Clinton Jokerst

Erdheim-Chester disease with interatrial septum involvement.

Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis with systemic manifestations. Most cases discuss radiologic findings once a pathologic diagnosis has already been established. We describe a patient with symptoms and no previously known diagnosis who was imaged with computed tomography, magnetic resonance imaging, and positron emission tomography. This case is unusual in that radiologic imaging demonstrated interatrial septum and diffuse cardiac involvement, in addition to the other characteristic lesions of Erdheim-Chester disease. The importance of this case to the radiologist is the expansion of the differential diagnosis of processes involving the interatrial septum and retroperitoneum.

ACR Appropriateness Criteria® Acute Chest Pain—Suspected Pulmonary Embolism

Pulmonary embolism (PE) remains a common and important clinical condition that cannot be accurately diagnosed on the basis of signs, symptoms, and history alone. The diagnosis of PE has been facilitated by technical advancements and multidetector CT pulmonary angiography, which is the major diagnostic modality currently used. Ventilation and perfusion scans remain largely accurate and useful in certain settings. Lower-extremity ultrasound can substitute by demonstrating deep vein thrombosis; however, if negative, further studies to exclude PE are indicated. In all cases, correlation with the clinical status, particularly with risk factors, improves not only the accuracy of diagnostic imaging but also overall utilization. Other diagnostic tests have limited roles. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer-reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

ACR Appropriateness Criteria® Routine Chest Radiography.

Chest radiographs are sometimes taken before surgeries and interventional procedures on hospital admissions and outpatients. This manuscript summarizes the American College of Radiology review of the literature and recommendations on routinely performed chest radiographies in these settings. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 3 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Imaging the Complications of Lung Transplantation

Imaging plays a key role in the diagnosis and management of complications following lung transplantation. This article outlines the imaging modalities available for evaluation of posttransplant complications with a focus on major indications and key strengths and weaknesses of each modality. A brief description of surgical technique and relevant anatomy is included. Descriptions of some of the more commonly encountered complications are outlined with a focus on imaging findings. Complications are grouped by anatomic or imaging-based findings and subcategorized chronologically to help order the differential diagnosis.

ACR Appropriateness Criteria Review ACR Appropriateness Criteria® Occupational Lung Diseases.

Occupational lung disease is a category of disease entities characterized by a reaction of the lung parenchyma to inhaled aerosolized particles found in the environment. This document summarizes the imaging appropriateness data for silicosis, coal worker pneumoconiosis, and asbestosis. The main points of the document are that computed tomography is more sensitive than radiography, computed tomography without contrast generally suffices for evaluation, and fluorodeoxyglucose-positron emission tomography may have utility in patients with mesothelioma. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 3 years by a multidisciplinary expert panel. The guideline development and review includes an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Myocardial Infarction Related to a Coronary Artery Aneurysm

CLINICAL PRESENTATION The patient was hemodynamically stable on admission with a heart rate of 98 beats/min and blood pressure of 140/80 mm Hg. There was no jugular venous distension. All peripheral pulses were palpable, and no cardiac murmurs were auscultated. There were no diagnostic electrocardiographic changes. However, her troponin I level was increasing, peaking at 34.0 ng/mL. She received aspirin, clopidogrel, and statin on presentation. Transthoracic echocardiography showed no obvious wall motion abnormality, and ejection fraction was 55%. Because she had no cardiovascular risk factors, a low Thrombolysis in Myocardial Infarction score, no significant electrocardiogram changes, and high troponins, the clinical suspicion was acute myocarditis. Cardiac magnetic resonance was performed, which showed focal transmural delayed myocardial enhancement in the mid-inferolateral segment without any associated myocardial edema (Figure 1A). These findings were consistent with focal myocardial infarction in the inferolateral territory. In this patient with no established coronary artery disease risk factors, we opted for a noninvasive evaluation of the coronary arteries. Computed tomography angiography was performed, which showed a small aneurysmal dilatation in the mid portion of an obtuse marginal artery (Figure 1B). The location of the aneurysm correlated with the vascular

ACR Appropriateness Criteria® acute respiratory illness in immunocompromised patients.

The respiratory system is often affected by complications of immunodeficiency, typically manifesting clinically as acute respiratory illness. Ongoing literature reviews regarding the appropriateness of imaging in these patients are critical, as advanced medical therapies including stem cell transplantation, chemotherapy, and immunosuppressive therapies for autoimmune disease continue to keep the population of immunosuppressed patients in our health care system high. This ACR Appropriateness Criteria topic describes clinical scenarios of acute respiratory illness in immunocompromised patients with cough, dyspnea, chest pain, and fever, in those with negative, equivocal, or nonspecific findings on chest radiography, in those with multiple, diffuse, or confluent opacities on chest radiography, and in those in whom noninfectious disease is suspected. The use of chest radiography, chest computed tomography, transthoracic needle biopsy, and nuclear medicine imaging is discussed in the context of these clinical scenarios. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 3 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or is not definitive, expert opinion may be used to recommend imaging or treatment.

Coronary CTA appearance of anomalous left coronary artery arising from the pulmonary artery with Intramural Aortic Route

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare anomaly in which the left main coronary artery (LM) typically arises from the left infero-lateral aspect of the main pulmonary artery just above the pulmonary valve. This case report presents a case of variant ALCAPA where the LM arises from the supero-medial aspect of the distal pulmonary artery, taking an unusual course within the wall of the aorta: ALCAPA with Intramural Aortic Route (ALCAPA-IAR). Both Coronary artery Computed Tomographic Angiography (CCTA) and digital subtraction angiography were used to image the patient prior to surgical intervention. ALCAPA-IAR was confirmed and repaired in the operating room. While this variant has been described in the literature, the appearance on CCTA has not yet been described. Recognizing this variant on preoperative imaging is vitally important as it can affect the surgical approach.

Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus

Bronchiolitis obliterans (BO) and paraneoplastic pemphigus are rare and ominous complications of Castleman disease. Collectively, these processes have been reported as part of paraneoplastic autoimmune multiorgan syndrome (PAMS), and they can occur in the setting of various hematologic malignant tumors, carcinoid tumors, and melanoma. Irrespective of the underlying malignancy driving PAMS, the clinical outcomes are uniformly poor, and there are no standard treatment regimens, given the clinical rarity of the syndrome. We describe 2 patients with unicentric Castleman disease complicated by paraneoplastic pemphigus and bronchiolitis obliterans. In addition to primary surgical resection for Castleman disease, we also used therapy from a treatment protocol used for bronchiolitis obliterans resulting from hematopoietic stem cell transplant (HSCT). We were able to treat the patients using intravenous immunoglobulin; rituximab; fluticasone, azithromycin, and montelukast (FAM); and rosuvastatin therapy. One patient demonstrated a favorable response, while the other demonstrated minimal response to this therapy.

Anticoagulation in Behçet related intrathoracic vasculitis

Behçet disease is a rare multisystem condition associated with HLA-B51 positivity that commonly afflicts individuals of Turkish or Middle Eastern descent, less than 10% of whom have pulmonary involvement. Behçet-related pulmonary vasculitis is an uncommon and heterogeneous group of conditions, often with associated pulmonary artery thrombus formation. These microthrombi can result in a misdiagnosis of acute pulmonary embolism. Anticoagulation therapy can be difficult, as blood thinners increase the risk of pulmonary hemorrhage without affording the same benefits as in pulmonary embolism management. We present two cases of pulmonary vasculitis in the context of Behçets syndrome, one in a Native American man with associated superior vena cava syndrome and pericarditis, with an increased risk of hemorrhagic pericardial effusion, and the other in an African American man with acute hypoxic respiratory failure with an increased risk of alveolar hemorrhage. We describe their management and the balancing act surrounding anticoagulation therapy in Behcet-related pulmonary vasculitis.