Showing 3 Abstracts.
Complex lymphatic anomalies (CLA) are extremely rare sporadic diseases of genetic origin, primarily affecting children, adolescents, and young adults. According to the International Society for the Study of Vascular Anomalies classification, there are five types of CLA: generalized lymphatic anomaly (GLA), Gorham-Stout disease (GSD), kaposiform lymphangiomatosis, central conducting lymphatic anomaly, and generalized lymphatic dysplasia. These lymphatic malformations are characterized by lymphatic proliferation under the influence of vascular endothelial growth factor. Our educational exhibit aims to briefly review the pathophysiology of CLA and illustrate the spectrum of lesions in 14 patients, including 9 with GSD and 5 with GLA. Gorham-Stout disease, also known as vanishing bone disease, is characterized by progressive bone destruction with cortical resorption. The primary lesion, a punched-out osteolysis, typically extends from the initially affected bone to nearby bones and is more frequently observed in the axial skeleton (skull, spine, ribs, pelvic bones and shoulder girdle). It can progress rapidly and sometimes stabilize spontaneously. There is usually no periosteal reaction or bone reconstruction. Patients can present with pain (particularly in case of pathological fracture and vertebral collapse), chronic lymphedema, lower limb length discrepancy, assymetric girth, scoliosis/kyphosis, pericardial ou pleural effusion, and rarely meningitis due to a breach of the skull base. Generalized lymphatic anomaly, formely called lymphangiomatosis, is a diffuse or multicentric disease involving soft tissues, bones, spleen, liver, intestine, lungs, mediastinum, and skin. Thoracic involvment may result in respiratory failure due to chylous pleural effusion, interstitial syndrome or restrictive syndrome related to spinal deformity, as well as pericardial effusion. Bone lesions primarily involve the axial skeleton, as in GSD. Compared with GSD, bone lesions may be multifocal, bone destruction is less severe and fracture are uncommon. Abdominal involvement includes splenic lesions, retroperitoneal mass, ascites, intestinal hemorrhage, and lymphorrhea. Read More
Meeting name: IPR 2026 Congress , 2026
Authors: Chalard François, Barillon Jeanne, Blondiaux Eleonore, Ducou Le Pointe Hubert
Keywords: Lymphatic Malformation, Generalized Lymphatic Anomaly, Children
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid tumor, a rare disease that is most often diagnosed in early childhood. Here, we present the radiological spectrum of manifestations of this disease. The organs most frequently affected are the bone, skin, central nervous system (CNS), lungs, bone marrow, liver, spleen and lymph nodes. Our review includes illustrations of the regression of severe lung lesions and neurodegenerative lesions, which can be observed under targeted therapy (mitogene-activated protein kinase inhibitor). In addition, for each lesion, we present a short list of differential diagnoses. Bone Langerhans cell histiocytosis is a potentially multisystem disease, but its most common presentation is a single-bone lesion. It predominantly affects the axial skeleton, with the skull being the most frequently affected bone. Bone lesions generally develop slowly and can sometimes be aggressive. Vertebra plana is a classic aspect of vertebral LCH ; spinal cord compression is rare. Langerhans cell histiocytosis of the ear primarily affects the mastoid. The ossicles, petrous apex and inner ear are rarely affected. Central nervous system The pituitary stalk is the most commonly affected anatomical region. Other affected sites include the infra- and supratentorial parenchyma, venous sinuses, dura mater, and ventricules. Neurodegenerative lesions are distinct from other LCH lesions and have an affinity for the dentate nucleus, cerebellar white matter, brainstem, and basal ganglia. Lung The initial lung lesions are nodules that gradually excavate to form thin-walled cysts that fuse and may lead to pneumothorax. Pulmonary fibrosis is a late stage of the disease. At risk organs The liver, spleen, bone marrow, and lymph nodes are affected in Letterer-Siwe syndrome, which occurs in infancy. Chronic hepatobiliary LCH is characterized by sclerosing cholangitis that can progress to biliary cirrhosis. Read More
Meeting name: IPR 2026 Congress , 2026
Authors: Chalard François, O'keane Aurélie, Blondiaux Eleonore, Ducou Le Pointe Hubert
Keywords: Langerhans Cell Histiocytosis, Imaging, Education
Five years ago the leaders of the world’s regional pediatric imaging societies launched the World Federation of Pediatric Imaging (WFPI), a pediatric imaging body with global overview. According to its mission statement "WFPI provides an international platform for pediatric radiology organizations united to address the challenges in global pediatric imaging training and the delivery of services". To guide WFPI's outreach endeavors as well as of other organizations interested in improving pediatric radiology services worldwide a project to better define the needs and gaps of pediatric radiologists and training centers worldwide was undertaken. The project aims to: -Establish the number of Pediatric Radiologists (as regionally defined) per country/region around the world -Outline the training pediatric radiologists receive and availability of training centers -Highlight major disparities and gaps in availability of pediatric radiologists and pediatric imaging training -Based on this information create a roadmap for WFPI’s global efforts. Read More
Meeting name: SPR 2017 Annual Meeting & Categorical Course , 2017
Authors: Soto Gloria, Nievelstein Rutger, Boechat Ines, Pool Kara-lee, Dehaye Amanda, Ducou Le Pointe Hubert, Halliday Kath, Lam Wendy, Lopez Pino Miguel, Mentzel Hans-joachim, Mwango Gladys