Development of a New Score Based on Image Defined Risk Factors to Standardize Surgical Risk in Neuroblastoma Resection - A SIOPEN Collaborative Study.

Background And Aims: Image Defined Risk Factors (IDRFs) assess surgical risk in neuroblastoma (NB) and guide neoadjuvant therapy. Despite chemotherapy IDRFs may persist in 70 % of cases. Several studies have suggested that not all IDRFs hold equal significance and that the presence of an IDRF does not inherently signify unresectability.

Congenital heart disease and arrhythmia disorders in newborns with congenital diaphragmatic hernia: a 23-year experience at a UK university pediatric surgical centre.

Purpose: Congenital diaphragmatic hernia (CDH) is associated with congenital heart disease (CHD) and index newborns reportedly may experience cardiac arrhythmia disorders [Tella et al.-Pediatric Critical Care Medicine 2022]. This study analyses, details and reports contemporary outcome metrics of CHD and cardiac rhythm disease (CRD) in CDH babies attending a university surgical centre.

Peter Paul Rickham: the Liverpool neonatal surgery unit 1953.

This article highlights the evolution, birth and legacy of the world's first neonatal surgical unit established at Alder Hey Children's Hospital Liverpool in 1953. Peter Paul Rickham, a creative pioneering and innovative surgeon, is credited here as a major driving force that helped shape and progress the modern day development of neonatal surgery. Rickham's vision was realised by studying neonatal surgical disorders and the mortality rate of congenital anomalies in Mersey Region while working as a young senior registrar with Isabella Forshall.

Management of Ganglioneuroma and Ganglioneuroblastoma Intermixed: A United Kingdom Children's Cancer and Leukaemia Group (UK CCLG) Nationwide Study Report.

Background: Ganglioneuroblastoma intermixed (GNBi) and ganglioneuroma (GN) represent benign variants of peripheral neuroblastic tumours. While historically surgical resection was recommended, watchful active observation has become the accepted management for a subset of patients.

Objectives: To review clinical features, biology, natural history and management of a retrospective UK CCLG study cohort of GN and GNBi, and compare outcomes of patients treated with surgical resection or watchful active observation.

Preoperative Identification of Adamkiewicz Artery in Pediatric Posterior Thoracic Tumors: Fact or Fiction? A Systematic Review from the International Society of Pediatric Surgical Oncology (IPSO).

Background And Aims: We aimed to review current literature on the impact of Preoperative Identification (POI) of the Adamkiewicz Artery (AKA) in solid pediatric Posterior Thoracic Tumors (PTT), comprising a spectrum of neuroblastic tumors and neuroblastoma, with particular focus on Complete Macroscopic Excision (CME) and Neurologic Complications/Sequelae (NCS).

Methods: A systematic review in accordance with PRISMA guidelines was undertaken. The study included reports on pediatric patients providing available data on POI of the AKA in PTT.

Utility of the oxygenation index in management of congenital diaphragmatic hernia: a report from a Thai University Surgical Centre.

Background: Oxygenation index (OI) is associated with severity of newborn pulmonary hypertension (PH) in congenital diaphragmatic hernia (CDH). Higher OI may indicate worst degree(s) of PH.

Objectives: This study reports OI dynamic(s) over the first 72 h of life and its correlation with (1) perioperative morbidity and (2) CDH mortality.

Paediatric diffuse oesophageal leiomyomatosis with Alport syndrome.

Diffuse oesophageal leiomyomatosis is a rare benign disease in the paediatric population. This report highlights a recent clinical case, together with a narrative review of current world literature.An early middle childhood girl with recurrent lower respiratory tract infections for 2 years was noted to have a retrocardiac lesion on chest X-ray, later confirmed to be an oesophageal mass on CT imaging.

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.

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.

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.

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.

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.

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.

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 pediatric population.

Background: PM for OS is recommended as the standard of care in both pediatric and adult treatment protocols. Recent results from the "Pulmonary Metastasectomy in Colorectal Cancer" trial demonstrate no survival benefit from PM in colorectal cancer in adults.

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.

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.

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.

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.

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.

Beyond image defined risk factors (IDRFs): a delphi survey highlighting definition of the surgical complexity index (SCI) in neuroblastoma.

Purpose: Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs do not all carry the same weighting in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize a surgical complexity (Surgical Complexity Index, SCI) in NB resection.

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.

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.

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.

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.