Cerebral fat embolism with turbid urine as the initial sign.

BACKGROUND: Cerebral fat embolism (CFE) is a rare but potentially fatal complication that can occur after long bone fractures. It represents one subcategory of fat embolisms (FE). Diagnosing CFE can be challenging due to its variable and nonspecific clinical manifestations. We report a case of CFE initially presenting with turbid urine, highlighting an often neglected sign. CASE PRESENTATION: A 69-year-old male was admitted after a traffic accident resulting in bilateral femoral fractures. Sixteen hours post-admission, grossly turbid urine was noted but received no special attention. Four hours later, he developed rapid deterioration of consciousness and respiratory distress. Neurological examination revealed increased upper limb muscle tone and absent voluntary movements of lower limbs. Brain MRI demonstrated a 'starfield pattern' of diffuse punctate lesions, pathognomonic for CFE. Urine microscopy confirmed abundant fat droplets. Supportive treatment and fracture fixation were performed. The patient regained consciousness after 3 months but had residual dysphasia and limb dyskinesia. CONCLUSION: CFE can present with isolated lipiduria preceding overt neurological or respiratory manifestations. Heightened awareness of this subtle sign in high-risk patients is crucial for early diagnosis and intervention. Prompt urine screening and neuroimaging should be considered when gross lipiduria occurs after long bone fractures.

Diagnosis of Bone Marrow Necrosis following Severe Vaso-Occlusive Crisis in Patient with Compound Heterozygous Sickle Cell Disease.

Introduction: Bone marrow necrosis is a rare entity that can develop in context of a sickle cell disease vaso-occlusive crisis. Its physiopathology is related to an endothelial dysfunction taking place in bone marrow microvasculature. Case Presentation: A 30-year-old patient with history of compound heterozygous sickle cell disease was admitted following SARS-CoV-2 infection with fever and diarrhea. After initial favorable evolution, he developed a severe vaso-occlusive crisis with intense hemolysis and multi-organ ischemic complications. Patient then developed high fever and hypoxemia. With the suspicion of acute thoracic syndrome, a red blood cell exchange was performed. Respiratory symptoms ceased but patient persisted febrile with very high levels of acute phase reactants, persistent pancytopenia, and leucoerythroblastic reaction. An infectious cause was ruled out. Afterward, bone marrow aspiration and bone marrow biopsy showed a picture of bone marrow necrosis, which is an extremely rare complication of vaso-occlusive crisis but, paradoxically, more frequent in milder heterozygote cases of sickle cell disease. Ultimately, large deposits of complement membrane attack complex (particles C5b-9) were demonstrated after incubation of laboratory endothelial cells with activated plasma from the patient. Discussion: The clinical presentation and findings are consistent with a case of bone marrow necrosis. In this setting, the demonstration of complement as a potential cause of the endothelial dysfunction mimics the pattern of atypical hemolytic uremic syndrome and other microangiopathic anemias. This dysregulation may be a potential therapeutic target for new complement activation blockers.

Fat embolism syndrome in a patient with a right undisplaced femoral neck fracture.

Fat embolism syndrome (FES) is a rare condition characterised by the classic triad of respiratory distress, neurologic symptoms and petechial rash. Here, we encountered a case of FES in a patient with an asymptomatic right undisplaced femoral neck fracture (Garden Stage II). FES was diagnosed based on the Gurd and Willson's diagnostic criteria and brain magnetic resonance imaging features. To the best of our knowledge, this is the first case of FES in a patient with an undisplaced femoral neck fracture. This study highlights the importance of considering the possibility of FES even in patients with undisplaced femoral neck fractures.

Post-traumatic cerebral fat embolism syndrome with a favourable outcome: a case report.

BACKGROUND: Fat embolism syndrome (FES) is a change in physiology resulting from mechanical causes, trauma, or sepsis. Neurological manifestations of FES can vary from mild cognitive changes to coma and even cerebral oedema and brain death. Here, we present an unusual case of cerebral fat emboli that occurred in the absence of acute chest syndrome or right-to-left shunt. CASE PRESENTATION: A previously healthy 57-year-old right-handed male was admitted to our department because of unconsciousness after a car accident for 3 days. He suffered from multiple fractures of the bilateral lower extremities and pelvis. This patient had severe anaemia and thrombocytopenia. Head MRI showed multiple small lesions in the whole brain consistent with a "star field" pattern, including high signals on T2-weighted (T2w) and fluid-attenuated inversion recovery (FLAIR) images in the bilateral centrum semiovale; both frontal, parietal and occipital lobes; and brainstem, cerebellar hemisphere, and deep and subcortical white matter. Intravenous methylprednisolone, heparin, mannitol, antibiotics and nutritional support were used. Although this patient had severe symptoms at first, the outcome was favourable. CONCLUSIONS: When patients have long bone and pelvic fractures, multiple bone fractures and deteriorated neurological status, cerebral fat embolism (CFE) should be considered. Additionally, CFE may occur without an intracardiac shunt. The early diagnosis and appropriate management of FES are important, and prior to and following surgery, patients should be monitored comprehensively in the intensive care unit. With appropriate treatment, CFE patients may achieve good results.

Complete neurological recovery from fat embolism syndrome in sickle cell disease after sequential red cell exchange transfusion and therapeutic plasma exchange.

Fat embolism syndrome in sickle cell disease is associated with great mortality, while more than half of survivors suffer severe neurological sequelae. Release of fat droplets leads to obstruction of the microcirculation as well as generation of proinflammatory cytokines that can cause direct tissue injury. Red cell exchange transfusion can be life-saving but the addition of therapeutic plasma exchange may further improve outcomes by removing such inflammatory mediators. Here, we describe the case of a 27-year-old male patient with sickle cell anaemia presenting with typical features of fat embolism syndrome including neurological involvement with greatly reduced level of consciousness. MRI of his brain showed multiple widespread microhemorrhages giving the characteristic "star field" pattern but also a cytotoxic lesion of the corpus callosum, known to be the result of direct neurotoxicity by proinflammatory cytokines. The patient underwent emergency red cell exchange transfusion leading only to modest clinical improvement but fully regained consciousness after three cycles of therapeutic plasma exchange. This case highlights the deleterious effect of the hyperinflammatory state characteristic of many sickle cell complications and supports further exploring the potential benefit from plasma exchange as an adjunct to red cell exchange in order to remove proinflammatory cytokines during acute complications of sickle cell disease.

A Case of Fat Embolism Syndrome with Cerebral Involvement in Sickle Cell Anemia.

Fat embolism syndrome (FES) is a rare condition that can occur as a complication of sickle cell disease. We describe a case of a patient with sickle cell disease (homozygous Hb S or HBB: c.20A>T) presenting with initial signs and symptoms consistent with a vaso-occlusive crisis (VOC). Within 24 hours, the patient developed evidence of coagulopathy, multi organ failure and a reduced level of consciousness (LOC) prompting intubation. A diagnosis of FES was made on the basis of the patient's clinical presentation, in conjunction with magnetic resonance imaging (MRI) of the brain revealing innumerable tiny foci of restricted diffusion, intracytoplasmic microvesicular fat on Sudan Red staining of bronchoalveolar lavage samples and evidence of a pulmonary shunt on echocardiogram bubble study. Red blood cell (RBC) exchange transfusion was initiated 3 days following initial presentation and no further exchange transfusions were needed on the basis of subsequent Hb S (HBB: c.20A>T) levels. The LOC gradually improved and the patient was extubated 12 days following presentation. Neurological improvement was slow, with mild cognitive impairment initially evident at 3 months and no cognitive or neurological deficits remaining within 6 months of admission. This case highlights the importance of understanding the pathophysiology and clinical presentation of FES, as early exchange transfusion may improve survival in patients with sickle cell disease and FES.

Ursodeoxycholic acid protects against lung injury induced by fat embolism syndrome.

Acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) is a life-threatening disease with a high mortality rate, which was a common complication of fat embolism syndrome (FES). Ursodeoxycholic acid (UDCA) has been reported to exert potent anti-inflammatory effects under various conditions. In vivo, perinephric fat was injected via tail vein to establish a rat FES model, the anti-inflammatory effects of UDCA on FES-induced lung injury were investigated through histological examination, ELISA, qRT-PCR, Western blot and immunofluorescence. In vitro, human lung microvascular endothelial cells (HPMECs) were employed to understand the protective effects of UDCA. The extent of ALI/ARDS was evaluated and validated by reduced PaO2 /FiO2 ratios, increased lung wet/dry (W/D) ratios and impaired alveolar-capillary barrier, up-regulation of ALI-related proteins in lung tissues (including myeloperoxidase [MPO], vascular cell adhesion molecule 1 [VCAM-1], intercellular cell adhesion molecule-1 [ICAM-1]), elevated protein concentration and increased proinflammatory cytokines levels (TNF-α and IL-1ß) in bronchoalveolar lavage fluid (BALF). Pre-treatment with UDCA remarkably alleviated these pathologic and biochemical changes of FES-induced ALI/ARDS; our data demonstrated that pre-treatment with UDCA attenuated the pathologic and biochemical changes of FES-induced ARDS, which provided a possible preventive therapy for lung injury caused by FES.

The intersection of cerebral fat embolism syndrome and traumatic brain injury: a literature review and case series.

OBJECTIVE: To review the historical, clinical, radiographic, and outcome characteristics of individuals diagnosed with an acquired brain injury (ABI) due to cerebral fat embolism syndrome (CFES) with and without features of traumatic brain injury (TBI). METHODS: A retrospective chart review of individuals with the diagnosis of CFES admitted to an ABI rehabilitation program. Cases were divided into two cohorts 1) individuals with evidence of classic features of CFES alone, and 2) individuals with evidence of CFES in conjunction with features of TBI. RESULTS: 14 individuals were identified, seven individuals with diagnosis of CFES alone, and seven with CFES and TBI. Median initial GCS was 15 for the isolated CFES cohort and 8 for the dual diagnosis cohort (p =.006). There were clear qualitative differences in MRI findings with characteristic patterns between the two groups. CONCLUSION: The diagnosis of CFES is an important consideration for individuals who have new neurologic impairment following a trauma, especially in cases where initial GCS was high. MRI has an important role in differentiating lesions of CFES from TBI and should be utilized for prognostication and management decisions. Individuals with neurologic injury secondary to CFES had good functional recovery outcomes as measured by Glasgow Outcome Scale.

Neurologic Complications of Fat Embolism Syndrome.

PURPOSE OF REVIEW: Fat embolism syndrome (FES) is a rare disorder with potentially devastating neurologic complications. This article reviews the history, pathophysiology, clinical features, diagnosis, and treatment of FES with a focus on its neurologic aspects. RECENT FINDINGS: The neurologic complications of FES are more commonly recognized with current diagnostic testing and increase awareness of the disorder. FES may present initially with neurologic manifestations. Prompt diagnosis of FES and of its neurologic manifestations could be lifesaving. This includes respiratory support and management of neurological complications. The classic clinical triad of pulmonary insufficiency, neurologic disturbances, and petechial skin rash typically presents 24 to 72 h following an initial insult, most commonly a traumatic long bone fracture. Early onset (< 24 h) and delayed onset (> 72 h) have been described. Neurologic manifestations may include ischemic/hemorrhagic strokes, retinal ischemia, seizures, autonomic dysfunction, and diffuse brain injury. Diagnosis remains clinical. Management consists mainly of supportive care.

Integrating Fat Embolism Syndrome Scoring Indices in Sickle Cell Disease: A Practice Management Review.

Fat embolism syndrome (FES) has been described in the literature as a rare complication of sickle cell disease (SCD). A review article published in 2005 reported 24 cases of FES associated with SCD. In many cases, a definitive diagnosis of FES in SCD is made on autopsy because of the lack of early recognition and the paucity of sensitive and specific testing for this syndrome. Patients with FES usually have a fulminant, rapidly deteriorating clinical course with mortality occurring within the first 24 hours. We postulate that FES is not well recognized in SCD and that FES scores are useful diagnostic tools in patients with SCD. We queried the electronic medical records with the diagnostic codes for SCD with acute chest syndrome (ACS), pulmonary embolism, or acute respiratory distress syndrome admitted to our hospital from 2008 to 2016 to identify patients suspected of having FES. In addition, we performed an extensive literature review to evaluate the management practice of pediatric patients with FES and SCD from 1966 to 2016. Six patients met our selection criteria from the hospital records, and 4 case reports from the literature search were also included. We applied the Gurd and Wilson criteria and the Schonfeld Fat Embolism Index to identify patients who met the criteria for FES. Nine patients fulfilled Gurd and Wilson criteria, and 9 patients who were evaluable met the Schonfeld criteria for FES. A rapidly deteriorating clinical course in a patient with SCD presenting with ACS or severe vaso-occlusive crisis should trigger a high index of suspicion for FES. Gurd and Wilson criteria or the Schonfeld Fat Embolism Index are useful diagnostic tools for FES in SCD.

Isolated and early-onset cerebral fat embolism syndrome in a multiply injured patient: a rare case.

BACKGROUND: Fat embolism syndrome (FES) is a rare complication that can occur between 12 and 72 h after the initial insult. Isolated cerebral FES without pulmonary symptoms is rarer. Early fracture fixation might prevent FES. We report a case of multiple-fracture with FES despite definite fixation three hours post-injury. CASE PRESENTATION: A 54-year-old man presented with multiple fractures: left femoral shaft (AO B2), left distal radius (AO C3), left comminuted patella, right comminuted 1st metatarsal base and left 2nd-4th metatarsal neck. Because he was stable, we gave him early total care and definite fixation, which required seven hours and yielded no complications. After he recovered from anesthesia, however, his eyes deviated right, his right upper arm was paralyzed, his consciousness level was poor, and his Glasgow Coma Scale score was E3VeM4. Chest X-rays showed clear lung fields, and brain computed tomography showed no intracranial hemorrhage. He did, however, have tachycardia, anemia, and thrombocytopenia. Brain magnetic resonance images showed a hyperintensive starfield pattern on diffuse weighted images, which suggested cerebral FES. After supportive care, his consciousness cleared on postoperative day 17, and he recovered full right upper arm muscle power after four months; however, he had a significant cognitive deficit. One-year post-injury, after regular rehabilitation therapy, he was able to independently perform his activities of daily living but still had a residual mild cognitive deficit. CONCLUSION: Early fixation can attenuate but not eliminate the incidence of FES. Early assessment and rehabilitation therapy might be required for patients with cerebral FES and cognitive deficits; however, such deficits are difficult to predict and need long-term follow-ups.

Rapamycin alleviated pulmonary injury induced by fat embolism syndrome in rats.

Fat embolism syndrome (FES) is a serious complication after trauma, surgery and fat emulsion input and can lead to serious pulmonary injury. Autophagy controls the cell survival and homeostasis by removing the mis-folded proteins and damaged organelles as well as intracellular pathogens through a lysosomal degradation pathway. Increasing research documented that autophagy was wildly involved in variety of human diseases and had huge therapeutic potential. However, the role and mechanism of autophagy in FES remains largely unknown. The rat model of FES was established by tail vein injection with fat and was assessed by Wet-to-Dry (W/D) ratio analysis, hematoxylin-eosin (HE) analysis, staining Oil red staining analysis and qPCR analysis. Western blots were employed to detect the expression of autophagy markers. The changes of pulmonary injury were observed after premedication of rapamycin (an autophagy activator). The alveolar structural damage, red free fat substances in the blood vessels of lung, increased the lung ratio, and the up-regulated MPO expression and activity were showed in the FES models. The expressions of autophagy markers were decreased and meanwhile, apoptosis markers were increased in the FES model. Rapamycin restored the expression of autophagy markers and inhibited the apoptosis and further, resulting in the improvement of the pulmonary injury. Thus, our study demonstrated that autophagy was inhibited and apoptosis was promoted in FES and further Rapamycin alleviated the pulmonary damage in FES via restoring the autophagy and inhibiting the apoptosis.

Cerebral Fat Embolism: Recognition, Complications, and Prognosis.

Fat embolism syndrome (FES) is a rare syndrome caused by embolization of fat particles into multiple organs including the brain. It typically manifests with petechial rash, deteriorating mental status, and progressive respiratory insufficiency, usually occurring within 24-48 h of trauma with long-bone fractures or an orthopedic surgery. The diagnosis of FES is based on clinical and imaging findings, but requires exclusion of alternative diagnoses. Although there is no specific treatment for FES, prompt recognition is important because it can avoid unnecessary interventions and clarify prognosis. Patients with severe FES can become critically ill, but even comatose patients with respiratory failure may recover favorably. Prophylactic measures, such as early stabilization of fractures and certain intraoperative techniques, may help decrease the incidence and severity of FES.

[To the question of laboratory diagnostics of fat droplets.]

This study aims to compare of study of blood plasma for the presence of fat globules using the method of V.A. Cherkasov and the method what we developed in 60 patients with severe combined trauma. The use of the developed method increases the accuracy of qualitative and quantitative analysis by reducing the emulsifying action of the ethyl alcohol of Sudan IV, preventing uneven coloring and destruction of the preparation within 3 minutes after preparation. A classification of fat globules has been developed, capillary-hazardous globules of ≥ 8 µm in size and arterioles-hazardous globules ≥ 50 µm.

An autopsy case of non-traumatic fat embolism syndrome.

Fat embolism syndrome (FES) occurs after long bone fractures and the symptoms appear 24-72 h after the initial trauma. Fat emboli can affect both the pulmonary and systemic circulation. Apart from the most common type of FES that originates from bone fracture, non-traumatic FES has been also reported. We have experienced an autopsy case of non-traumatic FES. An 81-year-old man with hepatocellular carcinoma associated with alcoholic liver cirrhosis suddenly lost consciousness before transcatheter arterial chemoembolization treatment for his disease and died 5 h after the episode. At autopsy, numerous fat droplets were detected in the alveolar capillaries of the lung and glomerular capillaries of the kidney. Lipid analysis of lung autopsy specimens by thin-layer chromatography showed that the emboli were composed mainly of tristearin. Free fatty acids (FFA) has been considered to be the main component of fat emboli and can be a cause of acute respiratory distress syndrome (ARDS). However, in the present case, the lung specimen contained tristearin and ARDS did not occur. This is the first report of non-traumatic FES in which lipid analysis of human autopsy specimens has been conducted.

Fat Emboli Syndrome in a Child with Duchenne Muscular Dystrophy After Minor Trauma.

BACKGROUND: Fat embolism syndrome is the result of systemic manifestations of fat emboli in the microcirculation. Duchenne muscular dystrophy is a condition that increases the risk of fracture resulting in fat emboli. CASE REPORT: We describe a patient with Duchenne muscular dystrophy who exhibited cardiopulmonary, neurologic, and ophthalmologic sequelae consistent with fat emboli syndrome after minor trauma. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Fat embolism syndrome is a rare but important consideration with significant morbidity and risk of mortality in patients with Duchenne muscular dystrophy after even minor trauma. Early recognition and aggressive resuscitation are crucial to positive clinical outcomes.

The Effects of Aquaporin-1 in Pulmonary Edema Induced by Fat Embolism Syndrome.

This study was designed to investigate the role of aquaporin1 (AQP1) in the pathologic process of pulmonary edema induced by fat embolism syndrome (FES) and the effects of a free fatty acid (FFA) mixture on AQP1 expression in pulmonary microvascular endothelial cells (PMVECs). In vivo, edema was more serious in FES mice compared with the control group. The expression of AQP1 and the wet-to-dry lung weight ratio (W/D) in the FES group were significantly increased compared with the control group. At the same time, inhibition of AQP1 decreased the pathological damage resulting from pulmonary edema. Then we performed a study in vitro to investigate whether AQP1 was induced by FFA release in FES. The mRNA and protein level of AQP1 were increased by FFAs in a dose- and time-dependent manner in PMVECs. In addition, the up-regulation of AQP1 was blocked by the inhibitor of p38 kinase, implicating the p38 MAPK pathway as involved in the FFA-induced AQP1 up-regulation in PMVECs. Our results demonstrate that AQP1 may play important roles in pulmonary edema induced by FES and can be regarded as a new therapy target for treatment of pulmonary edema induced by FES.

Neuroimaging findings in a case of cerebral fat embolism syndrome with delayed recovery.

A young male with multiple lower limb fractures admitted to our Intensive Care Unit was diagnosed with cerebral fat embolism syndrome (FES) based on clinical features and initial magnetic resonance imaging (MRI) which showed multiple hyperintensities on T2-weighted imaging, involving bilateral cerebral and cerebellar hemispheres, predominantly in the watershed territory. The serial MRI done at 3 weeks showed more prominent and larger sized lesions which were in line with the patient's initial low Glasgow Coma Score and indicated severe cerebral insult. The patient responded well to supportive intensive care therapy; his neurological recovery though slow was consistent as he could return to his full functional status after 6 months. The follow-up MRI showed resolution of the most of earlier lesions. This indicates potentially good outcomes even in severe cases of cerebral FES with appropriate medical care.

Fat embolism syndrome: Case report of a clinical conundrum.

Fat embolism syndrome is a rare clinical condition associated with trauma, particularly of long bones. FES after fracture of neck of femur or head of humerus is uncommon. We report a case of FES following fracture in neck of femur and head of humerus in a man with history of mitral valve replacement, on long-term oral anticoagulant therapy, with an alleged history of convulsions. Our dilemma in clinical diagnosis is discussed.