In recent decades lung ultrasound (LUS) has emerged as an invaluable bedside imaging tool for the evaluation of respiratory conditions such as pneumonia, bronchiolitis, pneumothorax, and pleural effusion in pediatric age, thanks to the thin chest wall and absence of rib ossification. Despite its growing application, optimizing technique and interpretation remains crucial for reliable diagnostic performance. the present educational paper provides practical tips and tricks to enhance the accuracy, efficiency, and clinical value of LUS in pediatric patients, focusing on probe selection, patient positioning, scanning technique, common artifacts, and interpretation pitfalls.

Background:Omental infarction is a rare pediatric disease. Ultrasound is a useful modality for a non-invasive pre-operative differential diagnosis between inflammatory conditions (as appendicitis) and omental infarction, especially by detecting immobility of the omentum adhered to the abdominal wall ("tetherd fat sign"). However, this is a dynamic sign that cannot be documented in a static image with B-mode technique. The goal of this work is to incorporate the versatile function of motion mode (M-mode) into omental infarction diagnosis to describe how the M-mode is useful in the evaluation of fat motion in children suspected of having omental infarction. In 2019 we suggested a new Ultrasound sign named "tethered fat sign" for an accurate non-invasive diagnosis of omental infarction in children. This finding was observed in 6 of the 234 seen children of our previous study with 4 laparoscopic confirmed diagnosis.Methods:From January 2019 to July 2021, we evaluated 195 children (91 boys and 104 girls, from 3 to 15 years) admitted to our Santobono-Pausilipon Children Hospital with acute right-sided abdominal pain. Abdominal ultrasound was performed to all the patients and the investigation of "tethered fat sign" was always included.Results:In 7 patients ultrasound showed the presence of a hyperechoic oval mass localized in the right upper abdominal quadrant and in 2 of these M-mode documented a normal subhepatic fat moving during respiratory movements in relation with the abdominal wall. The remaining 5 patients had an omental infarction showed as a subhepatic motionless mass tethered to the abdominal wall on M-mode. In these patients, a sonographic follow-up was performed every 15 d for 2 months showing a progressive reduction in size of the right-sided hyperechoic mass.Conclusions:In the evaluation of all children who showed the presence of the "tethered fat sign" the use of M-mode provide a certified image in diagnostic ultrasound.

To the Editor: Ataxia telangiectasia (AT) is a rare disorder caused by pathogenic variants in the ataxia telangiectasia-mutated (ATM) gene, characterized by progressive neurological involvement, oculocutaneous telangiectasia, variable degrees of immunodeficiency, radiosensitivity and increased susceptibility to tumors.1 The ATM kinase plays a role in detecting DNA lesions and in regulating the cell cycle progression, and its impairment is responsible for the instability of the genome and radiation sensitivity.2 Immunodeficiency may contribute to neoplasms development, particularly those associated with Epstein–Barr virus (EBV). EBV-associated smooth muscle tumor (EBV-SMT) is a rare mesenchymal tumor that generally occurs in immunocompromised individuals. To our knowledge, a single case report of EBV-SMT (laryngeal leiomyosarcoma) has been reported in the literature in a patient with AT.3 We present the case of a 9-year-old Romanian female from consanguineous parents frequently hospitalized for arthritis and pneumonia confirmed by serial chest radiographs. She was admitted to our hospital at the age of 7, for pneumonia and acute respiratory distress syndrome, displaying bulbar telangiectasias, ataxic gait, failure to thrive, and recurrent infections. Immunological evaluation revealed IgA deficiency, reduced naïve CD4 and CD8 T-cell counts, elevated alpha-fetoprotein, and sustained EBV replication (Table 1). Genetic testing identified a homozygous ATM gene deletion (c.8831_8832del).4-6 Both parents and her brother, diagnosed with acute myeloid leukemia, were heterozygous for this variant. Despite initial improvement with antibiotic prophylaxis and immunoglobulin replacement treatment, the patient continued to experience episodes of pneumonia. Clinical deterioration prompted bronchoalveolar lavage confirmed EBV pneumonia, with other microbiological tests negative. In light of persistent EBV replication, the patient received four weekly doses of rituximab. The radiosensitivity required a cautious approach to imaging. However, a computed tomography (CT) scan, considered essential due to the progressive decline in lung function, showed atelectasis in the lower left lobe and multiple bronchiectasis (Figure 1A). Her respiratory symptoms continued to worsen with frequent need for oxygen therapy despite antibiotic and asthma treatment. Lung ultrasound and magnetic resonance imaging (MRI) performed for follow-up showed fibrosis and dystelectasis in the left lower lobe (Figure 1B), leading to a recommendation for lobectomy. The histology of the removed lobe showed congestion and hepatization with extensive bronchial ectasias. Notably, a 1-cm nodular whitish mass was incidentally found (Figure 1D). Microscopic examination revealed a tumor composed of spindle cells with eosinophilic cytoplasm and oval nuclei, arranged in a fascicular pattern within a sparse stromal collagen matrix and accompanied by a lymphocytic infiltrate (Figure 1E). The surrounding lung parenchyma displayed pronounced congestion and severe inflammation, including bronchial abscesses filled with neutrophils, lymphocytic infiltration, and multiple foci of alveolar hemorrhage. The spindle cells were positive for α-smooth muscle actin (α-SMA) and negative for desmin, CD34, anaplastic lymphoma kinase (ALK, D5F3), and CD21. In situ hybridization for EBV-encoded RNA (EBER) was positive in the tumor cells, displaying nuclear localization (Figure 1F,G). These findings were diagnostic of EBV-SMT. Whole-body MRI showed no metastatic lesions or in situ macroscopic recurrence. Respiratory symptoms improved, although residual bronchiectasis persisted in the basal segments of the left lung (Figure 1C). EBV-SMT is a rare neoplasm associated with immunodeficiency, described in patients infected with human immunodeficiency virus (HIV),7 in the post-transplant setting,8 and in those with congenital immunodeficiency.9 It can manifest at any age, but rarely in pediatric patients with primary immunodeficiency. EBV-SMT typically manifests either as a single mass or multiple nodules often found in the liver, but can occur anywhere in the body.10 Although locally aggressive, metastasis are rarely reported.11 To the best of our knowledge, the literature has documented only a single case of EBV-SMT manifesting as laryngeal leiomyosarcoma in an AT patient.11 The case we present is notable for being an instance of pulmonary EBV-SMT. The differential diagnosis initially included inflammatory myofibroblastic tumor, but the presence of a monotypic T-lymphocyte infiltrate and EBER nuclear positivity suggested EBV-SMT. The pathogenesis of EBV-SMT remains a subject of scientific inquiry. The oncogenic role of EBV is well recognized, particularly in hematological malignancies.12, 13 Both EBV infection and immunodeficiency are critical factors in tumor development. In AT, thymic hypoplasia and restricted T-cell receptor repertoire led to T-cell lymphopenia mainly affecting naive T-cell impairing the control of EBV infection, allowing persistence in B cells. Additionally, the ATM protein is involved in the EBV lytic cycle, promoting its replication. However, the mechanism by which ATM mutation enhances EBV's oncogenic potential remains unclear.14 Finally, the intrinsic predisposition to cancer in AT may contribute to the pathogenesis of EBV-SMT.15 Therapeutic approach for EBV-SMT focuses on restoring T-cell function controlling EBV replication including antiretroviral therapy (ART) in HIV patients or reducing immunosuppression after transplant.9 Surgery is required for organ-compromising tumor masses. Despite the rarity of metastasis in EBV-SMT, strict follow-up is essential for timely intervention.16 Sirolimus, an mTOR-inhibitor, offers a potential therapeutic option, though its efficacy remains debated.17 In our AT patient, the coexistence of immunodeficiency (Table 1), EBV active replication, repeated chest x-rays for recurrent pneumonia, along with cancer predisposition and increased radiosensitivity may have collectively contributed to the cancer pathogenesis. In summary, EBV-SMT should be considered in the differential diagnosis of solid tumors, particularly in patients with primary immunodeficiency such as AT. Prompt diagnosis is crucial, as prognosis can be poor without appropriate treatment. We are grateful to patients and their parents, medical staff, and nurses. The authors declare that they have no conflicts of interest. Italian Ministry of Health with "Current Research funds"

Background and purpose: Differentiating pediatric posterior fossa (PF) tumors such as medulloblastoma (MB), ependymoma (EP), and pilocytic astrocytoma (PA) remains relevant, because of important treatment and prognostic implications. Diffusion kurtosis imaging (DKI) has not yet been investigated for discrimination of pediatric PF tumors. Estimating diffusion values from whole-tumor-based (VOI) segmentations may improve diffusion measurement repeatability compared to conventional region-of-interest (ROI) approaches. Our purpose was to compare repeatability between ROI and VOI DKI-derived diffusion measurements and assess DKI accuracy in discriminating among pediatric PF tumors. Materials and methods: We retrospectively analyzed 34 children (M, F, mean age 7.48 years) with PF tumors who underwent preoperative examination on a 3 Tesla magnet, including DKI. For each patient, two neuroradiologists independently segmented the whole solid tumor, the ROI of the area of maximum tumor diameter, and a small 5 mm ROI. The automated analysis pipeline included inter-observer variability, statistical, and machine learning (ML) analyses. We evaluated inter-observer variability with coefficient of variation (COV) and Bland-Altman plots. We estimated DKI metrics accuracy in discriminating among tumor histology with MANOVA analysis. In order to account for class imbalances, we applied SMOTE to balance the dataset. Finally, we performed a Random Forest (RF) machine learning classification analysis based on all DKI metrics from the SMOTE dataset by partitioning 70/30 the training and testing cohort. Results: Tumor histology included medulloblastoma (15), pilocytic astrocytoma (14), and ependymoma (5). VOI-based measurements presented lower variability than ROI-based measurements across all DKI metrics and were used for the analysis. DKI-derived metrics could accurately discriminate between tumor subtypes (Pillai's trace: p < 0.001). SMOTE generated 11 synthetic observations (10 EP and 1 PA), resulting in a balanced dataset with 45 instances (34 original and 11 synthetic). ML analysis yielded an accuracy of 0.928, which correctly predicted all but one lesion in the testing set. Conclusions: VOI-based measurements presented improved repeatability compared to ROI-based measurements across all diffusion metrics. An ML classification algorithm resulted accurate in discriminating PF tumors on a SMOTE-generated dataset. ML techniques based on DKI-derived metrics are useful for the discrimination of pediatric PF tumors.

Cystic fibrosis (CF) is a multisystem disorder characterized by progressive respiratory deterioration, significantly impacting both quality of life and survival. Over the years, lung ultrasound (LUS) has emerged as a promising tool in pediatric respiratory due to its safety profile and ease at the bedside. In the era of highly effective CF modulator therapies and improved life expectancy, the use of non-ionizing radiation techniques could become an integral part of CF management, particularly in the pediatric population. The present review explores the potential role of LUS in CF management based on available data, analyzing all publications from January 2015 to January 2024, focusing on two key areas: LUS in CF pulmonary exacerbation and its utility in routine clinical management. Nonetheless, LUS exhibits a robust correlation with computed tomography (CT) scans and serves as an additional, user-friendly imaging modality in CF management, demonstrating high specificity and sensitivity in identification, especially in consolidations and atelectasis in the CF population. Due to its ability, LUS could be an instrument to monitor exacerbations with consolidations and to establish therapy duration and monitor atelectasis over time or their evolution after therapeutic bronchoalveolar lavage. On the basis of our analysis, sufficient data emerged showing a good correlation between LUS score and respiratory function tests. Good sensitivity and specificity of the methodology have been found in rare CF pulmonary complications such as effusion and pneumothorax. Regarding its use in follow-up management, the literature reports a moderate correlation between LUS scores and the type, extent, and CT severity score of bronchiectasis. A future validation of ultrasound scores specifically in CF patients could improve the use of LUS to identify pulmonary exacerbations and monitor disease progression. However, further research is needed to comprehensively establish the role of LUS in the CF population, particularly in elucidating its broader utility and long-term impact on patient care.

Retrocardiac pneumomediastinum (RP) is the consequence of air trapping in the inferior and posterior mediastinum. It is characterized by the presence of a right or left para-sagittal infrahilar oval or pyramidal air collection on a chest X-ray. It is usually detected in neonates because of alveolar rupture after invasive ventilation or invasive manoeuvres applied on airways or the digestive tract. A healthy child came to the emergency department (ED) for acute respiratory failure due to viral bronchiolitis when he was 2 months old. Because of his clinical condition, he underwent helmet continuous positive airway pressure (HCPAP). When the condition allowed, he was discharged and sent home. He was re-admitted into the hospital for asthmatic bronchitis 3 months later. A frontal chest X-ray taken during the second hospitalization showed an oval-shaped retrocardiac air lucency not previously detected. Differential diagnosis including digestive and lung malformations was made. Finally, the diagnosis of RP was made. We report an unusual case of retrocardiac pneumomediastinum in a 5-month-old male infant after the application of continuous positive pressure via a helmet. RP presentation after the administration of non-invasive ventilatory support beyond the neonatal age is unusual. Although surgical drainage is curative, conservative treatment can be considered in hemodynamically stable patients.

Different screening strategies for developmental dysplasia of the hip (DDH) exist. Despite screening efforts, cases of late presentation continue to occur, often necessitating surgery. This systematic review and meta-analysis assess the effect of newborn selective ultrasound screening for DDH on the incidence of late presentation in infants and children, compared to a universal ultrasound strategy. A systematic search across Medline and EMBASE databases was performed between January 1950 and February 2021. A consensus-based evaluation of abstracts led to retrieval of relevant full text, original articles or systematic reviews in English only. These were assessed according to agreed eligibility criteria, and their reference lists were reviewed to identify additional eligible publications. Following final consensus on included publications, data was extracted, analysed and reported as per PRISMA and Prospero (CRD42021241957) guidelines. The 16 eligible studies consisted of 2 randomised controlled trials and 14 cohort studies, published between 1989 and 2014, with a total of 511,403 participants. In total, 121,470 (23.8%) received a neonatal hip ultrasound, of whom 58,086 and 63,384 were part of a selective or a universal ultrasound screening strategy, respectively. The difference in the proportion of late presentation between the universal and selective strategies was 0.0904 per 1,000 (P = 0.047). The time effect, i.e. the difference between early and late presentation defined respectively, as less than and more than 3 months of age, regardless of screening strategy, was not significant (P = 0.272). Although there was variability in study design and reporting, the quality of the evidence, based on the critical appraisal skills programme appraisal tools, was generally good. Compared to universal ultrasound screening for DDH, selective screening resulted in a slightly higher rate of late presentation. Uniformity in design and reporting of DDH studies and a cost-effectiveness analysis are needed.

Background Ataxia-telangiectasia (A-T) is a rare autosomal recessive DNA repair disorder, characterized by progressive cerebellar degeneration, telangiectasia, immunodeficiency, recurrent sinopulmonary infections, radiation sensitivity, premature aging and predisposition to cancer. Although the association with autoimmune and chronic inflammatory conditions such as vitiligo, thrombocytopenia and arthritis has occasionally been reported, an onset with articular involvement at presentation is rare. Case presentation We herein report the case of a 7-year-old Caucasian girl who was admitted to the Rheumatology Department with a history of febrile chough and polyarthritis which led initially to the suspicion of an autoinflammatory disease. She had overt polyarthritis with knees deformities and presented with severe pneumonia. A chest Computed Tomography (CT) scan showed bilateral bronchiectasis, parenchymal consolidation and interstitial lung disease; rheumatoid factor and type I interferon signature resulted negative, therefore excluding COatomer Protein subunit Alpha (COPA) syndrome. A diagnosis of sarcoidosis had been suspected based on histological evidence of granulomatous liver inflammation, but ruled out after detecting normal angiotensin converting enzyme and chitotriosidase blood levels. Based on her past medical history characterized by at least six episodes of pneumonia in the previous 4 years, immunological phenotyping was performed. This showed complete IgA and IgE deficiency with defective antigen-specific antibodies to Pneumococcal, Tetanus toxin and Hemophilus Influenzae B vaccines. Additionally, low numbers of B cells and recent thymic emigrants (RTE) were found (CD4Ra 1.4%), along with a low CD4+/CD8 + T cells ratio (< 1). Finally, based on gait disturbances (wobbly wide-based walking), serum alfa-fetoprotein was dosed, which resulted increased at 276 ng/ml (normal value < 7 ng/ml). A diagnosis of Ataxia-Telangiectasia was made, strengthened by the presence of bulbar telangiectasia, and then confirmed by Whole Exome Sequencing (WES). Conclusions Although rare, A-T should always be ruled out in case of pulmonary bronchiectasis and gait disturbances even in the absence of bulbar or skin telangiectasia. Autoimmune and granulomatous disorders must to be considered as differential diagnosis.

INTRODUCTION:Plexiform neurofibromas (PN) represent the main cause of morbidity in patients affected by Neurofibromatosis Type 1 (NF1). Until recently, surgery has been the main treatment option in these patients, but it is burdened with a low efficacy rate and a high incidence of side effects as well as recurrence. In recent years, MEK inhibitors (MEKi) such as selumetinib and trametinib have shown great promise.METHODS:We retrospectively describe a single center cohort of NF1 patients affected by PN1 and treated with MEKi since 2019 to 2021. Patients recruited in the study were affected by PN that were not eligible to complete surgical excision, symptomatic or with major cosmetic deformation or functional neurological deficits.RESULTS:Most patients experienced improvement in clinical symptoms and quality of life, with reduction or stabilization of lesions. However, no complete response was achieved. The most common adverse effects involved the skin, affecting every patient. Importantly, no life-threatening adverse effects occurred.CONCLUSIONS:In our experience, MEKi treatment has been shown to be both safe and effective in improving symptomatology and quality of life.

In the last few years, current evidence has supported the use of point-of-care ultrasound (POCUS) for a number of diagnostic and procedural applications. Considering the valuable information that POCUS can give, we propose a standardized protocol for the management of neonates with a congenital diaphragmatic hernia (CDH-POCUS protocol) in the neonatal intensive care unit. Indeed, POCUS could be a valid tool for the neonatologist through the evaluation of 1) cardiac function and pulmonary hypertension; 2) lung volumes, postoperative pleural effusion or pneumothorax; 3) splanchnic and renal perfusion, malrotations, and/or signs of necrotizing enterocolitis; 4) cerebral perfusion and eventual brain lesions that could contribute to neurodevelopmental impairment. In this article, we discuss the state-of-the-art in neonatal POCUS for which concerns congenital diaphragmatic hernia (CDH), and we provide suggestions to improve its use. Impact This review shows how point-of-care ultrasound (POCUS) could be a valid tool for managing neonates with congenital diaphragmatic hernia (CDH) after birth. Our manuscript underscores the importance of standardized protocols in neonates with CDH. Beyond the well-known role of echocardiography, ultrasound of lungs, splanchnic organs, and brain can be useful. The use of POCUS should be encouraged to improve ventilation strategies, systemic perfusion, and enteral feeding, and to intercept any early signs related to future neurodevelopmental impairment.

Background The newly adopted European directive DE59/2013 mandates adequate patient information in procedures involving ionising radiation. Patient interest in knowing about their radiation dose and an effective communication method for dose exposure remain poorly investigated. Purpose This study is aimed at investigating both patient interest in radiation dose and an effective method to communicate radiation dose exposure. Material and methods The present analysis is based on a multi-centre cross-sectional data collection involving 1,084 patients from four different hospitals ‒ two general and two paediatric hospitals. Anonymous questionnaires were administered, consisting of an initial overview of radiation use in imaging procedures, a patient data section, and an explanatory section providing information in four modalities. Results 1009 patients were included in the analysis, with 75 refusing participation; 173 participants were relatives of paediatric patients. Initial information provided to patients was considered comprehensible. The information modality with symbols was considered the most readily understandable format by patients, with no appreciable differences in comprehension attributable to social or cultural background. The modality including dose numbers and diagnostic reference levels was preferred by patients with higher socio-economic background. The option ‘None of those’ was selected by one-third of our sample population, composed of four different clusters: female, over 60 years old, unemployed, and from low socio-economic backgrounds. Conclusions This study demonstrated a high level of interest amongst patients in knowing about radiation dose exposure. Pictorial representations were well understood by patients from a variety of different ages and education levels. However, a universally comprehensible model of communicating radiation dose information remains to be elucidated.

AIM: Intestinal duplication is a rare congenital malformation characterized by the presence of a duplicity of the alimentary canal. The intestinal duplication may contain pancreatic tissue in 3% of cases, as reported in the literature, andin rare cases - it can be completely isolated from the gastrointestinal tract itself with an independent vascular axis. CASE EXPERIENCE: Here the first case, as far as we know, is reported, of isolated intestinal duplication of the mesentery, with pancreatic tissue and gastric cells inside, found in a 2 -year -old child who presented clinical and ultrasound symptoms that simulated a perforated Meckel's diverticulum. CONCLUSIONS: Intestinal cystic duplications are a rare disease with a clinical spectrum ranging from asymptomatic peritonitis to perforation. It is important to underline that intestinal cystic duplication should be considered as a differential diagnosis in those patients who present a common symptomatology attributable to intestinal obstruction, peritonitis or appendicitis. In cases of accidental finding, these lesions must - in any case - be removed due to the risk of complications such as intestinal invagination on the "leading point", peritonitis or subsequent neoplastic degeneration.

Objective: The purpose of this study is to analyze the texture characteristics of chronic non-bacterial osteomyelitis (CNO) bone lesions, identified as areas of altered signal intensity on short tau inversion recovery (STIR) sequences, and to distinguish them from bone marrow growth-related changes through Machine Learning (ML) and Deep Learning (DL) analysis. Materials and methods: We included a group of 66 patients with confirmed diagnosis of CNO and a group of 28 patients with suspected extra-skeletal systemic disease. All examinations were performed on a 1.5 T MRI scanner. Using the opensource 3D Slicer software version 4.10.2, the ROIs on CNO lesions and on the red bone marrow were sampled. Texture analysis (TA) was carried out using Pyradiomics. We applied an optimization search grid algorithm on nine classic ML classifiers and a Deep Learning (DL) Neural Network (NN). The model’s performance was evaluated using Accuracy (ACC), AUC-ROC curves, F1-score, Positive Predictive Value (PPV), Mean Absolute Error (MAE) and Root-Mean-Square Error (RMSE). Furthermore, we used Shapley additive explanations to gain insight into the behavior of the prediction model. Results: Most predictive characteristics were selected by Boruta algorithm for each combination of ROI sequences for the characterization and classification of the two types of signal hyperintensity. The overall best classification result was obtained by the NN with ACC = 0.91, AUC = 0.93 with 95% CI 0.91–0.94, F1-score = 0.94 and PPV = 93.8%. Between classic ML methods, ensemble learners showed high model performance; specifically, the best-performing classifier was the Stack (ST) with ACC = 0.85, AUC = 0.81 with 95% CI 0.8–0.84, F1-score = 0.9, PPV = 90%. Conclusions: Our results show the potential of ML methods in discerning edema-like lesions, in particular by distinguishing CNO lesions from hematopoietic bone marrow changes in a pediatric population. The Neural Network showed the overall best results, while a Stacking classifier, based on Gradient Boosting and Random Forest as principal estimators and Logistic Regressor as final estimator, achieved the best results between the other ML methods.

Background: Patients admitted to cardiac intensive care unit (CICU) are daily monitored through radiographic examinations. A clinically and diagnostically acceptable image quality is characterized by the visualization of the anatomical structures in absence of artefacts, good contrast and ability to answer the clinical question. However, the exam involves a patient exposure at X-ray that has to be minimized for radioprotection reasons, especially in childhood. Objective: Goal of the study was the image quality evaluation and the entrance surface air kerma (Ke) estimation of the radiological investigations for children admitted at CICU. Materials and methods: The study was conducted on 105 chest radiographs of children admitted to CICU, with age ranging from 0 to 5 years and classified into three age groups.Results: In the images assessment, all the exams resulted suitable to answer the clinical task. The absence of artefacts was observed and the image quality criteria were met for most part of the exams. Contrary, the colli-mation was not respected, especially the lateral one Concerning the Ke, it resulted lower than the reference value of 50 mu Gy for all the cases in study.Conclusion: Radiographic images acquired at CICU meet the image quality requirements; in particular, good visualization of the anatomical structures in absence of artefacts, high contrast and ability to answer the clinical task. The radiation exposure of the patient resulted low and compatible with a large number of radiographic examinations, which may be requested for patients during the hospitalization period.

Vascular anomalies of the pediatric orbit represent a heterogeneous group that include both vascular tumors and vascular malformations. The disorder may initially be silent and then associated with symptoms and/or function damage, depending on the type of vascular anomaly and its extension. Vascular tumors include benign, locally aggressive (or borderline) and malignant forms while vascular malformations are divided into “simple”, “combined” and syndromic, or “low flow” or “high flow”. Both entities can arise in isolation or as part of syndromes. In this review, we describe the imaging findings of the vascular lesions of the orbit in the pediatric population, which are key to obtain a correct diagnosis and to guide the appropriate treatment in the light of the new genetic and molecular discoveries, and the role of the radiologist in their multidisciplinary management. We will also touch upon the main syndromes associated with orbital vascular abnormalities.