scholarly journals Unilateral Eyelid Edema as Initial Sign of Orbital Sarcoidosis

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Sílvia Miguéis Picado Petrarolha ◽  
Bruna Suda Rodrigues ◽  
Flávio David Haddad Filho ◽  
Rogério Aparecido Dedivitis ◽  
Samuel Brunini Petrarolha ◽  
...  
Keyword(s):  
Soft Tissues ◽  
Final Diagnosis ◽  
Lower Eyelid ◽  
Unknown Etiology ◽  
Ocular Motility ◽  
Eyelid Edema ◽  
Hilar Adenopathy ◽  
Initial Sign ◽  
Eye Examination ◽  
Histological Confirmation

Introduction. Sarcoidosis is a rare multisystemic granulomatous inflammatory disease of unknown etiology affecting the respiratory system, skin, and eyes. Sarcoidosis outside the lacrimal gland is rare. The case study concerns a patient with a final diagnosis of orbital sarcoidosis.Case Report. A 37-year-old male patient went to the ophthalmic emergency room complaining of pain in the left eye, diplopia, and decreased visual acuity. An external eye examination showed hard and cold edema of the lower eyelid, ocular motility with limitation of adduction, and discreet ipsilateral proptosis. Magnetic resonance of the orbit showed left eye proptosis and thickening and increase of soft tissues associated with heterogeneous impregnation of contrast in the infralateral region of the left eyelid. A biopsy of the lesion showed a chronic inflammatory process, with numerous compact nonnecrotizing granulomas surrounded by lamellar hyaline collagen, providing histological confirmation of sarcoidosis.Discussion. A biopsy of the orbital tumor is essential for the diagnosis of sarcoidosis, in addition to the search for systemic findings such as hilar adenopathy or parenchymal lung disease found in 90% of patients.

scholarly journals Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

Cells ◽  
2021 ◽  
Vol 10 (4) ◽  
pp. 766
Author(s):  
Pascal Sève ◽  
Yves Pacheco ◽  
François Durupt ◽  
Yvan Jamilloux ◽  
Mathieu Gerfaud-Valentin ◽  
...  
Keyword(s):  
Granulomatous Inflammation ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Initial Manifestation ◽  
Clinical Criteria ◽  
System Disease ◽  
Lupus Pernio ◽  
Splenic Involvement ◽  
Hilar Adenopathy ◽  
Histological Confirmation

Sarcoidosis is a multi-system disease of unknown etiology characterized by the formation of granulomas in various organs. It affects people of all ethnic backgrounds and occurs at any time of life but is more frequent in African Americans and Scandinavians and in adults between 30 and 50 years of age. Sarcoidosis can affect any organ with a frequency varying according to ethnicity, sex and age. Intrathoracic involvement occurs in 90% of patients with symmetrical bilateral hilar adenopathy and/or diffuse lung micronodules, mainly along the lymphatic structures which are the most affected system. Among extrapulmonary manifestations, skin lesions, uveitis, liver or splenic involvement, peripheral and abdominal lymphadenopathy and peripheral arthritis are the most frequent with a prevalence of 25–50%. Finally, cardiac and neurological manifestations which can be the initial manifestation of sarcoidosis, as can be bilateral parotitis, nasosinusal or laryngeal signs, hypercalcemia and renal dysfunction, affect less than 10% of patients. The diagnosis is not standardized but is based on three major criteria: a compatible clinical and/or radiological presentation, the histological evidence of non-necrotizing granulomatous inflammation in one or more tissues and the exclusion of alternative causes of granulomatous disease. Certain clinical features are considered to be highly specific of the disease (e.g., Löfgren’s syndrome, lupus pernio, Heerfordt’s syndrome) and do not require histological confirmation. New diagnostic guidelines were recently published. Specific clinical criteria have been developed for the diagnosis of cardiac, neurological and ocular sarcoidosis. This article focuses on the clinical presentation and the common differentials that need to be considered when appropriate.

scholarly journals Incidence, characteristics, determinants and prognostic impact of recurrent syncope

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Zimmermann ◽  
J Du Fay De Lavallaz ◽  
T Nestelberger ◽  
D Gualandro ◽  
P Badertscher ◽  
...  
Keyword(s):  
Patient Characteristics ◽  
Coronary Intervention ◽  
Final Diagnosis ◽  
Major Adverse Cardiovascular Events ◽  
Unknown Etiology ◽  
Funding Source ◽  
Prognostic Impact ◽  
Lasso Regression ◽  
Recurrence Rates ◽  
Increased Risk

Abstract Background The incidence, characteristics, determinants, and prognostic impact of recurrent syncope are largely unknown, causing uncertainty for both patients and physicians. Methods We characterized recurrent syncope including sex-specific aspects and its impact on death and major adverse cardiovascular events (MACE) in a large prospective international multicenter study enrolling patients ≥40 years presenting with syncope to the emergency department (ED). Syncope etiology was centrally adjudicated by two independent and blinded cardiologists using all information becoming available during syncope work-up and 12-month follow-up. MACE were defined as a composite of all-cause death, acute myocardial infarction, surgical or percutaneous coronary intervention, life-threatening arrhythmia including cardiac arrest, pacemaker or implantable cardioverter defibrillator implantation, valve intervention, heart-failure, gastrointestinal bleeding or other bleeding requiring transfusion, intracranial hemorrhage, ischemic stroke or transient ischemic attack, sepsis and pulmonary embolism. Results Incidence of recurrent syncope among 1790 patients was 20% (95%-confidence interval (CI) 18% to 22%) within 24 months. Patients with an adjudicated final diagnosis of cardiac syncope (hazard ratio (HR) 1.50, 95%-CI 1.11 to 2.01) or syncope of unknown etiology even after central adjudication (HR 2.11, 95%-CI 1.54 to 2.89) had an increased risk for syncope recurrence (Figure). LASSO regression fit on all patient information available early in the ED identified more than three previous episodes of syncope as the only independent predictor for recurrent syncope (HR 2.13, 95%-CI 1.64 to 2.75). Recurrent syncope within the first 12 months after the index event carried an increased risk for all-cause death (HR 1.59, 95%-CI 1.06 to 2.38) and MACE (HR 2.24, 95%-CI 1.67 to 3.01), whereas recurrences after 12 months did not have a significant impact on outcome measures. Conclusion Recurrence rates of syncope are substantial and vary depending on syncope etiology. There seem to be no reliable patient characteristics available early on the ED that allow for the prediction of recurrent syncope with only a history of more than three previous syncope being associated with a higher risk for future recurrences. Importantly, recurrent syncope within the first 12 months carries an increased risk for death and MACE. Figure 1 Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Swiss National Science Foundation, Swiss Heart Foundation

scholarly journals The accuracy of initial diagnoses in coma: an observational study in 835 patients with non-traumatic disorder of consciousness

2021 ◽  
Vol 29 (1) ◽  
Author(s):  
Maximilian Lutz ◽  
Martin Möckel ◽  
Tobias Lindner ◽  
Christoph J. Ploner ◽  
Mischa Braun ◽  
...  
Keyword(s):  
Emergency Care ◽  
Final Diagnosis ◽  
Class I ◽  
Prehospital Emergency Care ◽  
Unknown Etiology ◽  
Care Providers ◽  
Cohen’S Kappa ◽  
Disorder Of Consciousness ◽  
Cohen's Kappa ◽  
Suspected Diagnosis

Abstract Background Management of patients with coma of unknown etiology (CUE) is a major challenge in most emergency departments (EDs). CUE is associated with a high mortality and a wide variety of pathologies that require differential therapies. A suspected diagnosis issued by pre-hospital emergency care providers often drives the first approach to these patients. We aim to determine the accuracy and value of the initial diagnostic hypothesis in patients with CUE. Methods Consecutive ED patients presenting with CUE were prospectively enrolled. We obtained the suspected diagnoses or working hypotheses from standardized reports given by prehospital emergency care providers, both paramedics and emergency physicians. Suspected and final diagnoses were classified into I) acute primary brain lesions, II) primary brain pathologies without acute lesions and III) pathologies that affected the brain secondarily. We compared suspected and final diagnosis with percent agreement and Cohen’s Kappa including sub-group analyses for paramedics and physicians. Furthermore, we tested the value of suspected and final diagnoses as predictors for mortality with binary logistic regression models. Results Overall, suspected and final diagnoses matched in 62% of 835 enrolled patients. Cohen’s Kappa showed a value of κ = .415 (95% CI .361–.469, p < .005). There was no relevant difference in diagnostic accuracy between paramedics and physicians. Suspected diagnoses did not significantly interact with in-hospital mortality (e.g., suspected class I: OR .982, 95% CI .518–1.836) while final diagnoses interacted strongly (e.g., final class I: OR 5.425, 95% CI 3.409–8.633). Conclusion In cases of CUE, the suspected diagnosis is unreliable, regardless of different pre-hospital care providers’ qualifications. It is not an appropriate decision-making tool as it neither sufficiently predicts the final diagnosis nor detects the especially critical comatose patient. To avoid the risk of mistriage and unnecessarily delayed therapy, we advocate for a standardized diagnostic work-up for all CUE patients that should be triggered by the emergency symptom alone and not by any suspected diagnosis.

scholarly journals A Case of Sarcoidosis Disseminated to Skeletal Tissues

2013 ◽  
Vol 1 (1) ◽  
pp. 43-45
Author(s):  
Edon Rabinowitz ◽  
Chinwe Ogedegbe ◽  
Joseph Feldman
Keyword(s):  
Cell Tumor ◽  
Unknown Etiology ◽  
Granulomatous Disease ◽  
Presenting Symptoms ◽  
Organ Systems ◽  
Mediastinal Germ Cell Tumor ◽  
History Of ◽  
Hilar Adenopathy ◽  
Eye Lesions ◽  
Musculoskeletal Involvement

Sarcoidosis is a systemic granulomatous disease of unknown etiology that typically affects young adults. Diagnostic criteria for sarcoidosis include involvement of two or more of the following organ systems: 1) pulmonary infiltrates; 2) bilateral hilar adenopathy; and 3) skin and/or eye lesions. Musculoskeletal system is less commonly involved. For that reason potential presenting symptoms can vary and make the diagnosis very challenging; particularly if a patient has symptoms that mimic other conditions. Musculoskeletal involvement for example can mimic malignancy. The following case describes a patient with known history of primary metastatic mediastinal Germ Cell Tumor (GCT) with teratomatous elements who is diagnosed with sarcoidosis involving skeletal tissues.

scholarly journals A Rare Case of Kimura Disease in the Wrist

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4881-4881
Author(s):  
Parastou Tizro ◽  
Taraneh Hashemi Zonouz ◽  
Fawaz Almutairi ◽  
Donald Karcher
Keyword(s):  
Soft Tissue ◽  
Soft Tissues ◽  
Conflicts Of Interest ◽  
Soft Tissue Mass ◽  
Germinal Centers ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Kimura Disease ◽  
High Endothelial Venules ◽  
Tissue Mass

Kimura disease is a rare benign chronic inflammatory disorder of unknown etiology that typically involves lymph nodes and soft tissues. This disease is most common in middle-aged Asian men. Although the disorder most frequently involves the head and neck region, it has also been reported to involve the extremities. Lesions typically show follicular lymphocytic hyperplasia with prominent interfollicular venules and a mixed inflammatory infiltrate, including numerous eosinophils. We describe a case of Kimura disease in a 29-year-old female presenting with a slowly enlarging, painless soft tissue mass in her left wrist for five months. The lesion was non-tender, mobile, and compressible. Lab results were significant for peripheral blood eosinophilia and elevated IgE. Microscopically, the mass was circumscribed, with apparent fibrous capsule or pseudocapsule, prominent high-endothelial venules, and inflammatory cells consisting of lymphocytes and eosinophils. There were prominent secondary B-lymphocytic follicles with germinal centers containing many penetrating small blood vessels, and some follicles exhibiting follicle lysis and a small amount of eosinophilic extracellular material. Polykaryocytes were noted in many germinal centers. Angiolymphoid hyperplasia with eosinophilia, a main differential diagnosis of Kimura disease, was considered; however, the combined clinical and morphologic findings appeared to be more consistent with Kimura disease, particularly in the context of eosinophilia and elevated IgE level. Kimura disease may present as a rare cause of a soft tissue mass and a diagnostic challenge for clinicians and pathologists, especially in less common sites such as the wrist. Disclosures No relevant conflicts of interest to declare.

Comparative Study of the Usefulness of the Binocular Spot Vision Screener Autorefractor in the Detection of Childhood Visual Disorders

2018 ◽  
Vol 235 (04) ◽  
pp. 416-419 ◽  
Author(s):  
Nathalie Voide ◽  
Nicole Hoeckele ◽  
Pierre-François Kaeser
Keyword(s):  
Visual Acuity ◽  
Clinical Examination ◽  
Screening Tool ◽  
Ocular Motility ◽  
Primary Position ◽  
Visual Potential ◽  
Visual Acuity Loss ◽  
Eye Examination ◽  
Visual Screening ◽  
Vision Screener

Abstract Background The Spot Vision Screener (SVS) is designed to detect significant ametropia, anisometropia, and strabismus in non-dilated eyes. This study evaluates the efficacy of the SVS in paediatric visual screening. Patients and Methods All children screened during the paediatric visual screening day in Lausanne in 2016 were evaluated with the SVS, conventional monocular autorefractors, and clinical orthoptic examination. Recommendations for a further eye examination of the SVS were compared with those issued from traditional clinical screenings (monocular refraction and orthoptic examination). Results One hundred and sixty-eight consecutive children were included. The median age was 3.9 years. The SVS median spherical equivalent (SE) was + 0.25 D OU and it detected seven cases of (4.2%) anisometropia (SE difference ≥ 1 D). The conventional monocular autorefractor median SE was − 0.13 D OU and 20 cases of anisometropia (11.9%) were detected. Refraction could not be measured in 1.2% of patients with SVS versus 17.2% with monocular refractors. The SVS screened two manifest strabismus cases against five manifest and > 100 latent strabismus with orthoptic examination. As expected, the SVS was unable to assess reactions to monocular occlusion, visual acuity, and stereovision as well as to detect ocular motility disorders without strabismus in the primary position, and missed two cases of abnormal Brückner reflexes. Overall, the SVS identified 66 suspect patients (39.3%) against 102 (60.7%) after complete clinical examination. Conclusions The SVS can be a useful objective screening tool for non-ophthalmologists. However, because it fails to detect ocular motility troubles, organic visual acuity loss, or to assess the visual potential, it should only be used in association with a clinical examination, even in routine screening procedures.

scholarly journals SUN-605 Mediastino-Abdominal Lipomatosis: Deep Accumulation of Fat Mimicking a Respiratory Disease

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Ricardo Rafael Correa ◽  
Ghada Elshimy ◽  
Mahmoud Alsayed
Keyword(s):  
Healthy Lifestyle ◽  
Macrocytic Anemia ◽  
Final Diagnosis ◽  
Definitive Treatment ◽  
Unknown Etiology ◽  
Exertional Dyspnea ◽  
Low Fat Diet ◽  
Ct Angiogram ◽  
The Face ◽  
History Of

Abstract Introduction: Multiple systemic lipomatosis (MSL) is a rare disorder with unknown etiology. It is characterized by the massive development of non-encapsulated lipomas in subcutaneous tissues. Lipomatosis of the face, head, neck, extremities, abdomen, and pelvis have been reported in the literature. Case: We report a case of a 65 years old female (BMI: 34 kg/m2) with past medical history of hypertension, hyperlipidemia(DLD) diabetes mellitus type 2 and sleep apnea that was brought into emergency room (ER) for worsening shortness of breath. In the ER, she was having an oxygen saturation of 75% and required intubation. The patient was afebrile, with a BP of 120/75 mm Hg and a heart rate of 70/minute. Trans-thoracic echocardiogram revealed normal ejection fraction and normal pulmonary pressure with no wall motion abnormality. CXR showed no infiltration or consolidation. CT angiogram (CTA) ruled out pulmonary embolism but it was notable for large deposits of fat involving the abdomen and thorax, with invasion into the mediastinum and the space between the liver and diaphragm. Mesenteric fat was increased. Tissue was biopsied, which confirmed the diagnosis of fatty invasion. Discussion: Abdominal lipomatosis is characterized by massive enlargement of the abdomen due to intraperitoneal and retroperitoneal fatty deposits. Phenotypically patients can appear to be thin or obese, however, it is more common in the overweight population like our patient. Mediastinal lipomatosis is a benign cause of mediastinal widening, however patients can develop respiratory symptoms like exertional dyspnea due to compression of airways. MSL affects white caucasian between 25-60 years old and it is associated with DLD, impaired glucose tolerance, hyperuricemia, macrocytic anemia, and peripheral neuropathy. The pathophysiology is not fully understood, some theories stated that it is related to defective lipid mobilization in lipomatocytes, other suggested disorder in the mitochondria of brown fat. Conclusion: CT and MRI of the abdomen and chest are very helpful in the diagnosis of MSL but a tissue biopsy is what makes the final diagnosis. There is no definitive treatment; the recommendations are a healthy lifestyle including a low-fat diet, abstinence from alcohol and exercise. In severe cases, surgery is recommended.

scholarly journals Langerhans cell histiocytosis of the rib presenting with pathological fracture: a case report

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Tao Zuo ◽  
Ping Jiang ◽  
Junjie Yu ◽  
Ke Zhao ◽  
Yong Liu ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pathological Fracture ◽  
Soft Tissues ◽  
Langerhans Cell ◽  
Rib Fracture ◽  
Unknown Etiology ◽  
Case Presentation ◽  
Left Chest ◽  
Rib Tumor

Abstract Introduction Langerhans cell histiocytosis (LCH) is a rare neoplastic hyperplasia with an unknown etiology. It is clinically rare for patients with solitary rib lesion and pathological fracture; moreover, its diagnosis and treatment are quite difficult. The purpose of this study is to present a case for the pathogenesis, clinical features, imaging, and treatment of this disease. Case presentation A 52-year-old female patient complained of left chest pain for one week. CT showed a fracture in the left 5th rib. The rib tumor was then resected and the surrounding muscles and soft tissues were accordingly resected. The patient was diagnosed with pathological rib fracture, and the patient was pathologically diagnosed with LCH. After surgery, no local recurrence or distant metastasis was reported during the two-year follow-up. Conclusions LCH should be treated by observation, chemotherapy, radiotherapy, or surgery, or using a combination of several methods. Moreover, primary tumor should be considered when rib fracture without trauma and tumor metastasis.

Immunosuppressant-Related Lower Eyelid Edema in Transplant Patients

2017 ◽  
Vol 33 ◽  
pp. S58-S60 ◽  
Author(s):  
Sarah A. Logan ◽  
Preeti J. Thyparampil ◽  
Michael T. Yen
Keyword(s):  
Lower Eyelid ◽  
Transplant Patients ◽  
Eyelid Edema

Rare proliferative and inflammatory pathologies localized in the temporal bone - a review of the literature

2019 ◽  
Vol 8 (2) ◽  
pp. 1-5
Author(s):  
Adam Roszkowski Roszkowski ◽  
Alicja Witkowska ◽  
Piotr Baranek ◽  
Anna Rzepakowska ◽  
Emilia Wnuk ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Fibrous Dysplasia ◽  
Temporal Bone ◽  
Inflammatory Pseudotumor ◽  
Soft Tissues ◽  
Bone Structure ◽  
Histopathological Examination ◽  
Fibrous Tissue ◽  
Diagnostic Methods ◽  
Unknown Etiology

Proliferative-inflammatory pathologies may occupy the temporal bone, resulting in: hearing loss, vestibular dysfunction, and neuropathies from cranial nerve compression. Although their occurrence is episodic, the appropriate diagnostic procedure is extremely important to achieve expected therapeutic effect. The aim of study was characterization of selected proliferative-inflammatory pathologies that may occupy the temporal bone: fibrous dysplasia, inflammatory pseudotumor, osteoradionecrosis, and presentation of diagnostic methods for the differentiation of these diseases as well as discussion on appropriate therapeutic options. Fibrous dysplasia (fibrous dysplasia) is a slowly progressive, benign bone disorder of unknown etiology characterized by abnormal proliferation of fibrous tissue. IPT (inflammatory pseudotumor) is a rare, non-malignant inflammatory process of unknown etiology, characterized by connective tissue proliferation and infiltration of inflammatory cells. Osteoradionecrosis of the temporal bone (TB-ORN) is a rare but potentially fatal complication of radiotherapy for head and neck cancer. Due to the similarity of symptoms with typical inflammatory conditions of middle ear (pain, otorrhea, hearing loss), selected disorders may be a dilemma regarding the diagnosis and proper further treatment. The clinical examination is mandatory, however radiological imaging may demonstrate the existence of specific changes and direct the diagnosis. The computed tomography of fibrous dysplasia shows the abnormal organization of the bone structure. Magnetic resonance, as the most sensitive for inflammatory pseudotumors, visualizes inflammatory infiltrates in soft tissues. The spiral tomography of temporal identifies the erosion in the course of osteoradionecrosis. However the final diagnosis may be establish post the histopathological examination and exclusion of the neoplastic process.

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