Showing 9 Abstracts.
Metachromatic leukodystrophy is an autosomal recessive lysosomal storage disorder that primarily affects the white matter tracts of the central nervous system. This occurs due to the build up of sulfatides, which leads to demyelinating disease in the brain. On T2 weighted MR imaging, this disorder often exhibits hyperintense periventricular white matter sparing of the subcortical U-fibers. One may also see hypointense stripes and dots within the affected areas. This is commonly referred to as the tigroid or leopard pattern and is a result of peri-vascular sparing. Other features include low signal on T1 weighted images and typically no enhancement on contrast-enhanced T1 weighted images. Metachromatic leukodystrophy can also lead to sulfatide buildup in organs outside the central nervous system, such as the gallbladder, and lead to gallbladder polyposis. This educational exhibit will provide a comprehensive review of the imaging features of metachromatic leukodystrophy, discuss the clinical categorization, and will highlight some of the most common differential diagnoses. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Priya Lakshmi, Illimoottil Mathew, Errampalli Eric, Kosaraju Sriya, Orscheln Emily
Keywords: Metachromatic leukodystrophy
Hemophagocytic Lymphohistiocytosis (HLH) presents unique challenges for diagnosis and management. This educational exhibit aims to elucidate the multifaceted nature of HLH through a comprehensive exploration of multimodality imaging techniques. HLH is a rare, potentially life-threatening hyperinflammatory disorder often affecting pediatric patients, necessitating early diagnosis and intervention. Through a variety of radiological modalities, including computed tomography (CT), magnetic resonance imaging (MRI), ultrasound, and positron emission tomography (PET) we will highlight the complexity of HLH manifestations and the utility of different imaging methods in facilitating early and accurate diagnosis. Furthermore, we will delve into the role of functional imaging in assessing HLH. The exhibit will emphasize the dynamic nature of HLH and its capacity to evolve rapidly, underscoring the need for timely and accurate imaging to guide clinical management. This educational exhibit invites participants to engage with complex HLH cases and explore the pivotal role of multimodality imaging in the diagnosis and management of this challenging disease. By presenting a spectrum of cases and highlighting the strengths of different imaging techniques, we aim to equip radiologists with the knowledge and confidence needed to tackle HLH effectively in pediatric patients. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Illimoottil Mathew, Errampalli Eric, Kosaraju Sriya, Priya Lakshmi, Orscheln Emily
Keywords: hemophagocytic lymphohistiocytosis
Ankle fractures in the pediatric population pose unique diagnostic and therapeutic challenges due to the evolving anatomy of the child. However, when knowledge of this anatomic evolution intersects with knowledge of injury mechanisms that are often at play, the radiologist can understand that pediatric ankle fractures often occur in predictable patterns. This educational exhibit explores the various classifications of pediatric ankle injuries, encompassing Dias-Tachdjian, Salter Harris, and transitional fractures. Recognizing the significant anatomical and developmental differences between skeletally immature and mature ankles, we investigate the dissimilarities in fracture patterns that emerge in these two populations. Our exhibit explores the influence of the physis, ossification centers, supporting soft tissue structures, and differing anatomical proportions on fracture patterns in these populations, providing pediatric radiologists with valuable insights into the interpretation of pediatric ankle fractures. This comprehensive overview ensures a well-rounded understanding of the unique injury patterns and facilitates improved decision-making for treatment. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Illimoottil Mathew, Errampalli Eric, Kosaraju Sriya, Priya Lakshmi, Orscheln Emily
Pneumoperitoneum is the presence of air or gas within the peritoneal cavity. Prompt identification of pneumoperitoneum is critical due to its association with life-threatening pathologies. Through case-based discussion, the following exhibit will highlight various radiological signs of pneumoperitoneum on radiography as well as false cases of pneumoperitoneum, coined pseudopneumoperitoneum, an important distinction to avoid unnecessary invasive procedures for patients. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Errampalli Eric, Kosaraju Sriya, Illimoottil Mathew, Priya Lakshmi, Orscheln Emily
Keywords: Pneumoperitoneum
Hepatocellular carcinoma is the second most common pediatric liver tumor. Metabolic disorders, hepatitis B or C infection, and certain genetic mutations have been implicated in the development of HCC, and adolescents are the most commonly affected age group. The prevalence of this disease has decreased with increased hepatitis B immunization efforts, but approximately 50-70% of all pediatric hepatocellular carcinoma develops in the absence of underlying liver disease or genetic predisposition. Five-year survival outcome ranges between 13% and 28% with the best outcomes seen in the setting of complete surgical resection. Therefore, imaging plays an important role in diagnosis as well as in guiding management of these patients. Through this educational exhibit, we will discuss the imaging findings of hepatocellular carcinoma, initial imaging evaluation and staging, the role of imaging in ongoing management, and briefly review locoregional treatment options which can play a role in palliation, bridge to liver transplant, or potentially in downstaging disease. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Errampalli Eric, Kosaraju Sriya, Illimoottil Mathew, Priya Lakshmi, Orscheln Emily
Keywords: Hepatocellular Carcinoma, oncology
Fractures are a common occurrence in pediatric patients with around one third of children sustaining at least one by the age of 17 years. As the pediatric osseous structures develop, secondary ossification centers will become radiographically evident and there may be several of these ossification centers in single bone. As a child approaches skeletal maturity, the physis will gradually close and the primary and secondary ossification centers fuse. These processes often occur in known and predictable patterns. However, there are numerous cartilaginous physes and secondary ossification center variants that can potentially create a diagnostic dilemma for the radiologist. Knowledge of location and expected time of secondary ossification center appearance and physeal fusion in the pediatric patient is vital to distinguish them from fractures in a trauma setting. Accurate diagnosis of fractures is necessary for facilitation of appropriate management and prevention of subsequent deformity in the growing skeleton. Upper extremity fractures are encountered significantly more frequently than lower extremity fractures in pediatrics. However, there are a number of normal structures that may serve as fracture mimics in the upper extremity, particularly at the shoulder and elbow. In this educational exhibit, we aim to describe the expected changes in visible ossification centers and physes in the osseous structures of the shoulder and elbow, highlight some common and uncommon variations in the expected sequence, and highlight potential pitfalls related to this variation that may be mistaken for fracture or may result in an unrecognized fracture. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Kosaraju Sriya, Errampalli Eric, Illimoottil Mathew, Priya Lakshmi, Orscheln Emily
Appendicitis is an important cause of abdominal pain and is the leading reason for emergent surgery in children. Prompt and accurate diagnosis is important because delayed diagnosis increases the risk of rupture and false positive diagnosis may result in unnecessary surgery. While it can occur at any age, it most frequently occurs in pediatric patients. Further, the rate of perforation is higher in younger children. However, the differential diagnosis for pediatric abdominal pain is broad and includes many non-surgical diagnoses. Localization of pain can also be more difficult in children, posing an additional challenge. In this educational exhibit, we will discuss some clinical mimics of appendicitis and characteristic imaging findings to help differentiate them. Topics covered include omental infarct, typically a self-limited condition, but a cause of abdominal pain that has been identified in 0.024 to 0.1% of cases of surgery for appendicitis. In the rare cases that do require surgery, the surgical approach differs from that for appendicitis. In addition, we will discuss epiploic appendagitis, yet another self limiting condition in which proper diagnosis prevents unnecessary surgery. We will discuss Meckel’s diverticulum, which when inflamed may mimic acute appendicitis very closely in imaging appearance as well as clinical presentation. However, these entities can be differentiated if the inflamed structure can be identified as arising from the ileum rather than the cecum. Finally, we will also discuss other clinical appendicitis mimics such as mesenteric adenitis, inflammatory bowel disease, ileocolic intussusception, and ovarian torsion, and we will contrast the imaging appearance of lymphoid hyperplasia of the appendix with acute appendicitis. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Kosaraju Sriya, Errampalli Eric, Illimoottil Mathew, Priya Lakshmi, Orscheln Emily
Keywords: appendicitis, omental infarct, abdomen
Osteogenesis imperfecta (OI) is a rare genetic disorder that affects bone development and structure, resulting in bone fragility and susceptibility to fractures. This educational exhibit offers a comprehensive examination of OI, with particular emphasis on the clinical types I, II, III, IV, and V. With a focus on diagnostic tools, management strategies, and emerging treatments, this exhibit aims to enhance the understanding of OI within the pediatric radiology community. We will present a detailed overview of OI types, emphasizing the genetic and clinical variations that distinguish them. Attendees will gain insights into the pathophysiology of OI and its impact on bone health, growth, and overall well-being in affected children. We will highlight imaging techniques and genetic testing that facilitate accurate diagnosis and characterization of OI types. Through case studies, we will demonstrate the pivotal role of radiologists in early detection and improved patient outcomes. Treatment modalities for OI, including bisphosphonates, orthopedic interventions, and emerging therapies, will be explored. Our goal is to empower pediatric radiologists with the knowledge and tools necessary to provide accurate and timely diagnosis as well as to contribute to optimal disease management, ongoing research, and advancements in the field. By focusing on OI in all its diverse manifestations, this exhibit aims to strengthen the expertise of pediatric radiologists in identifying, characterizing, and managing this complex condition. Our collective effort in enhancing the care of children with OI will ultimately improve their quality of life and future prospects. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Illimoottil Mathew, Errampalli Eric, Kosaraju Sriya, Priya Lakshmi, Orscheln Emily
Keywords: Osteogenesis Imperfecta
Nail-patella syndrome (NPS) is a multisystemic autosomal dominant disease with neurologic, ocular, renal, and musculoskeletal manifestations. The incidence of NPS is reportedly 1 in 50,000, although this may be an underestimate due to its phenotypic variability allowing this disease to remain undiagnosed for multiple generations. A loss of function mutation of the LMXB1 gene, which influences dopaminergic and serotonergic neuronal differentiation, periocular mesenchymal development, renal podocyte development, limb patterning, and skull patterning, leads to NPS. Clinical findings include open-angle glaucoma, ocular hypertension, neuropathic pain and numbness/tingling, and renal failure. Absent, hypoplastic, or dystrophic fingernails are noted in 98% of cases. Almost all patients present with absent, hypoplastic, or irregular patellae that frequently sublux. About 70% of patients present with pathognomic iliac horns, which are corticomedullary processes continuous with the iliac bones at the gluteus medius muscle origin. Loss of skin creases over the distal interphalangeal joints is also a sensitive finding for NPS. Decreased extremity muscle mass and bone formation can lead to limited knee and elbow joint range of motion. The subject of this case report is a 20-year-old female who presented to nephrology clinic for proteinuria. Onychia was noted on physical exam. Past medical history was significant for spastic diplegia, and surgical history included epiphysiodesis due to leg length discrepancy. The patient was relatively asymptomatic otherwise. CT of the pelvis demonstrated bilateral osseous excrescences of the iliac bones. Absence of the patella and posterior subluxation of the radial head were noted on radiographs. CT of the abdomen showed bilateral renal atrophy. Subsequent renal biopsy demonstrated findings consistent with NPS. The patient was placed on hemodialysis after progressing to renal failure. The prognosis of NPS is favorable with proper screening precautions and early intervention; however, complications of this disease can lead to poor outcomes. Imaging is critical in diagnosing NPS through its musculoskeletal findings on radiography. Characteristic findings include small or absent patella, pathognomic bilateral iliac horns, abnormalities of the femoral condyles and trochlea, and radial head dysplasia/subluxation. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Errampalli Eric, Kosaraju Sriya, Illimoottil Mathew, Mcgowan Bryanna, Boyd Alec, Allam Emad
Keywords: Nail-Patella Syndrome, Genetics, Multi-System