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.

Diverse manifestations of a sickle cell crisis.

We describe the case of a 21-year-old man with a background of sickle cell disease (SCD) who was on acute presentation in a sickle cell crisis required immediate intensive care admission with red blood cell exchange and ventilatory support. He had right frontal lobe infarcts and extensive bilateral deep white matter lesions most likely secondary to fat embolism. Inpatient investigations demonstrated a patent foramen ovale, explaining the route of spread of the fat embolus. He then had a transcatheter closure of the atrial defect. The patient needed prolonged inpatient rehabilitation. He was discharged from hospital in a wheelchair secondary to severe lower limb neurology and bilateral knee heterotopic ossification. He lives with the possibility of early onset dementia and cognitive decline, requiring constant care. The case highlights the multiple manifestations of SCD and their diverse and debilitating consequences.

VEGF mediates fat embolism-induced acute lung injury via VEGF receptor 2 and the MAPK cascade.

Fat embolism (FE) is a lethal medical emergency often caused by fracture of long bones and amputation of limbs. Vascular endothelial growth factor (VEGF) promotes angiogenesis and increases vascular permeability. We tested the hypothesis that VEGF plays a critical role in FE-induced acute respiratory distress syndrome (ARDS) and acute lung injury (ALI). Fat tissues were collected from male Sprague-Dawley rats, and animal oil was extracted and mixed with water to form fatty micelles. The micelles were then injected into the tail vein to produce FE and ALI in rats. Lung weight gain was measured as the index of pulmonary edema. The expression of pulmonary VEGF was evaluated by real-time PCR and western blot analysis. Inducible nitric oxide synthase (iNOS) and phosphorylation of mitogen-activated protein kinase (MAPK) were determined by western blot analyses. Interleukin-1ß (IL-1ß) was quantified by ELISAs. Hematoxylin and eosin staining was used to evaluate the pathological damage of ALI. In this study, we found that animal oil-induced FE significantly increased pulmonary VEGF expression and MAPK phosphorylation. We also evaluated the inflammatory response after FE and found that iNOS and IL-1ß significantly increased after FE. Systemic administration of SU-1498, an antagonist of VEGF receptor 2 (VEGFR-2), significantly attenuated the FE-induced inflammatory response and histological damage. This study suggested that VEGF is involved in FE-induced ARDS via the VEGFR-2 and MAPK cascades, which induce IL-1ß release and iNOS upregulation. Blockade of could be used to treat FE-induced pulmonary damage.

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.

Fat attacks!: a case of fat embolisation syndrome postliposuction.

Liposuction is a procedure commonly performed in the UK usually with a low incidence of serious sequelae; however with larger patients and increased volumes of lipoaspirate, complications have been reported more frequently. One of the rare but very serious complications postliposuction is fat embolism syndrome (FES), a life-threatening condition difficult to diagnose and limited in treatment.The authors present the case of a 45-year-old woman who was admitted to the intensive care unit postelective liposuction for bilateral leg lipoedema. She presented with the triad of respiratory failure, cerebral dysfunction and petechial rash requiring a brief period of organ support. This case highlights that with the recent increase in liposuction procedures worldwide, FES is a differential to always consider. Although still a rare condition this article emphasises the importance of thinking outside the box and how to identify and manage such a life-threatening complication.

Functional end-arterial circulation of the choroid assessed by using fat embolism and electric circuit simulation.

The discrepancy in the choroidal circulation between anatomy and function has remained unsolved for several decades. Postmortem cast studies revealed extensive anastomotic channels, but angiographic studies indicated end-arterial circulation. We carried out experimental fat embolism in cats and electric circuit simulation. Perfusion defects were observed in two categories. In the scatter perfusion defects suggesting an embolism at the terminal arterioles, fluorescein dye filled the non-perfused lobule slowly from the adjacent perfused lobule. In the segmental perfusion defects suggesting occlusion of the posterior ciliary arteries, the hypofluorescent segment became perfused by spontaneous resolution of the embolism without subsequent smaller infarction. The angiographic findings could be simulated with an electric circuit. Although electric currents flowed to the disconnected lobule, the level was very low compared with that of the connected ones. The choroid appeared to be composed of multiple sectors with no anastomosis to other sectors, but to have its own anastomotic arterioles in each sector. Blood flows through the continuous choriocapillaris bed in an end-arterial nature functionally to follow a pressure gradient due to the drainage through the collector venule.

Inflammatory response after nailing.

Intramedullary nailing, as the gold standard stabilisation method of most long bones, has been tailed by its extensive use as the basic tool of investigating the immune response to trauma in many large and small animal models, as well as at the clinical setting. Over the last few decades a complex map of interactions between pro and anti-inflammatory pathways has been the result of these significant global research efforts. Parallel to the evolution of modern nailing and reaming techniques, significant developments at the fields of other disciplines relevant to trauma care, has improved the contemporary management of injured patients, challenging previous concepts and altering clinical barriers. The current article aims to summarise the current understanding of the effect of instrumenting the medullary canal after trauma, and hint on potential future directions.

Timing of definitive fixation of major long bone fractures: Can fat embolism syndrome be prevented?

Fat embolism is common in patients with major fractures, but leads to devastating consequences, named fat embolism syndrome (FES) in some. Despite advances in treatment strategies regarding the timing of definitive fixation of major fractures, FES still occurs in patients. In this overview, current literature is reviewed and optimal treatment strategies for patients with multiple traumatic injuries, including major fractures, are discussed. Considering the multifactorial etiology of FES, including mechanical and biochemical pathways, FES cannot be prevented in all patients. However, screening for symptoms of FES should be standard in the pre-operative work-up of these patients, prior to definitive fixation of major fractures.

[Morphological diagnostics of fat embolism].

The present review of the literature concerns the problem of morphological diagnostics of fat embolism, i.e. mechanical obturation of multiple blood vessels with fat globules, that can be detected by a variety of methods including polarization microscopy, staining of native, frozen, and paraffin-embedded histological sections with the use of immunohistochemical techniques, electron microscopy, etc.

Fat infiltration of left ventricle - a rare cause of sudden cardiac death.

Cor adiposum is a rare disorder of the heart, where the normal heart tissue is replaced by fibro-fatty infiltrates. We report one such case of a middle-aged female who was declared dead shortly after a syncopal episode. At autopsy, the pericardium was intact and firmly adhered to the heart. Histopathology revealed fatty infiltrates extending into the left ventricle of the heart. A post-mortem diagnosis of Cor adiposum was made which is an uncommonly reported cause of sudden cardiac death.

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.

Embolia gordurosa no diagnóstico diferencial de “cor pulmonale” agudo: relato de caso / Fat embolism in differential diagnosis of acute cor pulmonale: case report

Embolia gordurosa (EG) é definida como a presença de partículas de gordura na circulação sanguínea as quais têm potencial para gerar fenômenos embólicos e lesão tecidual local, principalmente em pacientes vítimas de traumas e fraturas dos ossos longos. Síndrome de embolia gordurosa (SEG) caracteriza-se pelo surgimento de sinais e sintomas específicos secundários ao acometimento de órgãos-alvo como pulmões, cérebro e pele, na vigência de EGDiscutiremos o caso de uma paciente feminina jovem com síndrome de embolia gordurosa e “cor pulmonale” agudo 48 horas após acidente de trânsito com fratura do fêmur.

A prospective randomized study to compare systemic emboli using the computer-assisted and conventional techniques of total knee arthroplasty.

BACKGROUND: Conventional total knee arthroplasty is performed with use of an intramedullary alignment guide, which produces elevated intramedullary pressure that can create fat emboli. Total knee arthroplasty performed via computer-assisted surgery does not require an intramedullary femoral rod, raising the question of whether computer-assisted surgery generates less embolic material than conventional total knee arthroplasty. The purpose of this study was to compare the emboli produced in the two techniques. METHODS: Fifty-seven patients were randomized into two groups: the computer-assisted surgery group (n = 29) and the conventional total knee arthroplasty group (n = 28). An intramedullary femoral alignment jig was used in the conventional total knee arthroplasty group but not in the computer-assisted surgery group. Intraoperative invasive monitoring was performed with use of transesophageal echocardiography and a pulmonary artery catheter. RESULTS: The mean embolic score was 6.21 points for the conventional technique group and 5.48 points for the computer-assisted surgery group (p = 0.0161). After tourniquet deflation, fat emboli were observed in the blood of five patients in the conventional surgery group and one patient in the computer-assisted surgery group. CONCLUSIONS: The patients in the computer-assisted surgery group had lower embolic loads compared with the patients in the conventional total knee arthroplasty group. In patients with an uncompromised cardiopulmonary system, the embolic load difference between the techniques was not clinically relevant. LEVEL OF EVIDENCE: Therapeutic Level I. See Instructions for Authors for a complete description of levels of evidence.

Exertional fat embolism after hip joint replacement: a case report.

INTRODUCTION: We present the case of a patient with exertional fat embolism on isolated exercise of his right leg two and four months after right total hip joint replacement. His immediate post-operative period had also been complicated by an acute episode of chest pain and hypotension, treated as acute coronary syndrome. To the best of our knowledge, this is the first reported case of exertional fat embolism following orthopedic surgery. CASE PRESENTATION: A 71-year-old Caucasian man underwent elective cementless total right hip joint replacement. His acute post-operative period was complicated by an episode of chest pain and hypotension. This was treated as acute coronary syndrome. Two months later, a routine stress echocardiography demonstrated a shower of small, echodense bubbles in his right heart, reproduced on exercise of his right leg but not his left. Computed tomography pulmonary angiography excluded pulmonary thromboemboli. A technetium-99m colloid scan confirmed pulmonary fat emboli. Similar findings occurred again four months after the operation but had resolved at six months. CONCLUSIONS: Fat embolism is a well-described phenomenon in the acute setting after long-bone trauma or intramedullary manipulation, and the rare fat embolism syndrome can be fatal. Exertional fat embolism months after joint replacement, however, is an undescribed phenomenon that may have implications in the sub-acute post-operative phase. This may be of particular interest to those involved in orthopedics, cardiology and rehabilitation, but the large volume of patients undergoing joint replacements may broaden the clinical scope of this unusual presentation far beyond these specialties.

[Dyspnea, hypoxia and a confusional state--a post-recovery diagnosis].

We present a case of a 48 year old female, with a medical history significant for paraplegia due to a cervical cord injury and a history of transitional cell carcinoma, which necessitated a urinary bladder ileal reconstruction. The patient was hospitalized due to an acute confusional state, severe respiratory distress and hemolytic anemia. One day prior to hospitalization, she was involved in a minor motor vehicle accident, yet she and her family denied any injuries due to the accident and the patient did not complain of pain. During the course of her hospitalization the patient was febrile, yet we did not find any evidence of an infectious cause for her symptoms. Notably, lumbar puncture and MRI scan were relatively contraindicated, hence a CNS infection was not completely ruled out. We also thoroughly investigated her respiratory symptoms, but could not reach a conclusive diagnosis. Nevertheless, after approximately 14 days of diagnostic efforts, empirical antibiotic treatment and supportive care, all clinical and laboratory abnormalities had resolved. The patient was discharged with a presumed diagnosis of a poorly understood infectious process. However, not long after, she returned to the emergency department complaining of a red, painful, swollen right knee. Imaging studies demonstrated a right supracondylar as well as a tibial plateau fracture. Consequently, a post-recovery diagnosis of fat emboli syndrome was made.

Massive cerebral involvement in fat embolism syndrome and intracranial pressure management.

Fat embolism syndrome (FES) is a common clinical entity that can occasionally have significant neurological sequelae. The authors report a case of cerebral fat embolism and FES that required surgical management of intracranial pressure (ICP). They also discuss the literature as well as the potential need for neurosurgical management of this disease entity in select patients. A 58-year-old woman presented with a seizure episode and altered mental status after suffering a right femur fracture. Head CT studies demonstrated hypointense areas consistent with fat globules at the gray-white matter junction predominantly in the right hemisphere. This CT finding is unique in the literature, as other reports have not included imaging performed early enough to capture this finding. Brain MR images obtained 3 days later revealed T2-hyperintense areas with restricted diffusion within the same hemisphere, along with midline shift and subfalcine herniation. These findings steered the patient to the operating room for decompressive hemicraniectomy. A review of the literature from 1980 to 2012 disclosed 54 cases in 38 reports concerning cerebral fat embolism and FES. Analysis of all the cases revealed that 98% of the patients presented with mental status changes, whereas only 22% had focal signs and/or seizures. A good outcome was seen in 57.6% of patients with coma and/or abnormal posturing on presentation and in 90.5% of patients presenting with mild mental status changes, focal deficits, or seizure. In the majority of cases ICP was managed conservatively with no surgical intervention. One case featured the use of an ICP monitor, while none featured the use of hemicraniectomy.

Fracture complications.

This article highlights 2 important complications of fracture: acute compartment syndrome and fat embolism syndrome (FES). FES is most commonly associated with long-bone and pelvic fracture, whereas acute compartment syndrome is often associated with tibia or forearm fracture. The onset of both of these complications may be difficult to assess in the nonverbal patient or in the patient with multiple trauma. Careful, serial assessment of the patient with fracture is necessary to recognize and treat these complications promptly. Early treatment and supportive care are crucial to positive outcomes for patients with complications of fracture.