Figure 3 - uploaded by Jean M Seely
Content may be subject to copyright.
Ventilation and perfusion scan (posterior view) of the lungs before stent insertion, showing 78.5% perfusion to the left lung, 21.5% perfusion to the right lung and normal ventilation
Source publication
Fibrosing mediastinitis is a rare benign condition, which can cause compression of the pulmonary or systemic vessels, tracheobronchial tree, coronary arteries or esophagus, leading to disabling clinical symptoms and even death. The case of a 26-year-old woman who presented with dyspnea is described. She was found to have 80% stenosis of the right p...
Context in source publication
Context 1
... scan of the thorax was normal. A quantitative ventilation-perfusion (V/Q) scan demonstrated 21.5% perfusion to the right lung, 78.5% perfusion to the left lung and normal ventilation (Figure 3). Dyspnea was progres- sive over the next eight months, and she was referred to a tho- racic surgeon. ...
Similar publications
Peripheral vascular disease is a common problem in the elderly population. This is commonly treated nowadays by percutaneous transluminal angioplasty (PTA). When PTA is used in the treatment of superficial femoral artery (SFA) occlusions 35% reocclude within 1 year. Endovascular stents (uncovered) have been used to try to overcome this problem; how...
Drug Eluting Balloon (DEB) angioplasty is a novel technology that has been proven to significantly reduce re-stenosis in coronary and femoro-popliteal arteries. We present in this study our experience in using DEB in the management of lower limb peripheral vascular disease and assessing the primary patency and target lesion revascularization (TLR).
Distal embolization (DE) commonly occurs during peripheral percutaneous interventions (PPI) of the lower-extremity arterial vessels. In this study we evaluate the predictors of DE in a large cohort of patients undergoing PPI at our center.
Patients who experienced clinically significant DE (requiring further mechanical or pharmacologic therapy as p...
Citations
... 1 Localised therapy is directed towards reopening of the occluded structure for symptom relief. This include endovascular, endo-bronchial or trans-oesophageal balloon dilatation/stenting. [12][13][14][15] In view of bilateral hilar involvement, endovascular stenting of stenosed pulmonary artery was considered as the best option in our case. Unfortunately, the patient died due to reperfusion injury. ...
Fibrosing mediastinitis is an uncommon, benign, progressive disorder caused by proliferation of fibrous tissue within mediastinum resulting in encasement of vital mediastinal broncho-vascular structures. Due to its rarity and variable clinical presentation, it is often misdiagnosed. We are presenting a case of fibrosing mediastinitis in an Ethiopian origin young male presenting with pulmonary hypertension due to simultaneous occlusion of pulmonary vein and arteries, clinically misdiagnosed as chronic pulmonary thromboembolism.
PurposeFibrosing mediastinitis is a rare disease characterized by fibrosis of mediastinal structures with subsequent constriction of the bronchi and pulmonary vessels leading to potential respiratory compromise and death. Presently, there is no effective curative treatment with available treatments focused on reducing symptomology, including placement of pulmonary artery stents. Limited studies examine the use of stents in fibrosing mediastinitis. Given this knowledge gap, we assessed stent patency, hemodynamics, complications, and secondary outcomes of clinical improvement of pulmonary artery stenting for fibrosing mediastinitis.Materials and Methods
Nine patients with fibrosing mediastinitis and pulmonary artery stents were retrospectively identified for inclusion (six females, three males; mean age 44.17 years, range 13–68; total 13 primary stents) from 2005 to 2018. Eight patients had history of PH. All patients had dyspnea on presentation. Seven patients had ventilation/perfusion studies demonstrating impairment. Results from computed tomography and echocardiography studies were collected to assess patency and physiologic response.ResultsAll patients received initial angioplasty and stenting of the right pulmonary artery (10 stents). Two patients underwent additional left-sided intervention (3 stents). Stenting significantly increased lesion luminal patency (54–79%; P < 0.005) and reduced systolic pressure gradients across stenoses (mean −9.38 mmHg; P < 0.005). Primary patency at one year was 90%. Two stents received reintervention at 276 and 497 days. 89% reported improvement in dyspnea in the initial post-stenting period. There were no mortalities or major complications.Conclusion
Pulmonary artery stenting improves vascular patency and provides symptomatic relief in patients with fibrosing mediastinitis.
Fibrosing mediastinitis is a rare disorder characterized by the invasive proliferation of fibrous tissue within the mediastinum. This fibrosis can result in compression of intrathoracic structures including the pulmonary vasculature leading to clinical symptoms and complications like pulmonary hypertension. Here, we present two cases of young patients with fibrosing mediastinitis complicated by pulmonary artery stenosis requiring medical and endovascular management with excellent results. Keywords: Fibrosing mediastinits, Pulmonary artery stenosis, Pulmonary hypertension
We report a case of a 29-year-old female with a history of asthma, post-partum ARDS, and pulmonary hypertension who presents with severe shortness of breath. The patient describes her shortness of breath as progressive over the past 10 years. Chest radiography and CT angiography of the thorax showed findings consistent with fibrosing mediastinitis with severe stenosis of the left main pulmonary artery. This resulted in appearance of unilateral absent left lung perfusion on quantitative Tc-99-MAA perfusion and Xe-133 ventilation (V/Q) scan.
Introduction Chest radiography is widely used as the initial imaging study in patients with suspected thoracic disease. Mediastinal abnormality is often manifested as unexpected findings on plain radiography performed for unrelated indications. Computed tomography (CT) is the imaging modality of choice for further characterization of suspected mediastinal masses because it can define the anatomy and characterize the tissues. CT is also a popular imaging modality for the evaluation of the mediastinum because of its wide availability and rapid image acquisition ability. Magnetic resonance imaging (MRI) can allow further soft tissue characterization and functional studies. MRI is often used to evaluate soft tissue pathologies, cardiovascular function, and spinal abnormalities. Although there is no physical boundary to separate the compartments, the mediastinum is often arbitrarily divided into compartments to develop a differential diagnosis. Disease processes can easily spread across contiguous compartments. There are many ways of dividing the mediastinal compartments on images, which have been attempted by radiologists Felson, Zylak, and Heitzman –4. Felson’s approach divides the mediastinum into anterior, middle, and posterior compartments, based on two imaginary lines drawn on a lateral chest radiograph. The first line is drawn from the thoracic inlet to the diaphragm along the posterior heart border and anterior tracheal wall, dividing the anterior and middle mediastinum. The second line is drawn 1cm posterior to the anterior margin of the dorsal vertebrae, and separates the middle and posterior mediastinum (Fig. 2.1).
Acute and chronic mediastinitis are relatively infrequent conditions that are usually diagnosed and treated clinically. Indeed, most cases of mediastinitis are seen by pathologists at autopsy, following descending infections from the neck, complications of endoscopy, ingestion of foreign objects and complications of cardiac surgery or other surgical procedures 1-4. Mediastinal biopsies are usually performed to diagnose mediastinal neoplasms but occasionally show only variable amounts of inflammation and/or fibrosis. The diagnosis of mediastinitis should be rendered cautiously in these instances, as the inflammatory reaction may be secondary to an adjacent neoplasm (e.g. Hodgkin’s lymphoma) that was not properly sampled. The inflammatory conditions of the mediastinum can be classified according to their clinical course and histologic characteristics into acute and chronic mediastinitis, granulomatous lymphadenitis, and sclerosing mediastinitis –11. They can also be classified according to their etiology as idiopathic, infectious (e.g. fungi, mycobacteria, other), autoimmune conditions (e.g. Riedel’s thyroiditis, IgG4 disease, Behcet’s disease) and other conditions such as sarcoidosis, and Wegener’s granulomatosis –23.
This book provides a comprehensive, up-to-date review of the pathology of the neoplastic and non-neoplastic diseases that occur in the mediastinum. The pathologic and relevant clinical features are discussed, and detailed diagnostic criteria for various diseases are given, along with information about the most relevant laboratory tests. The features of thymomas, thymic carcinomas, neuroendocrine carcinomas and germ cell tumors are discussed in detail, including the utilization of immunostains and other ancillary tests in diagnosing these diseases. An algorithmic approach to the differential diagnosis of mediastinal lymphomas is given, and a novel clinico-pathologic classification helps readers diagnose and treat thymomas. Cardiac lesions, mesothelioma and other intrathoracic lesions are also reviewed. The book features more than 650 images, and downloadable copies of these are included on a CD-ROM, packaged with the print book. Pathology of the Mediastinum will be of interest to practising surgical pathologists, thoracic surgeons, oncologists and radiologists.
Because of the developmental association that the thymus has with other foregut-derived structures, a diversity of pathologic epithelial lesions other than thymic or germ cell tumors may arise in the anterior mediastinum. In addition, the thorax is the second most common site (after the retroperitoneum) in which paragangliomas arise, and most of them are seen in either the anterior or posterior mediastinum. This chapter considers the attributes of those lesions. Parathyroid tumors of the mediastinum In light of the intimate embryological relationship between the thymus and the parathyroid glands, it should not be surprising that parathyroid neoplasms may be encountered in the mediastinum. Readers will recall that the third branchial pouch gives rise to the thymus and the inferior pair of parathyroid glands, whereas the superior parathyroids are derived from the fourth branchial pouch. During the fifth week of gestation, these structures begin their ventral-inferior descent from the upper cervical region into the lower neck or anterior mediastinum. If abnormalities occur in this migratory process, portions of the thymus may be left in the supraclavicular cervical region; conversely, the parathyroids may descend into the parathymic tissues or the posterior mediastinal compartment. Also, up to 6.5% of otherwise normal individuals have supernumerary, ectopic parathyroids (in addition to four retrothyroidal glands) within the thoracic confines.
Thymic involution The thymus is relatively large in childhood, grows during puberty, and decreases in weight after adolescence as a result of a normal involution process that is completed by age 40 1-6. Abnormal acute involution with marked reduction in the weight of the gland can also occur in response to stress, as a result of a pathologic process that is mediated by endogenous corticosteroid secretion 3,4,7,8. Acute involution can be detected on imaging studies and is associated with hyaline membrane disease in newborns, trauma, infection, radiation therapy, and other conditions. Microscopically, the thymus in acute involution shows widespread lympholysis and infiltration by macrophages with large somewhat foamy cytoplasm (Fig. 5.1), giving the gland a typical “starry-sky” appearance (Fig. 5.2). These changes are most prominent in the thymic cortex. Studies using immunohistochemistry have demonstrated that the epithelial network remains unchanged in the acutely involuted thymus, while there is a progressive loss of cortical immature proliferating lymphoid cells and medullary interdigitating cells. The degree of acute thymic involution in infancy and childhood correlates with the duration of an acute illness.
Even if degenerative change in true neoplasms is considered, intrathoracic cysts are relatively uncommon; most lesions in this category are probably congenital, and they comprise 10-15% of radiographically-detected masses in the mediastinum 1. Histologically, several tissue types are represented in neoplastic and non-neoplastic mediastinal cysts (Table 12.1), including parathyroid, thymic, bronchogenic, enteric, germinal, lymphoid, pericardial, and metastatic epithelial elements 2,3. The clinicopathologic attributes of such lesions are considered in this chapter. Embryologic information To understand the likely origins of most cysts in the mediastinum, it is appropriate to consider selected details of thoracic embryologic development. Specifically, these concern the pharyngeal pouches, the respiratory diverticulum, the primitive gut, and the pleuropericardial membranes.
