or small bowel obstruction are probably best treated by laparoscopic-assisted extracorporeal resection. For other symptomatic diverticula laparoscopic diverticulectomy is a reasonable approach with a less than 1% risk of leaving residual ectopic gastric mucosa.

Standard surgical repair of esophageal atresia/tracheoesophageal fistula (EA/TEF) is via a right posterolateral thoracotomy. A recognized complication is the later development of scoliosis. The prevalence and pathogenesis of secondary scoliosis are poorly understood. We, therefore, conducted a systematic review on this topic. All English language articles reporting incidence, outcomes and/or interventions for scoliosis in children after EA repair via thoracotomy were identified. Fourteen relevant articles published between 1969 and 2019 reporting 1338 children were included in the analysis. The aggregate prevalence of scoliosis among 937 children without congenital vertebral anomalies was 13%, but this figure varied widely between studies. Severity of scoliosis was documented in 181 children; eight children had a Cobb angle > 40° and 10 had undergone spinal surgery. The spinal curvature in affected individuals was dominantly or exclusively convex to the left. In conclusion, the reported prevalence of scoliosis varies widely but on average affects about one in eight children after open repair of EA/TEF. Most cases are mild and do not require intervention. It is currently uncertain whether secondary scoliosis is preventable by using meticulous thoracotomy techniques or thoracoscopic repair.Level of evidence IV.

Background: The aim of this study was to report the contemporary management of Hirschsprung disease (HD) in New Zealand., Methods: We undertook a national multi-centre retrospective review of all newly diagnosed cases of HD during a 16-year period (2000-2015). Demographics, genetic and syndromic associations, family history, radiology and histology results and surgical interventions were analysed., Results: A total of 246 cases (males:females 4:1) were identified, an incidence of 1:3870 live births. Short-segment disease was present in 81.7%, long-segment disease in 8.5%, total colonic aganglionosis in 6.5% and unknown in 3.3%. HD was diagnosed by 4 weeks' corrected gestational age in 67%. Thirty cases (12%) also had Trisomy 21. Fifty-three (21.5%) patients required a repeat rectal biopsy for definitive diagnosis. A contrast enema was performed in 55% and identified the transition zone with 69% accuracy. Primary pull-through surgery was undertaken in 59% (65% of short-segment cases) at a median age of 27 days; others were initially managed by a defunctioning stoma. The commonest definitive procedure was a Soave-Boley endorectal pull-through (79%) (or similar variant). During a median follow-up of 7.4 years, six (2.5%) survivors underwent a redo pull-through, 13 (5.5%) an appendicostomy, 16 (6.8%) a defunctioning stoma and 10 never had a definitive procedure. Total colonic aganglionosis was significantly more likely to be fatal (12.5% versus 0.5%, P < 0.0005) or associated with a permanent end stoma (27.5% versus 4.5%, P < 0.0005)., Conclusions: Most New Zealand born infants with short-segment HD are currently managed by primary pull-through, usually in the first months of life., (© 2020 Royal Australasian College of Surgeons.)

Purpose: The provision of neonatal intensive care to infants born at 23 or 24 weeks' gestation poses medical, surgical and ethical challenges. Gastrointestinal perforation is a well-recognized complication of preterm birth, occurring most often as a result of necrotizing enterocolitis (NEC) or spontaneous intestinal perforation (SIP). Given the risk of morbidity and mortality in these 'periviable' infants, this complication may prompt transition from active management to palliative care. In our institution, the surgical care of periviable infants with gut perforation has not been dictated by gestational age. This study reports our outcomes., Methods: A retrospective cohort analysis of integrated neonatal medical and surgical care of all infants born between 23 +0 and 24 +6 weeks' gestation admitted to a tertiary level neonatal intensive care unit (NICU) during a 16 year period (2002-2017)., Results: A total of 198 periviable neonates (73 born at 23 weeks' gestation and 125 born at 24 weeks) were admitted during the 16-year period; most were inborn with only 26 retrieved from regional centers. Twenty-six of these infants developed gut perforation: 14 SIP, 8 NEC, 3 esophageal perforation and one after reduction of an incarcerated inguinal hernia. Twelve (46%) periviable infants with gut perforation survived to discharge home, seven of whom had no/mild disability at 2-3 years corrected gestational age. Of the 198 periviable infants admitted to NICU, 116 (58%) were alive at a corrected gestational age of 2-3 years and 29 of the 56 (51%) assessed had mild or no disability., Conclusions: In the setting of combined medical and surgical care in a tertiary level NICU almost half of all periviable infants with a gut perforation survived, many with no/mild disability at 2-3 years corrected gestational age. Rigid protocols that rely on gestational age alone to guide treatment are not appropriate. These results support the contention that, when possible, extremely preterm infants should be born and cared for in units with combined medical and surgical expertise., Level of Evidence: Level III cohort study., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Epidemiological studies have suggested that there may be ethnic variations in the prevalence of Hirschsprung disease (HD) but no study has systematically investigated this issue or potential ethnic variations in the extent of aganglionosis in HD. This study aimed to investigate this in a childhood population in New Zealand., Methods: A multicentre national retrospective review was undertaken of all newly diagnosed cases of HD at each of the four paediatric surgical centres in New Zealand over a 16-year period (January 2000 to December 2015). Original histological, radiological and operative reports were obtained and analysed. Self-identified ethnicity was recorded from admission documents. Birth statistics were obtained from Statistics New Zealand., Results: A total of 246 cases of HD were identified. The prevalence of HD was 1:3790 live births for European, 1:6610 among Māori, 1:1834 among Pacific Peoples, 1:3847 among Asian and 1:5694 among Middle Eastern. The prevalence of HD was statistically significantly greater in Pacific Peoples (P < 0.0005). The proportion of children with long-segment HD was also significantly greater in Pacific and Asian populations than others (P = 0.04). These findings were not due to differences in the proportion of familial cases of HD among the different populations., Conclusions: The prevalence and phenotype of HD varies significantly between different ethnic groups within New Zealand. This may well be related to variations in the frequencies of HD-associated gene mutations within these populations., (© 2018 Royal Australasian College of Surgeons.)

Objective: To determine barriers and facilitators to the uptake of findings from the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) study into clinical care by pediatric rheumatologists (PR) and allied health professionals (AHP) caring for children with juvenile idiopathic arthritis (JIA) in Canada., Methods: PR and AHP participated in this qualitative study through telephone interviews. Interview guides were developed using the Theoretical Domains Framework and focused on the use of information from the ReACCh-Out study in the practice of counseling patients and families. A directed content analysis approach was used for coding., Results: Nineteen interviews (8 PR and 11 AHP) were completed. All PR had knowledge of the ReACCh-Out study. Three major themes were identified: (1) both groups are motivated to use information from research in clinical care; (2) volume and emotional effect of information on families are barriers; and (3) specific timepoints in care trigger providing this information. AHP had less knowledge of the ReACCh-Out study, did not feel it was their primary role to provide this information, and have a desire for more opportunity to participate in academic forums related to research., Conclusion: We have described a comprehensive overview of the barriers and facilitators perceived by healthcare providers in the translation of knowledge from JIA research into use in clinical practice. These findings provide a foundation for the development of knowledge translation strategies in the care of children with JIA and other rheumatic diseases.

Splenectomy is indicated in selected children with haemolytic anaemia. Postoperatively, thrombocytosis occurs in at least 80% and is one of the factors implicated in the development of acute portal venous thrombosis after splenectomy in adults. A literature review shows that children are also at risk of this complication, but the incidence is low. The risk is greatest in those with particularly large spleens. Laparoscopic splenectomy does not reduce the risk of this complication. Early detection and anticoagulation usually leads to successful resolution of the thrombosis and can mitigate the risk of developing cavernous transformation of the portal vein and chronic portal hypertension. Any child with severe or unexplained postoperative abdominal pain, fever and/or vomiting after splenectomy demands urgent abdominal imaging to exclude this complication. In asymptomatic individuals, a routine Doppler ultrasound scan 1 week postoperatively is advisable if they had a particularly large spleen, longer than usual duration of surgery and/or have a marked postoperative thrombocytosis. There is no evidence for routine administration of antiplatelet drugs and/or subcutaneous heparin prophylaxis in children after splenectomy, including those who develop postoperative thrombocytosis, but children with particularly large spleens may be a subset that benefit., (© 2018 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

The value of laryngotracheobronchoscopy (LTB) immediately prior to repair of esophageal atresia with or without tracheo-esophageal fistula is contentious. Currently, there is a wide range of opinion on the utility of this investigation which is reflected by huge variation in clinical practice. This review is a critical analysis of the arguments for and against performing routine LTB prior to esophageal atresia repair. Reserving LTB for selected cases only is potentially disadvantageous since it limits the surgeon's and anesthetist's familiarity with the procedure, equipment, and range of potential findings. There is sufficient evidence to suggest that routine preoperative LTB becomes the standard of care.