Spontaneous pneumothorax and hemothorax frequently precede the arterial and intestinal complications of vascular Ehlers-Danlos syndrome.

Vascular Ehlers-Danlos syndrome (vEDS) is a connective tissue disorder due to defective type III collagen production and is associated with arterial rupture, spontaneous intestinal perforation, and gravid uterine rupture. Spontaneous pneumothorax and/or hemothorax (P/HTX) also occurs in vEDS patients. The temporal relation of pulmonary manifestations to arterial and intestinal complications in vEDS has not been well described. This was investigated in a multi-institutional retrospective case series of vEDS patients with confirmatory testing for COL3A1 mutation between 2000 and 2012. Data abstracted included demographics, family histories, presentation, and management of associated complications. Ninety-six cases (39% males, mean age 38.6 ± 15.5 years, range 8-79) had confirmatory testing for vEDS. P/HTX was documented in 17 (17.7%) cases. Most P/HTX preceded the diagnosis of vEDS (81%). Diagnosis of vEDS was made after arterial or intestinal complications at a mean of 7 years (range 0-26) post the initial P/HTX. In conclusion, spontaneous P/HTX is an early manifestation of vEDS frequently preceding an arterial complication or intestinal perforation. Thus, a spontaneous P/HTX in a young patient should trigger a differential diagnosis that includes vEDS. This should lead to an investigation of other vEDS features and subsequent genetic testing if vEDS features are present.

IgG4-related disease can present as recurrent spontaneous hemothorax: a case report.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) encompasses a group of immune-mediated disorders that are gaining increasing recognition. Pulmonary presentations are common, with four types of patterns been described on radiography, including solid nodular, bronchovascular, ground glass opacities, and alveolar interstitial. Pleural thickening and pleural effusion have also been reported. However, there have been no reports of IgG4-RD that presents as spontaneous hemothorax. CASE PRESENTATION: A 61-year-old Chinese woman experienced recurrent right-sided chest pain and transient syncope. A significant decrease in her hemoglobin level and thick bloody pleural fluid demonstrated spontaneous hemothorax. The elevated serum IgG4 and histopathological analysis of the right pleura and intercostal lymph node specimens all supported the diagnosis of IgG4-RD in this patient. Further diagnostic evaluation did not reveal other causes for spontaneous hemothorax. She received steroids and no recurrent bleeding event occurred during a follow-up period of more than 1 year. CONCLUSION: Recurrent spontaneous hemothorax can be a rare manifestation of IgG4-RD, with pleural involvement as the most probable mechanism.

Neurofibromatosis 1 and massive hemothorax: a fatal combination.

Type 1 neurofibromatosis (NF 1), a rare genetic disease with autosomal dominant transmission, has typical dermatologic manifestations with pathognomonic Lisch nodules, and is rarely known for vascular alterations. Among these, aneurysmal dilatation is the most common form. We report a fatal case of massive hemothorax due to a spontaneous rupture of the left pulmonary artery branch micro-aneurysm in a NF 1 patient. Indeed, spontaneous rupture of these pathologic vessels is very rare in clinical practice and the literature, but, for its potentially life-threatening complications, there is the need for it to be taken into account in differential diagnosis. The origin of bleeding was first confirmed by computed tomography angiography (CTA). The patient's condition worsened suddenly leading to pulmonary hemorrhage and death. A clinical autopsy was required to assess the definitive cause of death.

Spontaneous Hemothorax, A Rare Face of Vertebral Osteochondroma.

BACKGROUND: Osteochondroma is the most common benign tumor of the bone. It is usually asymptomatic, but complications may result from mechanical injury to adjacent anatomic structures, such as the diaphragm and lung, when located intrathoracically. CASE REPORT: We report the unusual occurrence of a large hemothorax and lacerated right diaphragm in a 41-year-old woman caused by vertebral osteochondroma affecting the eleventh thoracic vertebra. Thoracoscopic exploration with resection of the osteochondroma and repair of the diaphragm was performed. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Spontaneous hemothorax is a potential life-threatening condition when the initial diagnosis is postponed and hemodynamic instability and hypovolemic shock occurs. Osteochondroma as a cause of spontaneous hemothorax is uncommon but may require urgent surgical intervention with video-assisted thoracoscopic surgery of thoracotomy to control the hemorrhage and prevent recurrence.

Hemoptysis in a patient with multifocal primary pulmonary angiosarcoma.

Primary pulmonary angiosarcoma (PPA) is a rare tumour arising from arterial or venous pulmonary vessels of various size. It is characterized by aggressive course and poor prognosis. The early diagnosis is difficult due to diverse clinical and radiological manifestations. We present a case report of 70 year-old man, active cigarette-smoker, with a 2-month history of non-massive hemoptysis. The thorax CT revealed several solid pulmonary nodules surrounded by areas of ground glass opacity. As bronchoscopy failed to deliver adequate tissue samples, video assisted thoracic surgery (VATS) with pleura and lung biopsy was necessary. Histopathological findings were consistent with pulmonary angiosarcoma. Since no extrapulmonary lesions were demonstrated, the final diagnosis of primary pulmonary angiosarcoma was made. The patient died three months after the onset of symptoms. Our case report highlights that differential diagnosis in patients with hemoptysis and pulmonary nodules should include primary pulmonary sarcoma.

Intercostal artery aneurysm presenting as a spontaneous hemothorax in a patient with neurofibromatosis.

Beyond the commonly known clinical presentation of neurofibromatosis, vascular pathologies are increasingly becoming a known complication. We present a case of a 41-year-old adult with neurofibromatosis type 1 who came with a right-sided spontaneous hemothorax due to a ruptured 13-mm fusiform aneurysm of the right posterior T9 intercostal artery. Patient underwent a transcatheter angiographic embolization with subsequent video-assisted thoracic surgery (VATS) for a retained hemothorax. Patient was discharged home on Hospital Day 5, and follow-up imaging demonstrated a complete resolution of the hemothorax. This presented case contributes to literature by demonstrating intra-arterial embolization as a viable option to obtain hemostasis in fragile vessels. However, this may not always result in hemostasis, and VATS should be considered to achieve and ensure complete hemostasis.

An Unusual Case of Hemothorax in an Ehlers-Danlos Syndrome Patient Following Sildenafil Use.

Ehlers-Danlos syndrome is a disorder of collagen production that affects the connective tissues of the body. It can cause several conditions and severely affect patients' quality of life and activities of daily living. Here, we present an unusual case of hemothorax in a patient with Ehlers-Danlos syndrome after sildenafil use. This manifested in shortness of breath and cough and prompted the patient to visit the emergency room. The hemothorax was treated surgically, and the patient recovered well. Sildenafil was initially prescribed via a telemedicine service without in-person consultation. It is important that physicans perform a thorough history and physical examination prior to prescribing medications to patients, especially those at risk for complications.

Case report: Spontaneous hemothorax following anticoagulation for microthrombi in severe COVID pneumonia.

Background: Anticoagulant therapy has become a hallmark of treatment for critically ill COVID patients. Gastrointestinal and intracranial hemorrhage are known major complications of anticoagulation, but spontaneous hemothorax is a rare event, particularly in the absence of pre-existing structural lung disease, vascular malformations, or genetic bleeding diatheses. Herein is a case of spontaneous hemothorax following anticoagulation for microthrombi in a patient with acute hypoxic respiratory failure due to Covid pneumonia. Case presentation: A 49 year old male with hypertension, asthma, and obesity was admitted for acute hypoxic respiratory failure due to Covid pneumonia. He was treated with dexamethasone, baricitinib, and therapeutic enoxaparin as empiric therapy for severe COVID disease. He subsequently developed a massive right hemothorax with associated hemorrhagic shock, which required initiation of massive transfusion protocol, vasopressor support and mechanical ventilation. No clear etiology for the hemothorax was determined upon investigations. The patient eventually improved and was discharged to a skilled nursing facility on chronic oxygen therapy. Conclusions: Various mechanisms have been proposed for the development of non-traumatic hemothoraces, including tearing of adhesions and rupture of vascularized bullae. Such explanations find support in radiologic and pathologic studies of pleural changes in Covid pneumonia, and likely played a role in the hemorrhage experienced by our patient.

Pleural angiosarcoma presenting with spontaneous hemothorax.

Angiosarcomas (ASs) are very rare and constitute 1-2% of soft tissue malignancies. Primary pleural AS (PPAS) is a very rare neoplasm, with only 50 cases reported in the literatüre, and is a tumor with a high tendency for local recurrence and metastasis, with an aggressive course and a generally poor prognosis unless diagnosed early. It originates from the endothelial cells of small blood vessels and therefore can affect many organs. The etiology and definitive method in the treatment is still unclear. Patients usually present with nonspecific symptoms such as cough, dyspnea, chest pain, and hemoptysis. Recurrent exudative or hemorrhagic pleural effusion may develop due to its pleural location. The diagnosis can be made by histopathological and immunohistochemical examinations of excisional biopsy specimens. The effectiveness of chemotherapy and radiotherapy is weak and can be applied for palliative purposes. Surgical approach can be used for diagnostic and palliative purposes. Due to the high degree of malignancy and insidious course of PPAS, patients usually die within months after diagnosis. In these patients, surgical exploration is important for the diagnosis and palliative/definitive treatment of the disease. We present a 61-year-old male patient who presented with dyspnea, chest pain, and massive pleural effusion findings in the left hemithorax and was diagnosed with PPAS as a result of pleural biopsy.

Rapid progressive spontaneous hemothorax caused by carcinoma of unknown primary: A very rare case.

We present a very rare case of spontaneous hemothorax which was caused by carcinoma of unknown primary. To the best of our knowledge, there are no other such cases reported worldwide. The patient was 72-year-old male who was referred to our department for massive hemothorax. We undertook surgical treatment for haemostasis, and found multiple tumours which led to the diagnosis of carcinoma of unknown primary as the cause of haemorrhage. The tumours increased and grew rapidly after surgery and hemothorax progressed despite our treatment. The patient died from disseminated intravascular coagulation caused by continuous bleeding on postoperative day 19.

Spontaneous hemothorax caused by rivaroxaban treatment for pulmonary embolism: A case report.

Hemothorax is a rare and potentially fatal condition characterized by pleural effusion containing over 50% of the patient's hematocrit. A massive hemothorax involves blood loss exceeding 1.5 L. Common causes include chest trauma, invasive thoracic procedures, anticoagulant medications, vascular anomalies, malignancies, and hematologic abnormalities. Spontaneous hemothorax may be seen in conjunction with pulmonary infarction and spontaneous pneumothorax. Anticoagulation is a key therapeutic strategy for certain thromboembolic events, such as pulmonary embolism. Historically, these events were treated with vitamin K antagonists (VKAs), which have demonstrated variable plasma concentrations and an increased risk of hemorrhage. With the advent of direct oral anticoagulants (DOACs), treatment has become as effective as VKAs while significantly reducing the risk of hemorrhage. However, some researchers have speculated that hemorrhagic complications in certain cases could be worse with DOACs than with VKAs. In the case presented here, we identified a genuine association between the use of rivaroxaban and spontaneous hemothorax following the initiation of treatment for pulmonary embolism.

Spontaneous hemothorax in a 10-year-old boy with COVID-19.

Spontaneous hemothorax occurs in the absence of trauma or iatrogenic causes. Etiologies of spontaneous hemothorax in children include connective tissue disease, neoplasia and coagulopathy, which is associated with thromboembolic events. We present the case of a 10-year-old chronic hemodialysis patient with spontaneous hemothorax with a concurrent COVID-19 infection.

Spontaneous hemothorax and implication of diagnostic evaluation: A case report.

The most critical intervention for large hemothorax is draining the blood out of the pleural cavity by placing a thoracostomy tube but it can be disastrous if done without due consideration. We report a rare case of spontaneous hemothorax due to posterior intercostal artery aneurysm and implication of diagnostic evaluation on its management.

A Case of Bilateral Hemorrhagic Pleural Effusion Due to Dual Antiplatelet Therapy in a Dialysis Patient.

Etiologies of hemorrhagic pleural effusions (hemithoraces) are multifactorial. They can be traumatic, non-traumatic, or idiopathic in nature. In this report, we present a rare case of a 64-year-old male with end-stage renal disease (ESRD) on chronic hemodialysis and dual antiplatelet therapy (DAPT), due to a recent history of coronary arterial stent placement, who presented with progressive shortness of breath for one month. The CT of the chest revealed bilateral large pleural effusions (left > right) with a complete collapse of the left lung and partial collapse of the right lung. Ultrasound-guided left-sided thoracentesis revealed hemorrhagic pleural effusions. After the discontinuation of DAPT, drainage from the right-sided pleural effusion via a pigtail catheter showed continued drainage of pleural fluid without hemorrhage. The effusion on the left side was also noted to have resolved on the repeat chest X-ray. Prompt recognition of this rare cause of any hemorrhagic pleural effusion is essential for patients on dialysis to avoid complications. This report focuses on the possible etiology and potential complications of a hemorrhagic pleural effusion, followed by a brief discussion on the rare but significant association involving the incidence of a hemorrhagic pleural effusion in a dialysis patient receiving DAPT.

Spontaneous, Loculated, and Massive Hemothorax: An Uncommon Complication of Warfarin Therapy.

Warfarin, a commonly used oral anticoagulant, is associated with several adverse drug reactions, principally bleeding. Of all hemorrhagic complications from warfarin therapy, thoracic hemorrhage accounts for only 3% and is usually related to trauma. Cases of spontaneous hemothorax secondary to anticoagulation therapy are rarely reported in the literature.

Spontaneous hemothorax caused by concomitant low-dose rivaroxaban and itraconazole in a 95-year-old patient: case report and literature review.

Although direct-acting oral anticoagulants (DOACs) decrease the bleeding risk compared with vitamin K antagonists (VKAs), DOACs might cause spontaneous hemothorax in very elderly patients, even at a very low dose. Interactions between drugs might increase the risk of bleeding. In this article, we report a case of a 95-year-old man who developed spontaneous hemothorax while taking rivaroxaban 2.5 mg twice daily, 3 days after concomitant use of itraconazole. Rivaroxaban was discontinued, and thoracentesis was performed to drain grossly bloody pleural effusion. To our knowledge, this is the first case report of spontaneous hemothorax that might have been caused by concomitant low-dose rivaroxaban and azole anti-fungal agents. This case highlights the potential risk of spontaneous hemothorax in very elderly patients while taking rivaroxaban and azole anti-fungal agents simultaneously. Special attention should be paid to interactions between drugs that might increase the risk of bleeding. Drugs that have competing metabolic pathways should be avoided. Closer monitoring, including testing for anti-Xa and additional reassessment, should be considered in high-risk patients.

A case of rivaroxaban associated spontaneous hemothorax.

New oral anticoagulants have emerged as an alternative for warfarin for thromboembolic prevention in patients with nonvalvular atrial fibrillation. Although new oral anticoagulants have better compliance and safety margin compared to warfarin, we must be cautious with their usage. In this article, we report a case of spontaneous hemothorax related to rivaroxaban treatment. According to our research, this is the first case of spontaneous hemothorax secondary to rivaroxaban treatment.

Hemoneumotórax espontaneo, una manifestación inusual de la endometriosis pleural / Spontaneous hemopneumothorax, an unusual manifestation of pleural endometriosis

El hemotórax y neumotórax es una condición usualmente relacionada con etiología traumática, infecciosa, inflamatoria o coagulopatía. La aparición espontanea en pacientes jóvenes precisa la búsqueda de etiologías diferenciales. La expresión clínica de la endometriosis pleural incluye neumotórax, hemotórax, hemoptisis y nódulos pulmonares. La radiografía de tórax, la tomografía computarizada, la resonancia magnética nuclear, la broncoscopia y la biopsia se constituyen como el pilar del diagnóstico. Presentamos un caso de un paciente joven con disnea y hemo-neumotórax espontaneo con diagnóstico de endometriosis pleural. (AU)

Hemothorax and pneumothorax is a condition usually related to traumatic, infectious, inflammatory or coagulopathy etiology. The spontaneous appearance in young patients requires the search for differential etiologies. The clinical expression of pleural endometriosis includes pneumothorax, hemothorax, hemoptysis, and pulmonary nodules. Chest X-ray, computed tomography, nuclear magnetic resonance, bronchoscopy and biopsy constitute the mainstay of diagnosis. We present a case of a young patient with dyspnea and spontaneous hemo-pneumothorax diagnosed with pleural endometriosis. (AU)

[Spontaneous hemothorax revealing Wegener's vasculitis in a pregnant woman]. / Hémothorax spontané révélant une vascularite de Wegener chez une femme enceinte.

Spontaneous hemothorax is a rare condition. Its causes are multiple but sometimes they remain unknown. In some patients, thoracotomy may be the only means to determine hemothorax origin. Vasculitis have not been reported as a common cause of spontaneous hemothorax. Pregnancy does not appear to have causal or aggravating effect on spontaneous hemothorax or on vasculitis. We here report the peculiar case of a young patient presenting during pregnancy with spontaneous hemothorax secondary to Wegener's vasculitis. The latter was diagnosed by pleural biopsy performed during exploratory thoracotomy and confirmed by ANCA assays.

Spontaneous hemothorax associated with neurofibromatosis type I: A review of the literature.

Neurofibromatosis is generally a benign disease, but has the potential for rare and fatal complications, such as spontaneous hemothorax. We report a case of massive hemothorax due to neurofibroma in a 49-year-old woman with neurofibromatosis type 1. The configuration of the radiological opacity and frank blood withdrawn on thoracentesis should suggest the diagnosis of hemothorax in a patient with neurofibromatosis. Surgical treatment for hemothorax is limited by arterial fragility and the prognosis is relatively poor. Any evidence of aneurysmal disease in the thoracic vessels should be aggressively managed percutaneously by coil embolization to prevent future rupture.