scholarly journals A 57-year-old man with rapidly progressive pulmonary hypertension

2021 ◽  
Author(s):  
Federico Raimondi ◽  
Caterina Conti ◽  
Luca Novelli ◽  
Francesco Tarantini ◽  
Giuseppe Ciaravino ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Presentation ◽  
Rare Condition ◽  
Hemodynamic Instability ◽  
Pulmonary Vasculature ◽  
Cancer History ◽  
Progressive Dyspnea ◽  
History Of ◽  
Neoplastic Disorders ◽  
Prompt Diagnosis

Pulmonary Tumor Thrombotic Microangiopathy (PTTM) is a rare condition associated with neoplastic disorders, predominantly gastric cancer, leading to pre-capillary Pulmonary Hypertension (PH). The pathologic mechanism involved is a fibrocellular intimal proliferation of small pulmonary vessels sustained by nests of carcinomatous cells lodged in pulmonary vasculature. Clinical presentation is nonspecific, including progressive dyspnea and dry cough. Diagnosis of PTTM is extremely challenging ante-mortem and prognosis is poor. Here we describe the case of a middle-aged man, without known previous cancer history. The clinical course was rapidly unfavorable, with progressive dyspnea and PH associated with hemodynamic instability, eventually culminating in patient’s death. PTTM diagnosis was made post-mortem. PTTM should be considered in any patient presenting with unexplained PH, especially if it is rapidly progressive, poorly responsive to standard approaches or there is suspected history of malignancy. A prompt diagnosis of PTTM could help in bringing light into this still under-recognized condition.

scholarly journals Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review

2016 ◽  
Vol 6 ◽  
pp. 32 ◽  
Author(s):  
Ronald Tan ◽  
Joanne Martires ◽  
Nader Kamangar
Keyword(s):  
Soft Tissue ◽  
Clinical Presentation ◽  
Late Complication ◽  
Rare Condition ◽  
Prior History ◽  
Fibrosing Mediastinitis ◽  
Granulomatous Infection ◽  
Progressive Dyspnea ◽  
History Of ◽  
Active Processes

Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded.

Intraorbital Abscess: A Rare Cause of Orbit Compression

FACE ◽  
2021 ◽  
pp. 273250162110050
Author(s):  
Samuel Ruiz ◽  
Rizal Lim
Keyword(s):  
Pediatric Population ◽  
Rare Complication ◽  
Disease Process ◽  
Rare Condition ◽  
External Drainage ◽  
Direct Extension ◽  
Life Threatening ◽  
History Of ◽  
High Index Of Suspicion ◽  
Prompt Diagnosis

Introduction: Intraorbital abscess is a rare complication of rhinosinusitis that affects most commonly the pediatric population. It is thought to be caused by direct extension or venous spread of infections from contiguous sites and can lead to life-threatening complications, like permanent visual loss and cerebral abscesses. Objectives: Intraorbital abscess is a rare condition that requires prompt diagnosis and treatment to avoid serious complications. Our objectives are to provide an overview of this rare disease process and its management including our successful treatment experience. Case Description: We present a 2 case report of a 13-year-old pediatric male and a 66-year-old male with history of chronic sinusitis who presented with a right intraorbital abscess successfully treated with external drainage with decompression of the orbit. Conclusion: When intraorbital abscess is encountered, a high index of suspicion is needed to allow prompt and accurate diagnosis for this infrequent condition. Timely surgical drainage of the abscess is needed to prevent the development of fatal complications.

scholarly journals Obstetrical Outcomes of Head and Neck (Nonthyroid) Cancers: A 27-Year Retrospective Series and Literature Review

2019 ◽  
Vol 09 (01) ◽  
pp. e15-e22
Author(s):  
Ernesto Figueiró-Filho ◽  
Richard Horgan ◽  
Nidal Muhanna ◽  
Jacqueline Parrish ◽  
Jonathan Irish ◽  
...  
Keyword(s):  
Head And Neck ◽  
Clinical Presentation ◽  
Demographic Data ◽  
Perinatal Outcomes ◽  
Rare Condition ◽  
Review Literature ◽  
Review Of The Literature ◽  
Obstetrical Outcomes ◽  
Time Period ◽  
History Of

Objective To describe the clinical presentation and obstetrical outcomes of nonthyroid head and neck cancers (HNCs), and to review literature on this rare condition in pregnancy. Study Design Pregnant women with nonthyroid HNC were identified retrospectively from 1990 to 2017. Maternal, neonatal, pregnancy, and demographic data were collected. A review of the literature from January 1980 to May 2018 was performed. Results Over the 27-year time period, 16 women with history of nonthyroid HNC were identified (9 diagnosed during and 7 diagnosed before current pregnancy). The cases were analyzed in detail and the most updated review of management of each type of HNC was provided. Conclusions HNCs are rare with diagnosis and management challenges during pregnancy. In this series, the cases diagnosed and managed previously to pregnancy presented better perinatal outcomes than the cases presented during pregnancy. The maternal outcomes appeared similar for HNC diagnosed before or after pregnancy.

scholarly journals Identification of the Novel Variants in Patients With Chronic Thromboembolic Pulmonary Hypertension

2020 ◽  
Vol 9 (21) ◽  
Author(s):  
Nobuhiro Yaoita ◽  
Kimio Satoh ◽  
Taijyu Satoh ◽  
Toru Shimizu ◽  
Sakae Saito ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Vasculature ◽  
Nonsynonymous Variant ◽  
Whole Exome ◽  
Thromboembolic Pulmonary Hypertension ◽  
History Of ◽  
Pulmonary Arterial

Background Although chronic thromboembolic pulmonary hypertension (CTEPH) and acute pulmonary embolism (APE) share some clinical manifestations, a limited proportion of patients with CTEPH have a history of APE. Moreover, in histopathologic studies, it has been revealed that pulmonary vasculature lesions similar to pulmonary arterial hypertension existed in patients with CTEPH. Thus, it remains unknown whether these 3 disorders also share genetic backgrounds. Methods and Results Whole exome screening was performed with DNA isolated from 51 unrelated patients with CTEPH of Japanese ancestry. The frequency of genetic variants associated with pulmonary arterial hypertension or APE in patients with CTEPH was compared with those in the integrative Japanese Genome Variation Database 3.5KJPN. Whole exome screening analysis showed 17 049 nonsynonymous variants in patients with CTEPH. Although we found 6 nonsynonymous variants that are associated with APE in patients with CTEPH, there was no nonsynonymous variant associated with pulmonary arterial hypertension. Patients with CTEPH with a history of APE had nonsynonymous variants of F5 , which encodes factor V. In contrast, patients with CTEPH without a history of APE had a nonsynonymous variant of THBD , which encodes thrombomodulin. Moreover, thrombin‐activatable fibrinolysis inhibitor, which is one of the pathogenic proteins in CTEPH, was significantly more activated in those who had the variants of THBD compared with those without it. Conclusions These results provide the first evidence that patients with CTEPH have some variants associated with APE, regardless of the presence or absence of a history of APE. Furthermore, the variants might be different between patients with CTEPH with and without a history of APE.

scholarly journals Pelvic and Bladder Catecholamine-Producing Tumors: A Review of the Literature

2019 ◽  
Author(s):  
Luigi Petramala ◽  
Valeria Bisogni ◽  
Federica Olmati ◽  
Antonio Concistrè ◽  
Monia Celi ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Case Reports ◽  
Bladder Neoplasms ◽  
Signs And Symptoms ◽  
Review Of The Literature ◽  
Current Management ◽  
Prisma Statement ◽  
History Of ◽  
Biochemical Remission ◽  
Prompt Diagnosis

Abstract Background Although PGLs may arise at any site where physiologic paraganglionic tissue exists, pelvic origins are not frequent (about 2%), and the most common localization is the retroperitoneal space, whereas bladder PGLs account for less than 0.06% of all bladder neoplasms and less than 1% of all PGLs. Starting from the description of two interesting our cases, we wondered to review the literature on pelvic and bladder paragangliomas (PGLs) with the aim of illustrating clinical characteristics and current management, and enlightening the needed of a prompt diagnosis to avoid the onset of several complications.Methods We herein performed a brief review of the literature about pelvic PGLs in the PubMed library, conducted according to the PRISMA statement, including studies published from 1954 to 2019. We also discuss clinical presentation, morphologic appearance, and management of two cases of pelvic PGL, both of them characterized by a history of paroxysmal high blood pressure episodes uncontrolled by antihypertensive therapy associated with disabling symptoms, misdiagnosed for many years.Results Overall, we evaluated 108 case reports. The majority were females, presenting typical signs and symptoms, such as arterial hypertension (54.2%), hematuria (35.1%), and headache (33.6%). After treatment, the clinical and biochemical remission was showed in only 55% of analyzed cases.Conclusions For their singular localization, pelvic and bladder PGLs often might be misdiagnosed. The resulting delay in the proper treatment could be responsible for the relative low percentage of complete clinical and biochemical remission.

scholarly journals Trigeminal Trophic Syndrome—A Unique Clinical Presentation of a Rare Condition

2019 ◽  
Vol 98 (10) ◽  
pp. 606-608
Author(s):  
Kathryn H. Bradburn ◽  
Dirk Elston ◽  
Alexander W. Murphey ◽  
Krishna G. Patel
Keyword(s):  
Clinical Presentation ◽  
Rare Condition ◽  
Targeted Treatment ◽  
Rare Syndrome ◽  
Appropriate Treatment ◽  
Nasal Ala ◽  
Trigeminal Trophic Syndrome ◽  
Prompt Diagnosis

This study describes a unique clinical presentation of trigeminal trophic syndrome (TTS), which is not well described within the otolaryngology literature. Trigeminal trophic syndrome classically presents with a triad of symptoms: trigeminal anesthesia, facial paresthesias, and crescent-shaped ulceration of the lateral nasal ala. The patient discussed in this report had a self-induced, waxing and waning ulceration of the frontal scalp for 7 years and was evaluated and treated ineffectively by multiple physicians, including otolaryngologists, before TTS was diagnosed and a targeted treatment was initiated. Although extranasal presentation is uncommon, this condition must be considered when ulcers are encountered in the trigeminal dermatome. This case highlights the variability in presentation and the importance of awareness of this rare syndrome. We aim to facilitate more prompt diagnosis and expedite the initiation of appropriate treatment for TTS in the field of otolaryngology.

INTRAOSSEOUS ANGIOLIPOMA OF THE CRANIUM

Neurosurgery ◽  
2009 ◽  
Vol 64 (1) ◽  
pp. E189-E190 ◽  
Author(s):  
Kenny Yu ◽  
James Van Dellen ◽  
Philip Idaewor ◽  
Federico Roncaroli
Keyword(s):  
Clinical Presentation ◽  
Subcutaneous Tissue ◽  
Rare Condition ◽  
Surgical Excision ◽  
Benign Tumors ◽  
Complete Surgical Excision ◽  
History Of ◽  
Titanium Cranioplasty ◽  
Intraosseous Lesion ◽  
Slow Growing

Abstract OBJECTIVE We describe an intraosseous angiolipoma of the cranium and discuss the outcome. Angiolipomas are benign tumors that consist of mature adipose tissue and abnormal vessels. They occur predominantly in the subcutaneous tissue of the trunk and upper limbs. Only 4 examples of intraosseous angiolipomas have been reported in the literature, all of which involved the mandible and ribs. CLINICAL PRESENTATION A 39-year-old man presented with a right parietal swelling. The patient initially refused surgery; thus it was possible to follow this case for 11 years, allowing us to evaluate the natural history of this rare condition. INTERVENTION Complete surgical excision of the intraosseous lesion was achieved with a titanium cranioplasty performed at intervals. Fifteen months after surgery, no recurrence was seen. CONCLUSION This is the first known report of intraosseous angiolipoma of the cranium. Angiolipomas are rare, benign, slow-growing tumors with an excellent prognosis. On preoperative neuroimaging, they may mimic intraosseous angiomas, lipomas, or intraosseous meningiomas. Total resection is curative.

scholarly journals Mitochondrial dysfunction and pulmonary hypertension: cause, effect, or both

2018 ◽  
Vol 314 (5) ◽  
pp. L782-L796 ◽  
Author(s):  
Jeffrey D. Marshall ◽  
Isabel Bazan ◽  
Yi Zhang ◽  
Wassim H. Fares ◽  
Patty J. Lee
Keyword(s):  
Endothelial Cells ◽  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Mitochondrial Dysfunction ◽  
Clinical Manifestations ◽  
Progressive Increase ◽  
Pulmonary Vasculature ◽  
Progressive Dyspnea ◽  
Transport Chain

Pulmonary hypertension describes a heterogeneous disease defined by increased pulmonary artery pressures, and progressive increase in pulmonary vascular resistance due to pathologic remodeling of the pulmonary vasculature involving pulmonary endothelial cells, pericytes, and smooth muscle cells. This process occurs under various conditions, and although these populations vary, the clinical manifestations are the same: progressive dyspnea, increases in right ventricular (RV) afterload and dysfunction, RV-pulmonary artery uncoupling, and right-sided heart failure with systemic circulatory collapse. The overall estimated 5-yr survival rate is 72% in highly functioning patients, and as low as 28% for those presenting with advanced symptoms. Metabolic theories have been suggested as underlying the pathogenesis of pulmonary hypertension with growing evidence of the role of mitochondrial dysfunction involving the major proteins of the electron transport chain, redox-related enzymes, regulators of the proton gradient and calcium homeostasis, regulators of apoptosis, and mitophagy. There remain more studies needed to characterize mitochondrial dysfunction leading to impaired vascular relaxation, increase proliferation, and failure of regulatory mechanisms. The effects on endothelial cells and resulting interactions with their microenvironment remain uncharted territory for future discovery. Additionally, on the basis of observations that the “plexigenic lesions” of pulmonary hypertension resemble the unregulated proliferation of tumor cells, similarities between cancer pathobiology and pulmonary hypertension have been drawn, suggesting interactions between mitochondria and angiogenesis. Recently, mitochondria targeting has become feasible, which may yield new therapeutic strategies. We present a state-of-the-art review of the role of mitochondria in both the pathobiology of pulmonary hypertension and potential therapeutic targets in pulmonary vascular processes.

scholarly journals Tumoral pulmonary hypertension

2019 ◽  
Vol 28 (151) ◽  
pp. 180065 ◽  
Author(s):  
Laura C. Price ◽  
Michael J. Seckl ◽  
Peter Dorfmüller ◽  
S. John Wort
Keyword(s):  
Pulmonary Hypertension ◽  
Kinase Inhibitors ◽  
Chemotherapeutic Agents ◽  
Pulmonary Vasculature ◽  
Management Options ◽  
Pulmonary Vasodilators ◽  
Underlying Malignancy ◽  
History Of ◽  
Pulmonary Arterial ◽  
A Current

Tumoral pulmonary hypertension (PH) comprises a variety of subtypes in patients with a current or previous malignancy. Tumoral PH principally includes the tumour-related pulmonary microvascular conditions pulmonary tumour microembolism and pulmonary tumour thrombotic microangiopathy. These inter-related conditions are frequently found inpost mortemspecimens but are notoriously difficult to diagnoseante mortem. The outlook for patients remains extremely poor although there is some emerging evidence that pulmonary vasodilators and anti-inflammatory approaches may improve survival. Tumoral PH also includes pulmonary macroembolism and tumours that involve the proximal pulmonary vasculature, such as angiosarcoma; both may mimic pulmonary embolism and chronic thromboembolic PH. Finally, tumoral PH may develop in response to treatments of an underlying malignancy. There is increasing interest in pulmonary arterial hypertension induced by tyrosine kinase inhibitors, such as dasatanib. In addition, radiotherapy and chemotherapeutic agents such as mitomycin-C can cause pulmonary veno-occlusive disease. Tumoral PH should be considered in any patient presenting with unexplained PH, especially if it is poorly responsive to standard approaches or there is a history of malignancy. This article will describe subtypes of tumoral PH, their pathophysiology, investigation and management options in turn.

scholarly journals Comatose patient with hypothermia, dyspnea, and general edema in the emergency department: a case report

2018 ◽  
Vol 46 (10) ◽  
pp. 4338-4342 ◽  
Author(s):  
Shang-Li Tsai ◽  
Chi-Chun Lin ◽  
Cheng-Yu Lin ◽  
Chang Keng-Wei ◽  
Cheng-Yu Chien
Keyword(s):  
Emergency Department ◽  
Cerebrovascular Accidents ◽  
Myxedema Coma ◽  
Multiple Organs ◽  
Appropriate Treatment ◽  
Progressive Dyspnea ◽  
General Weakness ◽  
Slow Presentation ◽  
History Of ◽  
Prompt Diagnosis

Patients presenting to the emergency department with hypothermia are rare and often require prompt diagnosis and management. Myxedema coma, which may cause severe hypothermia, is a true endocrine emergency requiring early and appropriate treatment. We report on a 47-year-old woman with a history of hyperthyroidism who underwent thyroidectomy 5 years previously, with no regular medication or examinations. She presented to the emergency department with a 1-month history of progressive dyspnea associated with general weakness. She also showed hypothermia, decreased mental status, and general edema. Echocardiography revealed increased pericardial effusion without tamponade. Laboratory examination suggested myxedema coma and hypothyroidism. She received thyroxine, glucocorticoid supplement, and intensive supportive care, after which she gradually improved and was discharged. This case suggests that myxedema coma should be considered in patients with hypothyroidism or a history of thyroidectomy who present with change in consciousness, hypothermia, or other symptoms related to critical or slow presentation in multiple organs. Moreover, long-standing hypothyroidism or precipitating acute events such as sepsis, cerebrovascular accidents, gastrointestinal bleeding, cold exposure, trauma, and some medications may also cause myxedema coma. Myxedema coma is associated with a high mortality, and patients suspected to be suffering from this condition should be treated without delay.

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