Langerhans Cell Histiocytosis (LCH) is a rare clonal disorder of the mononuclear phagocyte system, characterized by the proliferation of pathologic Langerhans cells that may involve one or multiple organ systems. Its clinical and imaging manifestations are remarkably diverse, ranging from isolated skeletal lesions to disseminated multisystemic disease. Given this heterogeneity and frequent overlap with other entities, imaging plays a pivotal role in establishing the diagnosis, evaluating disease extent, and guiding follow-up. To complement this educational overview, we performed a retrospective assessment of all pediatric LCH cases diagnosed in our institution over the past 10 years (2015-2025). A total of 81 patients were identified, with a mean age at diagnosis of 6.5 years (range 0–17.5) and an equal sex distribution. Patient were imaged using plain films as well as advanced imaging modalities including brain and whole body MR imaging and PET-CT or bone scintigraphy. Bone involvement was by far the most frequent manifestation, observed in approximately 83% of cases. Multisystemic disease occurred in about one-third of patients, typically at a younger age, while central nervous system lesions—often associated with cranial bone involvement—were identified in nearly 10% of cases. Involvement of the skin, liver, and marrow predominated among early-onset forms. These findings align with previously described epidemiologic and imaging patterns of pediatric LCH. By integrating imaging patterns and clinical presentation, this work aims to serve as a practical reference for pediatric radiologists. Through a systematic approach, it enhances familiarity with the imaging spectrum of LCH and supports improved diagnostic confidence and patient management. Read More

Meeting name: SPR 2026 Annual Meeting , 2026

Authors: Barbosa Alberto, Ladera Gonzalez Enrique, Navallas Maria, Inarejos Emili, Barber De La Torre Ignasi

Keywords: Histiocytosis, Langerhans Cell Histiocytosis, LCH

Learning objectives: 1.Review the main pediatric cardiomyopathy phenotypes. 2.Clarify the role of MRI across the care pathway. 3.Walk through the key MRI sequences we actually use in kids. 4.Recognize the essential imaging findings that help us differentiate among them. - Introduction: Pediatric cardiomyopathies are a rare heterogeneous group of myocardial diseases but are the first cause of heart transplantation in children. Causes are diverse—genetic mutations, coronary anomalies, infections, toxins, arrhythmias—and sorting out phenotypes can be tricky because adult criteria are often not applicable to kids and because many kids don’t tolerate long exams. - Imaging techniques: Echocardiography represents the first first-line modality but it is often complemented with CMR as it allows a radiation-free differentiation between different phenotypes with unique myocardial tissue characterization, playing a key role in diagnosis, risk stratification, and treatment assessment. - Key MRI sequences: Conventional cardiomyopathies protocols in children may use 1.5 or 3 Tesla field strengths and include the following sequences: Initial localizers. Cine imaging. Phase contrast. Parametric mapping. LGE. - Dilated Cardiomyopathy: Most common. Leading cause of heart transplant. Dilated chambers. Reduced systolic function. LGE: Patchy or longitudinal mid-wall, transmural, subepicardial, or diffuse subendocardial involvement. Presence of LGE indicates poor prognosis and increased risk of SCD. Native T1 mapping is suggested to be the strongest independent predictor of diffuse myocardial disease, allowing for the identification of patients at risk for adverse outcomes. - Hypertrophic Cardiomyopathy: 2nd most common. LV hypertrophy z-score >2.5 (no family history) or >2 (with family history/genetic test). LVOT obstruction and SAM. LGE: Independent risk factor for SCD. Differential diagnosis with athlete's heart. - Arrhythmogenic Cardiomyopathy: RV and/or LV dilatation and reduced EF. Wall motion abnormalities – most important finding in kids. Myocardial fibrosis. Fatty infiltration rare in children. - Restrictive Cardiomyopathy: Rarest CMP. Non-dilated biventricular failure and biatrial dilatation. Ddx: constrictive pericarditis. - Excessive trabeculation/NCCM: Non-compacted/Compacted myocardium ratio > 2.3 measured in diastole. In the absence of dilation or systolic dysfunction, can be considered a phenotypic trait not necessarily associated with CMP. Read More

Meeting name: SPR 2026 Annual Meeting , 2026

Authors: Navallas Maria, Zuccarino Flavio, Inarejos Clemente Emilio J, Marie Eman, Gerrie Samantha, Ladera Gonzalez Enrique, Barber De La Torre Ignasi

Keywords: Cardiomyopathy, Cardiac MRI