Pediatric surgery

The complementation Q group (FANCQ) subtype of Fanconi anemia (FA) caused by the ERCC4/XPF mutation is very rare. Two siblings, aged 13 and 10 with Fanconi phenotypic features, presented with right hemiparesis and focal-onset seizures. In both cases, cranial magnetic resonance imaging (MRI) showed mass-like lesions accompanied by peripheral edema and calcification. In one case, oral steroid treatment and surgical excision were performed, while in the other case, the cranial lesion regressed just with steroid treatment and without surgery...

PURPOSE: To examine the distribution and characteristics of pediatric foot fractures on radiographs with respect to age and skeletal maturation, and to identify predictors of surgery. METHODS: This retrospective study included children (≤ 18 years) with foot fractures, who underwent radiographic examinations (2020-2022). Electronic medical records were reviewed to obtain demographic and clinical data. Fracture characteristics, including anatomic location, presence of displacement, angulation, articular involvement, and, if skeletally immature, physeal involvement and Salter-Harris fracture pattern were collected...

Antifibrinolytics have gained increasing attention in minimizing blood loss and mitigating the risks associated with massive transfusions, including infection and coagulopathy in pediatric patients undergoing spine surgery. Nevertheless, the selection of optimal agent is still a matter of debate. We aim to review the utility of these agents and compare the efficacy of antifibrinolytics in pediatric and adolescent spine surgeries. A comprehensive search was performed in Scopus, Web of Science, and MEDLINE databases for relevant works...

The use of endoscopic third ventriculostomy (ETV) for treatment of pediatric hydrocephalus has higher failure rates in younger patients. Here we investigate the impact of select perioperative variables, specifically gestational age, chronological age, birth weight, and surgical weight, on ETV failure rates. A retrospective review was performed on patients treated with ETV - with or without choroid plexus cauterization (CPC) - from 2010 to 2021 at a large academic center. Analyses included Cox regression for independent predictors and Kaplan-Meier survival curves for time to-event outcomes...

BACKGROUND: Periventricular pediatric low-grade gliomas (pLGG) present a surgical challenge due to their deep-seated location, accessibility, and relationship with the subcortical network connections. Minimally invasive parafascicular approaches with tubular brain retractors (port brain surgery) have emerged, in recent years, as an alternative to conventional microsurgical and endoscopic approaches for removal of periventricular tumors. OBJECTIVES: To describe the minimally invasive approach with tubular brain retractors for periventricular pLGG, its technique, applications, safety, and efficacy...

INTRODUCTION: Structured emergency room concepts have been shown to contribute to patient safety. Until now there has been no uniform emergency room concept for critically ill and seriously injured children and adolescents in the emergency room at the Altona Children's Hospital in Hamburg. This concept has been newly developed in interdisciplinary cooperation and includes the use of new clinical premises as well as new responsibilities and team compositions. The introduction of new processes and rooms for handling emergencies is associated with a risk of overlooking latent safety deficiencies or detecting them only after the process has been implemented...

BACKGROUND: Duodenal ulcer (DU) causes various symptoms in children. The prevalence of Helicobacter pylori (Hp)-associated DU has been reducing in some regions, yet the updated trend in Taiwan is unknown. Risk factors of DU recurrence have not been comprehensively investigated in children. METHODS: This retrospective study included children diagnosed with DU to evaluate the demographics, symptoms, diagnostics, treatment, and outcomes. Specific populations (infant, surgery required) were sorted for subgroup analysis...

OBJECTIVES: Central venous catheterization is used widely in critical pediatric patients. The authors sought to compare the success rate and safety of ultrasound-guided subclavian vein cannulation performed via infraclavicular and supraclavicular approaches. DESIGN: The authors compared the success rate of the first puncture and other information for cannulation in the children with congenital heart disease requiring central venous catheterization who were assigned randomly to the supraclavicular approach group (group A) or infraclavicular approach group (group B)...

BACKGROUND: Epilepsy, a globally prevalent neurological condition, presents distinct challenges in management, particularly for focal-onset types. This study aimed at addressing the current challenges and perspectives in focal epilepsy management, with focus on the Italian reality. METHODS: Using the Delphi methodology, this research collected and analyzed the level of consensus of a panel of Italian epilepsy experts on key aspects of focal epilepsy care. Areas of focus included patient flow, treatment pathways, controlled versus uncontrolled epilepsy, follow-up protocols, and the relevance of patient-reported outcomes (PROs)...

PURPOSE: The potential of combining teledentistry and engaging parents as underutilised resources to monitor paediatric dental health was emphasised during the COVID-19 pandemic and remains underexplored. This study aims to assess parental acceptance and use of a commercially available intraoral camera (IOC) for effective remote monitoring. METHODS: 47 child-parent dyads, where the parent was the main caregiver and the child was treated under general anaesthesia for early childhood caries, were recruited...

BACKGROUND: Biliary intraepithelial neoplasia (BilIN), a noninvasive precursor of cholangiocarcinoma, can manifest malignant transformation. Since cholangiocarcinoma (CCA) may progress due to chronic inflammation in the bile ducts and gallbladder, choledochal cysts are considered a precursor to CCA. However, BilIN has rarely been reported in children, to date. METHODS: We reviewed medical records of patients (< 18 years of age, n = 329) who underwent choledochal cyst excision at Asan Medical Center from 2008 to 2022...

BACKGROUND: Myopericytoma is a rare spindle cell tumor of mesenchymal origin, typically benign, characterized by concentric proliferation of tumor cells around blood vessels within subcutaneous tissue. It primarily occurs in middle-aged adults and is often located in distal extremities, although cases have been reported in proximal extremities and head-neck regions. However, occurrences within the oral cavity are exceedingly rare. To date, literature reviews have identified only two cases in children under 10 years old and reported only five cases of myopericytoma occurring in the lip region...

BACKGROUND: Desmoplastic fibroma is an extremely rare primary bone tumor. Its characteristic features include bone destruction accompanied by the formation of soft tissue masses. This condition predominantly affects individuals under the age of 30. Since its histology is similar to desmoid-type fibromatosis, an accurate diagnosis before operation is difficult. Desmoplastic fibroma is resistant to chemotherapy, and the efficacy of radiotherapy is uncertain. Surgical excision is preferred for treatment, but it entails high recurrence...

Surgical management of pediatric Crohn's disease is fundamentally palliative, aiming to treat the sequalae of complicated disease while preserving intestinal length. Multidisciplinary discussion of risk factors and quality of life should take place prior to operative intervention. Though the surgical management of pediatric Crohn's disease is largely based on the adult literature, there are considerations specific to the pediatric population - notably disease and treatment effects on growth and development...

BACKGROUND: Both bone forearm fractures (BBFFs) are a common injury amongst the pediatric population. The main indications of surgical fixation are open, irreducible, or unstable fractures. The two most commonly used surgical techniques are closed or open reduction with intramedullary fixation (IMF) and open reduction with plate fixation (PF). The aim of this systematic review and meta-analysis was to determine which fixation method is superior for BBFFs. METHODS: PubMed, Scopus, Web of Science, and CENTRAL were searched to identify studies comparing IMF and PF...

BACKGROUND: Appendectomy remains a common pediatric surgical procedure with an estimated 80,000 operations performed each year. While prior work has reported the existence of racial disparities in postoperative outcomes, we sought to characterize potential income-based inequalities using a national cohort. METHODS: All non-elective pediatric (<18 years) hospitalizations for appendectomy were tabulated in the 2016-2020 National Inpatient Sample. Only those in the highest ( HI ) and lowest income ( LI ) quartiles were considered for analysis...

BACKGROUND: Long-term urinary outcomes after anorectal malformation (ARM) repair are affected by surgical approach and sacral anomalies. This study aimed to compare laparoscopic-assisted anorectoplasty (LAARP) and posterior sagittal anorectoplasty (PSARP) in terms of urinary complications. METHODS: Between 2001 and 2022, 45 patients were treated with LAARP or PSARP. The rectourethral fistula and inflow angle between the fistula and rectum was confirmed by preoperative colonography...

BACKGROUND AND HYPOTHESIS: The potential role of anesthesia as an independent risk factor for childhood bipolar disorder (BD) remains unclear. To address this, we conducted a population-based cohort study employing propensity score matching to compare BD incidence between pediatric patients undergoing surgery with and without general anesthesia. STUDY DESIGN: Our study included patients aged 0-3 years who received at least 1 episode of general anesthesia and were hospitalized for over 1 day in Taiwan between January 2004 and December 2014...

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Magnetic resonance imaging-guided laser interstitial thermal therapy (MRIgLITT) has been proven safe and effective for the treatment of focal epilepsy of different etiologies. It has also been used to disconnect brain tissue in more extensive or diffuse epilepsy, such as corpus callosotomy and hemispherotomy. In this study, we report a case of temporo-parieto-occipital disconnection (TPO) surgery performed using MRIgLITT assisted by a robotic arm for refractory epilepsy of the posterior quadrant. A highly realistic cadaver simulation was performed before the actual surgery...