1. Review of structural and metabolic imaging findings across various classes of FCD based on recent ILAE classification. 2. Review of correlation between imaging findings and pathologic features in different FCD groups. Read More

Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019

Authors: Watal Pankaj, Priya Sarv, Sato T Shawn, Bathla Girish

Keywords: focal cortical dysplasia, FCD

Neurocristopathies are a group of disorders characterized by a common origin in aberrant neural crest development. These include common pediatric disorders such as Hirschsprung’s disease, Treacher Collins syndrome, Di George syndrome, MEN type 2A/2B as well as common pediatric tumors such as neuroblastoma, pheochromocytoma, Ewing’s sarcoma, neurofibromatosis, medullary carcinoma of the thyroid and melanoma. Neural crest cells are derived from discrete cell masses that arise at the junction between the neural and epidermal ectoderm in neurula-stage vertebrate embryos. Neural crest cells migrate extensively in an organized manner and spread widely throughout the body. Derivatives of neural crest cells include Schwann cells in the leptomeninges, nerve root ganglia in the central nervous system, thyroid C cells, bone formation in the mandible and skull base, dermis of the head and neck, myenteric nerve plexuses of the intestines, pigment cells of the skin, paravertebral sympathetic ganglia, and adrenal medulla cells. Developmental disturbances of the neural crest cells give rise to a variety of disorders as listed above and have collectively been termed neurocristopathies by Bolande in 1974. Patients with one neurocristopahty have an increased risk of having other neurocristopathies. Familial inheritance has also been shown. There is a variability in the combinations of lesions found in the same patient or family. Recent advances in genetics and developmental biology have provided deeper insights into these collection of conditions. New technologies in biology including iPS cell technology are expected to further advance our understanding of neurocristopathies. Read More

Meeting name: SPR 2018 Annual Meeting & Postgraduate Course , 2018

Authors: Handa Atsuhiko, Priya Sarv, Sato T Shawn, Sato Yutaka

Keywords: neurocristopathy, neural crest cells, embryology

The prevalence of fibrosing diseases is uncommon in adult patients, and significantly more rare in the pediatric population. The spectrum of fibrosing diseases may be subdivided into two sub-categories: Inflammatory pseudotumors (IMT) and multifocal fibrosclerotic diseases. IMT has a predilection for visceral soft tissues and the most common sites of involvement include lung, abdominopelvic region, but virtually any site may be involved, including the somatic soft tissues, bone, larynx, uterus and CNS. Multifocal fibrosclerotic diseases encompasses retroperitoneal fibrosis, mediastinal fibrosis, reidel’s thyroiditis, orbital pseudotumor, and sclerosing cholangitis to name a few. IMT’s are predominantly neoplastic but may be post-traumatic or post-infectious. Fibrosclerosing diseases may be associated with inflammatory diseases (inflammatory bowel disease), autoimmune conditions (juvenile rheumatoid arthritis, systemic lupus erythematosus), malignant tumors (lymphoma), vasculitis and may arise secondary to drugs, toxins, trauma or radiation. Some may be idiopathic with no underlying cause. The clinical presentation can be quite variable and often depends upon the site of involvement as well which adjacent structures are affected by the fibrosis. Initial diagnosis can be suggested by imaging, but imaging findings are often non-specific. They can appear as mass forming and may be mistaken for more aggressive malignancy. Tissue is often needed for confirmation. Histopathology shows evidence of lymphocytic infiltration, activated fibroblasts, spindle shaped cells and granulation tissue. The key issue for the pediatric radiologist is to be aware of these rare conditions and thus include them in their differential diagnosis. This diagnosis should be considered to avoid over aggressive biopsy, operation and chemotherapy. In addition, it may warrant work up for other associated fibrosing diseases in the appropriate clinical scenario. In this presentation, we will provide a case based review of the features of pediatric inflammatory pseudotumors and fibrosclerotic diseases including myofibroblastic tumors, fibromatosis, fibrosarcomas, nodular and cranial fascitis as well as fibrotic conditions involving mediastinum, retroperitoneum, biliary tract and thyroid gland with their appropriate diagnostic work-up. Our aim is to make people aware of these rare presentations, so that they are not lost in the long differential! Read More

Meeting name: SPR 2018 Annual Meeting & Postgraduate Course , 2018

Authors: Priya Sarv, Watal Pankaj, Sato T Shawn, Sato Yutaka

Keywords: Fibrosclerosis, inflammatory pseudotumors

Interpretation of chest radiographs requires a good understanding of anatomy, the physiology of the lungs and cardiovascular system as well as good pattern recognition. Additionally, it requires a systematic approach to search for pathologies and pertinent clinical details for interpretation. With frequent use of CT / MRI, the residents (and even practicing radiologists) have become less skilled in the interpretation of chest x-rays, making one of the most commonly ordered exams the most challenging. With challenges of decreasing radiation exposure (especially in pediatric population), it is important that the relatively lost skill set of chest radiographs interpretation be revisited, for trainees (radiology and non-radiology services) and the practitioners. <b>OUTLINE</b> ● Historical perspective. ● Overall approach towards a chest x-ray and importance of clinical details. ● PA/AP and lateral radiograph anatomy and radiographic lines and stripes. ● Fleischer society standard terminology for radiographs. ● Radiographic appearance of abnormalities and pearls for differentiation. The abnormalities to be categorized as: ○ Pneumonia ○ Effusion ○ Atelectasis ○ Big heart ○ Pulmonary vascularity (plethora and oligemia) ○ Lucencies (Pneumo: thorax, mediastinum and cardia) ○ Masses Read More

Meeting name: SPR 2018 Annual Meeting & Postgraduate Course , 2018

Authors: Ferreira Da Silva Renato, Nagpal Prashant, Priya Sarv, Sato T Shawn, Sato Yutaka

Keywords: chest, radiograph, interpretation

Immunoglobulin4 related disease (IgG4RD) is an inflammatory condition involving multiple regions of the body resulting in fibrosis which can lead to eventual organ failure. This entity was originally described with autoimmune pancreatitis. Recently many other previously described lesions have been brought under the umbrella of IgG4RD. These include a spectrum of conditions involving the head and neck region (orbits, salivary and lacrimal glands), thyroid gland (Riedel’s thyroiditis), vasculature (periaortitis), kidneys, lungs, retroperitoneum, mesentery, pituitary gland, biliary tract, pericardium, lymph nodes and pachymeninges. Reports of IgG4RD are quite rare in the pediatric literature, however this may be due to potential unawareness about the condition as well as the variable presentations and non-specific imaging features of IgG4RD. The prevalence in pediatric population is poorly described. The exact pathophysiology of IgG4RD is yet to be completely elucidated. The imaging manifestations are non-specific, and primarily consist of tumefactive enlargement of involved organs and homogenous contrast enhancement and associated lymphadenopathy. IgG4RD may manifest in single organ or may present as widespread disease involving multiple organs. These features overlap with other mass forming conditions like malignancy or lymphoma. However, the presence of multifocal disease with more than one organ involvement may point towards possible IgG4 related disease. Other than IgG4 related autoimmune pancreatitis, there is no consensus on diagnostic criteria based upon imaging. Definitive diagnosis of IgG4RD is made with biopsy and the histology characterized by infiltration of lymphocytes and IgG4 plasma cells with storiform fibrosis and obliterative phlebitis. According to Boston consensus, the ratio of IgG4 /IgG in tissue should be more than 0.4 with more than 10 IgG4+ cells per high power field. Serum IgG4 levels range from normal to elevated. Steroids are effective as first line treatment in majority of patients. Our aim in this presentation is to familiarize radiologists with the spectrum of imaging features, and areas of involvement in IgG4 related disease using cases of IgG4RD collected at three different pediatric hospitals. It is important for pediatric radiologists to be familiar with this relatively newly described disease entity and be aware of the spectrum of manifestations of IgG4RD, ensuring prompt recognition and early treatment. Read More

Meeting name: SPR 2018 Annual Meeting & Postgraduate Course , 2018

Authors: Priya Sarv, Handa Atsuhiko, Ferreira Da Silva Renato, Lai Lillian, Khanna Geetika, Sato T Shawn

Keywords: IgG4, fibrosis