Point of care pediatric musculoskeletal POCUS scanning and scoring protocols for childhood arthritis have emerged in recent years. However, pediatric musculoskeletal POCUS curricula in rheumatology fellowship programs are limited due to availability of trained faculty and resources. This proof-of-concept study investigated the effectiveness of educational methods for a pediatric musculoskeletal POCUS scoring protocol among fellows and physicians of differing subspecialties. Educational methods assessed included recorded videos and virtual review sessions. Effectiveness was assessed by calculating interrater reliability for the musculoskeletal POCUS scoring systems using the intra-class correlation coefficient (ICC). Following training sessions, participants then underwent scoring exercise(s) until the goal of an excellent ICC ≥ 0.75 was reached. Four participants completed two rounds of virtual education, review, and scoring sessions. Excellent interrater reliability was achieved for most views. This proof-of-concept study demonstrated virtual education covering advanced concepts of pediatric musculoskeletal POCUS provides a knowledge base for physicians from different subspecialties and various experience.
Hemimegalencephaly, or unilateral megalencephaly, is a sporadic congenital brain malformation characterized by enlargement of a cerebral hemisphere due to an abnormal proliferation of neurons or glial cells. Hemimegalencephaly is part of a spectrum of disorders, increasingly referred to as mTORopathies, which arise as a result of dysregulation or hyperactivation of the mammalian target of rapamycin (mTOR)-signaling cascade resulting in less restricted cell growth and survival. The resultant cortical disorganization and enhanced neuronal excitability often manifest clinically in the form of seizures. Ultrasound and magnetic resonance imaging (MRI) are often used to characterize hemimegalencephaly. Typical imaging findings seen include diffuse unilateral enlargement of a cerebral hemisphere with overlying cortical malformation and ipsilateral dilation of the lateral ventricle. This paper will review an unusual case of focal hemimegalencephaly diagnosed on prenatal imaging. Initial in utero MRI revealed a mass-like lesion in the frontal lobe without associated perilesional cerebral edema. Keying in on abnormalities within the overlying cortex was crucial in suggesting focal hemimegalencephaly as a leading diagnosis and distinguishing it from alternative diagnoses such as a neoplasm. Follow-up fetal MRI demonstrated the evolution of the cerebral abnormality and confirmed the diagnosis. Early diagnosis facilitated appropriate counseling of the parents and guided postnatal imaging and management.
Lymphomatoid granulomatosis (LG) is an EBV-associated angiodestructive lymphoproliferative disease with multiorgan involvement that predominantly affects the lungs. We present a case of a 72-year-old man with a history of chronic lymphocytic leukemia who presented with upper respiratory symptoms and multiple erythematous skin papules. Chest CT showed ill-defined, irregular solid pulmonary nodules with peripheral ground-glass opacities in a peribronchovascular distribution. The differential for this pattern of lung disease is vast which includes but is not limited to infection, vasculitis, sarcoidosis, lymphoma, and Kaposi sarcoma. Subsequent PET/CT showed rapid progression of lung opacities and marked FDG uptake of pulmonary opacities and skin nodules, which raised the question of Richter syndrome. Wedge biopsy under video-assisted thoracoscopic surgery was performed. Pathology showed an extensive lymphoid infiltrate involving lymphatic and bronchovascular bundles and consisting of a mixture of large lymphocytes and inflammatory cells. Special stains showed that the large lymphocytes expressed B-cell markers and EBV virus. Overall, the findings were consistent with LG.
Various typical and atypical imaging findings for pulmonary sarcoidosis have been described in the literature. Ground-glass opacities are one of the atypical manifestations, reported as diffuse or patchy ill-defined opacities frequently associated with additional findings and interstitial nodules. We performed a literature review to determine if our case had previously been described. The literature describes cases of mass-like consolidations, but there are no reports of mass-like ground-glass opacities. The appearance of the ground-glass opacities in our case is unique, appearing as discrete well-defined mass-like ground-glass opacities in a peribronchovascular distribution without additional parenchymal findings typically seen in sarcoidosis.
