Treatment and outcomes in pediatric inflammatory myofibroblastic tumors - A systematic review of published studies.

Inflammatory myofibroblastic tumor (IMT) is a soft tissue neoplasm which can be locally invasive, recur, or in rare cases metastasize. Often originating from the abdomen or thorax, IMT most commonly affects children and young adults. Due to its rarity comprehensive reports detailing clinical management and outcome(s) are sparse and often based on limited index case numbers. This study systematically analyzes outcome metrics of pediatric IMT and identifies risk factors for mortality. Medline/Embase databases were searched in accordance with PRISMA guidelines. Final analysis included 57 studies with 673 IMT patients (355 males, 53 %). Individual patient data was available for 405 cases with a median follow-up period of 36 months. Tumor sites included abdomen/pelvis (n = 233, 58 %), thorax (n = 125, 31 %), head/neck (n = 34, 8 %), and extremities (n = 13, 3 %). Surgical tumor resection was the mainstay of treatment, while only 20 patients (5 %) were treated non-operatively. Recurrence(s) were reported in 80 patients (20 %) with 34 (12 %) requiring reoperation. Positive tumor margins were a significant risk factor for tumor recurrence (p < 0.0001). Chemo/radiotherapy was reported in 98 patients (25 %). Most patients (94 %) survived; 81 % (n = 237) with no evidence of recurrent disease, 14 % (n = 41) were alive with disease, and 25 (6 %) died of disease. Positive margins at primary operation, and metastatic disease were associated with mortality (p < 0.0001 for both). IMT is a rare tumor with favorable outcome for the majority of patients. Whilst most patients will present with benign tumors, complete surgical resection (R0) is crucial, as positive surgical margins are a significant risk factor for tumor recurrence and mortality.

Major surgical conditions of childhood and their lifelong implications: comprehensive review.

BACKGROUND: In recent decades, the survival of children with congenital anomalies and paediatric cancer has improved dramatically such that there has been a steady shift towards understanding their lifelong health outcomes. Paediatric surgeons will actively manage such conditions in childhood and adolescence, however, adult surgeons must later care for these 'grown-ups' in adulthood. This article aims to highlight some of those rare disorders encountered by paediatric surgeons requiring long-term follow-up, their management in childhood and their survivorship impact, in order that the adult specialist may be better equipped with skills and knowledge to manage these patients into adulthood. METHODS: A comprehensive literature review was performed to identify relevant publications. Research studies, review articles and guidelines were sought, focusing on the paediatric management and long-term outcomes of surgical conditions of childhood. The article has been written for adult surgeon readership. RESULTS: This article describes the aforementioned conditions, their management in childhood and their lifelong implications, including: oesophageal atresia, tracheo-oesophageal fistula, malrotation, short bowel syndrome, duodenal atresia, gastroschisis, exomphalos, choledochal malformations, biliary atresia, Hirschsprung disease, anorectal malformations, congenital diaphragmatic hernia, congenital lung lesions and paediatric cancer. CONCLUSION: The increasing survivorship of children affected by surgical conditions will translate into a growing population of adults with lifelong conditions and specialist healthcare needs. The importance of transition from childhood to adulthood is becoming realized. It is hoped that this timely review will enthuse the readership to offer care for such vulnerable patients, and to collaborate with paediatric surgeons in providing successful and seamless transitional care.

Should Pediatric Surgical Oncologists Receive Training in Vascular Surgery ? A Practice Survey Report From the International Society of Paediatric Surgical Oncology (IPSO).

BACKGROUND: Is vascular training in paediatric surgical oncology considered desirable ? METHODS: A voluntary survey of work practice was undertaken with the surgeon membership of The International Society Of Paediatric Surgical Oncology (IPSO) using a structured designed questionnaire. RESULTS: A total of 149 IPSO surgeon members completed the survey. 57% (N = 84) of surgeons surveyed had no specific training in vascular surgery. 43% surgeons (N = 63) stated they had acquired some skills in residency training and/or with transplantation surgery. 65% (N = 96) of respondent surgeons stated that vascular surgical training must be incorporated into pediatric surgical oncology training and 27% (N = 40) agreed that it was considered desirable. 89% (N = 133) of surgeon respondents had encountered major vascular injury during work practice while operating on pediatric solid tumors. Vascular injury repairs were undertaken and attempted by pediatric surgeons though expert assistance of vascular surgeons proved crucially essential in many instances. Emergent operations included patch repairs, vessel ligation techniques and insertion of vascular graft prostheses. Interventional radiology services to arrest life-threatening hemorrhage were also reportedly utilized by respondents. CONCLUSION: Vascular injuries have significant potential for devastating patient outcomes including never event 'mortality'. The IPSO surgeon survey highlights that there are visible 'gaps' in skills training. Training to be a pediatric oncology surgeon must incorporate acquisition of skill sets proficiency in vascular surgery.

International Society of Paediatric Surgical Oncology (IPSO): A Short History, Developments, and Current Status.

The International Society of Paediatric Surgical Oncology (IPSO) was officially inaugurated in 1991 through the creativeness and inspiration of a collective dynamic group of paediatric surgeons committed to advancing childhood cancer. This article traces the origins and birth of IPSO tracking its modern day development to a growing world community of paediatric surgeon oncology members. LEVEL OF EVIDENCE: 5.

Incidence and Risk Factors for Chyle Leaks After Neuroblastic Tumor Resection: A Systematic Review of Published Studies.

BACKGROUND: Chyle leakage/ascites after surgical resection of neuroblastic tumors may delay the start of chemotherapy and worsen prognosis. Previous studies have reported a highly variable incidence and risk factors remain largely unknown. This study aims to analyze the true incidence of chyle leaks and ascites and seeks to identify risk factors and optimal treatment strategies. METHODS: Medline/Embase databases were searched according to PRISMA guidelines. Literature reviews, case reports, and non-English papers were excluded. Data were extracted independently following paper selection by 2 authors. RESULTS: The final analysis yielded 15 studies with N = 1468 patients. Chylous ascites was recorded postoperatively in 171 patients (12%). Most patients experiencing chyle leaks were successfully treated conservatively with drainage, bowel rest, parenteral nutrition and octreotide with variable combinations of these treatment options. 7/171 (4%) patients required operative exploration to control troublesome persistent chyle leaks. In risk factor analysis, higher tumor stage was significantly associated with the risk of chyle leak (P < 0.0001) whereas no correlation was observed with adrenal vs non-adrenal tumor location, INRG risk groups and tumor laterality. CONCLUSION: Chyle leakage after surgery for neuroblastic tumors is a common morbid complication occurring in some 12% of patients. Higher INSS tumor stage portends greater risk(s). Conservative therapy strategies appear successful in the majority of cases. To avert this complication meticulous mesenteric lymphatic ligation is recommended especially for those patients with higher tumor stage(s) requiring extensive radical surgery including retroperitoneal lymph node resection. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Systematic review.

Female Fertility Cryopreservation Outcomes in Childhood Cancer: A Systematic Review.

BACKGROUND AND AIMS: As survival rates in childhood cancer progress significantly, health outcomes in adulthood are pivotal to quality of life (QoL). Female patients undergoing chemotherapy and radiation for childhood cancer may experience adverse effects such as gonadotoxicity-related ovarian insufficiency. Ovarian tissue cryopreservation (OTC) is well studied in adults, but has only recently started to be explored in an effort to preserve fertility in young patients with childhood cancer. This systematic review aims to critically highlight contemporary outcomes of cryopreservation in female pediatric cancer patients. METHODS: A systematic search was conducted in PubMed, Embase, and Web of Science databases to identify English-language full text articles and abstracts published between 2004 and 2022 describing cryopreservation among female children (0-21 years old) with cancer. Abstracts and full-text articles were screened for inclusion. Subsequently, data from eligible studies was extracted and analyzed. Descriptive statistics were utilized to estimate overall outcomes of cryopreservation. RESULTS: Of 104 abstracts and 34 full-text articles, 12 studies were included. Data was collected from 7 world countries and involved some 612 pediatric and adolescent patients with malignant disease. Most common cancers included hematological malignant disease (81%), CNS nervous system malignant tumors (56%), and sarcomas (39%). Of the 6 studies with full reporting, OTC was undertaken in 501 patients, and 5.9% (30/501) of these patients underwent ovarian tissue transplantation (OTT). After OTT, 27 patients desired pregnancy and 33% (9/27) became pregnant. Six of these 9 patients (67%) had live births. CONCLUSIONS: Preliminary analysis showed that OTC has been successfully performed but not yet studied thoroughly in pediatric cancer patients in a longitudinal manner. This study has further shown that cryopreservation outcomes are mainly reported among adult patients living in high income countries, demonstrating a crucial need for long-term outcome studies focused on pediatric and prepuberal OTC, subsequent OTT, and potential pregnancy. This work is considered critical to aid standardize recommendations of fertility preservation in childhood cancer patients and to better inform the efficacy of these procedures to benefit patients in world nations of all fiscal income levels. LEVEL OF EVIDENCE: Level III.

Do children with Osteosarcoma benefit from Pulmonary Metastasectomy? - A Systematic Review Of Published Studies and 'Real world' Outcomes.

OBJECTIVE: To critically examine the evidence-base for survival benefit of Pulmonary metastasectomy (PM) for Osteosarcoma (OS) in the paediatric population. SUMMARY BACKGROUND DATA: PM for OS is recommended as standard of care in both paediatric and adult treatment protocols. Recent results from the PulMiCC trial demonstrate no survival benefit from Pulmonary Metastasectomy (PM) in Colorectal Cancer in adults. METHODS: A systematic review was undertaken according to PRISMA guidelines. Medline, Embase and 2 clinical trials registers were searched for ALL STUDIES detailing paediatric OS patients (<18 y) undergoing PM with a comparison cohort group that did not receive PM. RESULTS: Eleven studies met inclusion criteria dating from 1984 - 2017. All studies were retrospective and none directly compared PM versus No PM in paediatric patients as its main objective(s). Three-year survival rates ranged from 0-54% for PM and 0-16% for no PM. No PM patients were usually those with unresectable disease and/or considered to have a poor prognosis. All studies were at high risk of bias and there was marked heterogeneity in the patient selection. CONCLUSIONS: There is a weak evidence-base (Level IV) for a survival benefit of PM for OS in paediatric patients likely due to selection bias of 'favourable cases'. The included studies many of which detailed outdated treatment protocols were not designed in their reporting to specifically address the questions directly. A randomised controlled trial - whilst ethically challenging in a paediatric population - incorporating modern OS chemotherapy protocols is needed to crucially address any 'survival benefit'.

Developing a core outcome set for the health outcomes for children and adults with congenital oesophageal atresia and/or tracheo-oesophageal fistula: OCELOT task group study protocol.

INTRODUCTION: Heterogeneity in reported outcomes of infants with oesophageal atresia (OA) with or without tracheo-oesophageal fistula (TOF) prevents effective data pooling. Core outcome sets (COS) have been developed for many conditions to standardise outcome reporting, facilitate meta-analysis and improve the relevance of research for patients and families. Our aim is to develop an internationally-agreed, comprehensive COS for OA-TOF, relevant from birth through to transition and adulthood. METHODS AND ANALYSIS: A long list of outcomes will be generated using (1) a systematic review of existing studies on OA-TOF and (2) qualitative research with children (patients), adults (patients) and families involving focus groups, semistructured interviews and self-reported outcome activity packs. A two-phase Delphi survey will then be completed by four key stakeholder groups: (1) patients (paediatric and adult); (2) families; (3) healthcare professionals; and (4) researchers. Phase I will include stakeholders individually rating the importance and relevance of each long-listed outcome using a 9-point Likert scale, with the option to suggest additional outcomes not already included. During phase II, stakeholders will review summarised results from phase I relative to their own initial score and then will be asked to rescore the outcome based on this information. Responses from phase II will be summarised using descriptive statistics and a predefined definition of consensus for inclusion or exclusion of outcomes. Following the Delphi process, stakeholder experts will be invited to review data at a consensus meeting and agree on a COS for OA-TOF. ETHICS AND DISSEMINATION: Ethical approval was sought through the Health Research Authority via the Integrated Research Application System, registration no. 297026. However, approval was deemed not to be required, so study sponsorship and oversight were provided by Alder Hey Children's NHS Foundation Trust. The study has been prospectively registered with the COMET Initiative. The study will be published in an open access forum.

Image-guided core-needle or surgical biopsy for neuroblastoma diagnosis in children: A systematic review and meta-analysis from the International Society of Pediatric Surgical Oncology (IPSO).

BACKGROUND: Image-guided core-needle biopsy (IGCNB) is a widely used and valuable clinical tool for tissue diagnosis of pediatric neuroblastoma. However, open surgical biopsy remains common practice even if children undergo more invasive and painful procedures. This review aims to determine the diagnostic accuracy and safety of IGCNBs in pediatric patients with neuroblastoma. METHODS: We conducted a systematic review of peer-reviewed original articles published between 1980 and 2023, by searching "pediatric oncology," "biopsy," "interventional radiology," and "neuroblastoma." Exclusion criteria were patients older than 18 years, studies concerning non-neurogenic tumors, case reports, and language other than English. Both the systematic review and meta-analysis were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. RESULTS: A total of 533 abstracts articles were analyzed. Of these, eight retrospective studies met inclusion criteria (490 infants, 270 surgical biopsies [SB], 220 image-guided biopsies). Tissue adequacy for primary diagnosis (SB: n = 265, 98%; IGCNB: n = 199, 90%; p = .1) and biological characterization (SB: n = 186, 95%; IGCNB: n = 109, 89%; p = .15) was similar with both biopsy techniques, while intraoperative transfusion rate (SB: n = 51, 22%; IGCNB: n = 12, 6%; p = .0002) and complications (%) (SB: n = 58, 21%; IGCNB: n = 14, 6%; p = .005) were higher with surgical biopsy. Length of stay was similar in both groups; however, no additional data about concurrent diagnostic or treatment procedures were available in the analyzed studies. CONCLUSIONS: IGCNB is a safe and effective strategic approach for diagnostic workup of NB and should be considered in preferance to SB wherever possible.

Acute pancreatitis in childhood - a comparative international study and tale of two cities.

BACKGROUNDS: To compare factor(s) contributing to aetiology, management and clinical outcome(s) of paediatric patients acquiring acute pancreatitis (AP) at two major university paediatric surgical centres in Liverpool and Bangkok. METHODS: All patients (<18 years) with an index diagnosis of AP (ICD 10 coding) during 2006-2016 were studied. RESULTS: 121 patients included n = 79 (65.3%) in Thailand versus n = 42 (34.7%) in the UK centre with no difference(s) in age at diagnosis at 10.4 ± 4.5 and 11.7 ± 6 years. (P = 0.12). Major AP aetiology(s) in Thailand were medications (39.2%) and choledochal cysts (8.9%). In the UK-gallstone disease (21.4%), and medications (16.7%) were leading factors (P < 0.01). Ultrasonography was deployed more frequently in the UK versus Thai centre (74.3% vs. 49.1%; P < 0.01). Pancreatitis was confirmed by imaging in 67.9% (Thai) and 62.9% (UK) patients (P = 0.47). Most patients at both centres had a mild-grade pancreatitis illness (95% Thai vs. 90.5% UK; P = 0.28) while 12.7% of Thai and 19% of UK children developed pancreatitis-related complication (P = 0.37). Overall mortality rate (%) was significantly higher in the Thai versus UK centre (27.8% vs. 9.5%; P = 0.02). CONCLUSIONS: Aetiology of acute pancreatitis appears to vary between UK and Thailand children. Timely early diagnosis and healthcare pathways may be driven by local patient-related factor(s). The higher mortality (%) observed in Thailand versus UK in this comparative study was linked to underlying co-existent chronic medical condition(s) in vulnerable patient cohorts.

Pre and postoperative diarrhoea associated with neuroblastoma resection - A systematic review of published studies.

Neuroblastoma is a malignant tumour affecting 10.5/1 million children annually. It arises from sympathetic nervous system precursor cells and is most frequently found in the adrenal gland and abdominal paravertebral ganglia. Diarrhoea as a presenting symptom of neuroblastoma is uncommon and usually linked to vasoactive intestinal peptide (VIP) tumour secretion. Even more rarely, postoperative diarrhoea may follow neuroblastoma tumour resection. Published studies generally associate postoperative diarrhoea with subadventitial tumour resection. These findings are however based on a handful of reports. This systematic review therefore aims to analyse the true incidence of postoperative diarrhoea and its morbid correlation with the extent/type of surgical resection. Pubmed/Embase databases were searched according to PRISMA guidelines. Final analysis consisted of 16 studies: N = 779 patients. Postoperative diarrhoea was significantly more common in all patients who underwent subadventitial resection compared to non subadventitial resection, p < 0.001 (OR 25.9, 95% CI 9.3-72.4). 5-year survival rates were equivalent in both groups. Preoperative diarrhoea was rarely reported in studies and always strongly linked to elevated VIP secretion. In the majority of neuroblastoma patients, preoperative diarrhoea ameliorated after gross tumour resection with elevated VIP normalized. The operative technique of subadventitial neuroblastoma resection portends significant risk(s) of post operative diarrhoea not seen in those patients undergoing other classical methods of tumour resection with 5-year survival rates strikingly similar. These findings affirm that subadventitial tumour resection should be avoided when undertaking surgery for neuroblastoma to minimize the risk(s) of persistent postoperative diarrhoea.

Ovarian-sparing surgery for ovarian tumors in children: A systematic review and meta-analysis.

INTRODUCTION: An increased number of children and adolescents with ovarian tumors have been managed with ovarian-sparing surgery in the last few years. However, comprehensive data on fertility outcomes and local relapse are scarce. In this study, we systematically describe the contemporary outcomes of ovarian-sparing surgery, as reported in the literature. MATERIALS AND METHODS: Using PRISMA guidelines, we analyzed studies reporting ovarian-sparing techniques for ovarian tumors in children and adolescents. from 1980 to 2022. Reports with fewer than three patients, narrative reviews, and opinion articles were excluded. Statistical analysis was performed for dichotomous and continuous variables. RESULTS: Of 283 articles screened, 16 papers (3057 patients) met inclusion criteria (15 retrospective/1 prospective) and were analyzed. The vast majority of studies had no long-term fertility follow-up data and direct comparison between ovarian-sparing surgery vs oophorectomy was reported in only a few studies. Ovarian sparing surgery was not associated with worse oncologic outcomes in terms of (i) tumour spillage or (ii) recurrence rates, and of key importance allowed a higher ovarian reserve at long term follow-up. CONCLUSIONS: Ovarian-sparing surgery is a safe and feasible technique for benign tumors. Long-term outcome studies are needed to show efficacy and fertility preservation.

COllaborative Neonatal Network for the first European CPAM Trial (CONNECT): a study protocol for a randomised controlled trial.

INTRODUCTION: Consensus is lacking on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM). For future studies, the CONNECT consortium (the COllaborative Neonatal Network for the first European CPAM Trial)-an international collaboration of specialised caregivers-has established consensus on a core outcome set of outcome parameters concerning respiratory insufficiency, surgical complications, mass effect and multifocal disease. These outcome parameters have been incorporated in the CONNECT trial, a randomised controlled trial which, in order to develop evidence-based practice, aims to compare conservative and surgical management of patients with an asymptomatic CPAM. METHODS AND ANALYSIS: Children are eligible for inclusion after the CPAM diagnosis has been confirmed on postnatal chest CT scan and they remain asymptomatic. On inclusion, children are randomised to receive either conservative or surgical management. Subsequently, children in both groups are enrolled into a standardised, 5-year follow-up programme with three visits, including a repeat chest CT scan at 2.5 years and a standardised exercise tolerance test at 5 years.The primary outcome is exercise tolerance at age 5 years, measured according to the Bruce treadmill protocol. Secondary outcome measures are molecular genetic diagnostics, validated questionnaires-on parental anxiety, quality of life and healthcare consumption-, repeated imaging and pulmonary morbidity during follow-up, as well as surgical complications and histopathology. This trial aims to end the continuous debate surrounding the optimal management of asymptomatic CPAM. ETHICS AND DISSEMINATION: This study is being conducted in accordance with the Declaration of Helsinki. The Medical Ethics Review Board of Erasmus University Medical Centre Rotterdam, The Netherlands, has approved this protocol (MEC-2022-0441). Results will be disseminated through peer-reviewed scientific journals and conference presentations. TRIAL REGISTRATION NUMBER: NCT05701514.

Sacrococcygeal teratoma: Long-term outcomes. A UK CCLG Surgeons Group Nationwide Study.

AIM: Sacrococcygeal teratoma (SCT) is a rare paediatric germ cell tumour (1:40,000). Long-term data regarding urinary tract and bowel function after SCT resection is limited to few studies. A UK Children's Cancer and Leukaemia Group (CCLG) Surgeons multicentre study aimed to critically analyse long-term functional outcomes in patients following resection of SCT. METHODS: Nationwide study of UK paediatric surgical oncology centres using a standardised data collection form. All index cases of newborn infants and children <16 years with SCT diagnosis during 2005-2015 were included. RESULTS: 165 SCT patients treated at 14 UK paediatric surgical oncology centres were included. Median age at presentation was 1 day [interquartile range, IQR: 0-25]; median age at surgery was 10 days [IQR: 4-150]. One hundred seventeen (70%) were female and 48 (30%) male. Antenatal diagnosis was made in 44% index cases. Total 59% of patients were Altman Stage I or II lesions. Follow-up data were available in 83% cases. Tumour recurrence occurred in 13 (7%) patients at median age 13 months [IQR: 8.75-30 months]. Fifty-nine (36%) of 165 patients had documented adverse bladder or bowel dysfunction. Twenty-two (37%) cases required urinary clean intermittent catheterisation (CIC) urology health care, with eight patients (14%) needing operative intervention to control management of bowel dysfunction. CONCLUSION: This UK CCLG study showed 36% of SCT patients develop bladder or bowel dysfunction after primary tumour resection. Functional assessment of bladder and bowel function is mandatory during after-care follow-up of all SCT patients. A multidisciplinary care pathway, with surgeon speciality groups including surgical oncology, paediatric urology and paediatric colorectal specialists, is strongly advised to facilitate 'best practice' monitoring of long-term health and improve patient quality of life (QoL) into adulthood.

Long-term functional outcomes of sacrococcygeal teratoma - A systematic review of published studies exploring 'real world' outcomes.

Sacrococcygeal teratoma (SCT) is a rare neoplasm affecting 1:35,000 newborns. Long-term follow-up from small observational studies report impaired bladder/bowel function. This current study comprehensively analyses all published studies to better define true long-term functional sequelae. Medline/Embase databases were searched with PRISMA guidelines. Final analysis yielded 37 studies involving 1116 patients (854 female; 77%). Individual datasets were available in 14 studies (222 patients). According to Altman classification - 298/845 (35%) were Type I, 252/845 (30%) Type II, 133/845 (16%) Type III, and 128/845 (15%) Type IV tumours. Most neoplasms were benign (640/858; 75%), 77/858 (9%) immature and 141/858 (16%) malignant. Abnormal bladder function was reported in 7/39 (18%) Altman Type I, 23/61 (37.7%) Type II, 11/34 (32.4%) Type III, and 15/25 (60%) Type IV cases (p = 0.007). Adverse urological outcomes were notably common in immature/malignant neoplasms vs benign tumours and in patients requiring reoperation(s); p = 0.002 and p = 0.01. Bowel dysfunction was evident in 19% index cases and constipation in 26% with no significant association(s) with tumour characteristics. Higher Altman stage, unfavourable tumour biology and reoperation are associated with poor functional outcome(s). Multidisciplinary management from primary diagnosis of SCT is crucially important for all patients to best optimise functional outcomes across surgical specialities.

Acute Pancreatitis in Childhood: A 10-Year Experience From A Thai University Surgical Center.

OBJECTIVES: This study aimed to describe etiology, management, and health outcomes of children developing acute pancreatitis at a tertiary Thailand pediatric surgery center. METHODS: Medical case records of all index cases during 2006-2016 were analyzed and reported. RESULTS: There were 42 male and 37 female patients, with a mean (standard deviation) age of 10.4 (4.5) years, included in the study. Medications were the commonest etiology for 39.3% of acute pancreatitis attacks, 11.4% for biliary tract disease cases, and 8% for postinterventional studies. In 30% of cases, no cause(s) was defined. Sixty-two patients (78.5%) had elevated serum lipase on hospital admission, whereas only 30.4% showed a raised amylase. Hospital stay was 15 days (interquartile range, 6-27 days). Two major complications in the series were pseudocysts (8.8%) and necrotizing pancreatitis (7.6%). Etiological factors and/or antibiotics were not directly linked to any specific complications. Seventeen children (22.8%) had 1 recurrent episode of acute pancreatitis documented. Mortality rate in index cases was 28%, with a higher percentage harboring a preexisting illness (34.4% vs 5.6%; P = 0.01) and in male than in female patients (41% vs 14%; P = 0.01). CONCLUSIONS: Deaths from pediatric acute pancreatitis are more prevalent in male individuals and those with a preexisting illness. Targeted strategies aimed at "highest-risk" patients may potentially offset mortality.

Thyroid cancer in children: A multicenter international study highlighting clinical features and surgical outcomes of primary and secondary tumors.

Background: Thyroid gland malignancies are rare in pediatric patients (0.7% of tumors); only 1.8% are observed in patients aged <20 years, with a higher prevalence recorded in women and adolescents. Risk factors include genetic syndromes, MEN disorders, autoimmune diseases, and exposure to ionizing radiation. Radiotherapy is also associated with an increased risk of secondary thyroid cancer. This study describes the clinical features and surgical outcomes of primary and secondary thyroid tumors in pediatric patients. Methods: Institutional data were collected from eight international surgical oncology centers for pediatric patients with thyroid cancer between 2000 and 2020. Statistical analyses were performed using the GraphPad Prism software. Results: Among 255 total cases of thyroid cancer, only 13 (5.1%) were secondary tumors. Primary thyroid malignancies were more likely to be multifocal in origin (odds ratio [OR] 1.993, 95% confidence interval [CI].7466-5.132, p = 0.2323), have bilateral glandular location (OR 2.847, 95% CI.6835-12.68, p = 0.2648), and be metastatic at first diagnosis (OR 1.259, 95% CI.3267-5.696, p > 0.999). Secondary tumors showed a higher incidence of disease relapse (OR 1.556, 95% CI.4579-5.57, p = 0.4525) and surgical complications (OR 2.042, 95% CI 0.7917-5.221, p = 0.1614), including hypoparathyroidism and recurrent laryngeal nerve injury. The overall survival (OS) was 99% at 1 year and 97% after 10 years. No EFS differences were evident between the primary and secondary tumors (chi-square 0.7307, p = 0.39026). Conclusions: This multicenter study demonstrated excellent survival in pediatric thyroid malignancies. Secondary tumors exhibited greater disease relapse (15.8 vs. 10.5%) and a higher incidence of surgical complications (36.8 vs. 22.2%).

Postoperative feeding in neonatal duodenal obstruction.

BACKGROUND: Findings from manometry studies and contrast imaging reveal functioning gastric physiology in newborns with duodenal atresia and stenosis. Stomach reservoir function should therefore be valuable in aiding the postoperative phase of gastric feeding. The aim of this study was therefore to compare the feasibility of initiating oral or large volume(s) gavage feeds vs small volume bolus feeds following operation for congenital duodenal anomalies. METHODS: Single-center electronic medical records of all babies with duodenal atresia and stenosis admitted to a university surgical center during January 1997-September 2021 were analyzed. A fast-fed group (FF) included newborns fed with oral or gavage feeds advanced at a rate of at least 2.5 ml/kg and then progressed more than once a day vs slow-fed group (SF) fed with gavage feeds at incremental rate less than 2.5 ml/kg/day for each time period of oral tolerance or by drip feeds. Total feed volume was limited to 120-150 ml/kg/day in the respective study cohort populations. RESULTS: Fifty-one eligible patients were recruited in the study - twenty-six in FF group and twenty-five in SF group. Statistically significant differences were observed in the (i) date of first oral feeds (POD 7.7 ± 3.2 vs 16.1 ± 7.7: p < 0.001), and (ii) first full feeds (POD 12.5 ± 5.3 vs 18.8 ± 9.7: p < 0.01) in FF vs SF study groups. CONCLUSION: Initial feeding schedules with oral or incremental gavage-fed rates of at least 2.5 ml/kg in stepwise increments and multi-steps per day is wholly feasible in the postoperative feeding regimens of neonates with congenital duodenal disorders. Significant health benefits are thus achievable in these infants allowing an earlier time to acquiring full enteral feeding and their hospital discharge.

The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey.

Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers. Proposed outcome parameters were scored according to level of importance, and the final COS was established through consensus. A total of 55 participants (33 surgeons, 22 non-surgeons) from 28 centers in 13 European countries completed the three rounds and rated 43 outcome parameters. The final COS comprises seven outcome parameters: respiratory insufficiency, surgical complications, mass effect/mediastinal shift (at three time-points) and multifocal disease (at two time-points). The seven outcome parameters included in the final COS reflect the diversity in priorities among this large group of European participants. However, we recommend the incorporation of these outcome parameters in the design of future studies, as they describe measurable and validated outcomes as well as the accepted age at measurement.