Showing 4 Abstracts.
Langerhans cell histiocytosis (LCH) is a rare neoplastic disorder of abnormal proliferation of Langerhans cells affecting predominantly pediatric populations; sites of involvement include cutaneous, osseous, hematopoietic, and multisystem disease (1). Cutaneous involvement is common in children, though thymic involvement is rarely described (2). We report a case of an 8-month-old female infant with a dyad of cutaneous and thymic LCH. The purpose of this paper is to highlight a potentially underdiagnosed manifestation of infantile LCH and consider thymic sonography in infant LCH staging evaluation. A one-month old female presented with skin lesions and pruritus suspicious for atopic dermatitis failing to respond to therapy. Punch biopsy demonstrated cutaneous LCH. Staging non-contrast chest computed tomography (CT) showed multiple very faint calcifications in the thymus (figure 1) which could have been obscured by intravenous contrast administration. Ultrasound demonstrated multiple echogenic foci in the thymus (figure 2). Pathology confirmed thymic LCH. Throughout her presentation, the patient’s clinical symptoms of LCH were limited to diffuse pruritic cutaneous lesions. Thymic involvement changed patient management to chemotherapy infusion. At the time of this report she has shown no signs of disease progression and she remains clinically stable. Read More
Meeting name: SPR 2023 Annual Meeting & Postgraduate Course , 2023
Authors: Goldstein Elianna, White Elena, Giamanco Nicole, Rooks Veronica
Keywords: Langerhans Cell Histiocytosis, Ultrasound, Thymus
Review the experience teaching pediatric radiology to first year radiology residents in the first year of a new residency program in Rwanda. One of the goals of the Human Resources for Health (HRH) program is to build a residency-trained physician workforce to create a sustainable health education infrastructure in Rwanda. Establishing a radiology residency program in a resource-poor African nation is a challenge being addressed by combining curricula from South Africa, Kenya, and United States and supplemented with ACGME materials. In Rwanda, the pediatric specialty is especially critical due to the high pediatric population as the country continues to recover from the 1994 genocide. Approximately 12 months of general radiology training, visiting faculty offered a two-month rotation in pediatric radiology. To assess efficacy, a pre- and post-rotation evaluation program was implemented. Objective, case-based tests consisting of 100 cases were implemented on the first and last day of the eight-week rotation, which comprised from nine to fifteen hours of formal lecture and case-based teaching each week. A paired t-test was used to compare pre- and post-rotation test results. View box examination scores for four first-year residents were recorded. Pretest mean: 27% (range 12-33% correct), Post-test mean 49% (range 27-62% correct), Average overall improvement: 22 percentage points (95% CI 12-32, p=0.005). Pediatric radiology knowledge did not increase as much as would be expected for developed world learners under the same curriculum. Complicating factors leading to complexity include basic medical knowledge, number of learning hours at view box vs didactic lecture, and the inconsistent caseload mix at local hospitals. Expected routine cases in the Western world are not commonplace in Africa; however, more challenging cases such as ischiopagus tetrapus, accessory limb, and extensive fat necrosis are seen. Language and cultural barriers impede teaching and uptake of new information. Diagnosis and communication must consider social, financial, and nutrition limitations. Equipment limitations, coupled with supply shortfalls, frequently influenced the exam recommendations. The challenges identified during this two-month experience should inform future efforts to teach medicine in low-resource countries. Curricula modifications may be needed to address language, social, financial and caseload challenges as well as equipment/resource shortages. Read More
Meeting name: SPR 2018 Annual Meeting & Postgraduate Course , 2018
Authors: Rooks Veronica, Rosman David, Kim Sung, Harris Robert
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe reaction to a drug that manifests with a wide variety of symptoms, typically several weeks after initial drug exposure. DRESS can involve multiple organ systems in addition to skin rash, eosinophilia, fever, renal, and liver abnormalities. Posterior reversible encephalopathy syndrome (PRES) is a cerebrovascular autoregulatory disorder hypothesized to occur from breakdown of the blood-brain barrier with multiple risk factors including hypertension as a common component. Neuroimaging findings of PRES in children have been noted to vary from those in adults. Children tend to have atypical findings of edema in regions such as the frontal, temporal, basal ganglia, brainstem, and cerebellum, in contrast to adults who usually present with parieto-occipital distribution. We present a unique case of DRESS and discuss imaging findings of atypical PRES in a child complicating treatment of an intracranial subdural empyema. A 5-year-old male patient underwent craniotomy and functional endoscopic sinus surgery for initial management of left pansinusitis complicated by a subdural empyema. The patient presented to the emergency room 3 weeks later with fever and a morbilliform rash and was diagnosed with DRESS, presumed secondary to a phenobarbital reaction. Initial imaging findings on abdominal ultrasound included hepatosplenomegaly, gallbladder wall thickening with cholelithiasis, pleural effusion, and findings consistent with bilateral nephritis. Subsequent imaging corroborated findings with increasing renal size and echogenicity commensurate with renal dysfunction in conjunction with steroid treatment for DRESS resulting in hypertensive crisis. Together with worsening encephalopathy, brain MRI confirmed presumed diagnosis of atypical PRES with diffusion negative increased cortical and subcortical white matter T2/FLAIR signal, indicating edema in an pattern involving watershed zones including bilateral posterior parietal, temporal, occipital lobes, left frontal lobe, and cerebellum. This report illustrates an interesting case of PRES as a complication of DRESS syndrome in a pediatric patient formerly treated with craniotomy for an intracranial subdural empyema. We highlight the importance of including PRES on the differential in a patient with severe hypertension. The radiologist should be familiar with the atypical PRES pattern which is more commonly seen in children compared to adults. Read More
Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024
Authors: Guo Grace, Marcelo Karina, Clarion Michael, Naun Christopher, Rooks Veronica
Keywords: Acute hypertensive encephalopathy, Cerebrovascular autoregulatory disorder
Fatty falciform ligament appendage torsion (FFLAT) is a rare phenomenon as there are only two reported pediatric cases of falciform ligament fatty appendage torsion in the literature. In this case, the diagnosis was established via ultrasound (US) and confirmed with computed tomography (CT). US showed an echogenic, ill-defined mass in the epigastric region that extending into the falciform ligament. CT showed the “hyperattenuating rim” sign. This report is the first reported female pediatric case of FFLAT that was diagnosed with US and CT, given a trial of analgesics, and definitively cured via minimally invasive surgical excision. A 13-year-old female presented to the emergency department with episodic waxing and waning abdominal pain for three days. The pain had localized to the mid-epigastrium and worsened with deep inspiration. On examination, vital signs were within normal limits. Focal tenderness was elicited upon palpation of the epigastrium. Laboratory evaluation revealed a mild leukocytosis 14.2 x 109/L, normal range (3.9-10.6 x 109/L). Read More
Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019
Authors: Horak Richard, Mega James, Tanton Phillip, Criman Erik, Tabak Benjamin, Rooks Veronica
Keywords: Torsion, Falciform ligament, Fatty appendage torsion