Decompressive Hemicraniectomy After Cerebral Fat Embolism.

Fat embolism syndrome is a common occurrence after orthopedic trauma and surgery. Cerebral fat embolism (CFE) may arise after fat globules enter the arterial circulation. The neurological manifestations of CFE vary and generally carries a favorable outcome. A small number of reports exist regarding patients with CFE who experienced severe neurological deficits and significant edema on radiographic studies, and subsequently underwent decompressive hemicraniectomy (DHC), some of which had full neurological recoveries. Here, we present the case of a 21-year-old male who presented after a motorcycle accident with multiple orthopedic injuries, who after fixation did not awake from anesthesia. The patient was ultimately found to have cerebral fat emboli, and developed significant edema and swelling. The patient underwent DHC with subsequent cranioplasty and returned to his neurological baseline seven months after his initial injury. DHC for CFE has been described in a few cases with some patients have had substantive recoveries, including the present case. This case emphasizes the importance of promptly recognizing and reversing elevated intracranial pressures and the possibility of promising recoveries.

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.

Fat embolism syndrome in Duchenne muscular dystrophy: Report on a novel case and systematic literature review.

We report a non-ambulatory 13-year-old boy with Duchenne muscular dystrophy who experienced severe acute respiratory distress syndrome and cerebral fat embolism following elective soft tissue surgery. Post-surgery radiological examination revealed bilateral femoral fractures and marked osteopenia that were believed to have caused disseminated pulmonary and cerebral fat embolism. The patient had never been on glucocorticoid treatment. Five months post-surgery, he remained in a state of minimal consciousness. A literature review was performed and eleven publications included, providing case reports of a total number of 23 patients with Duchenne muscular dystrophy with fat embolism syndrome. The most common causes were falls from the wheelchair that predominantly resulted in femoral fractures. Median age at the event was around 14 years. Seven patients succumbed to complications of fat embolism. No event was described in the context of surgery. We want to raise awareness that spontaneous unnoticed fractures may occur especially in adolescents with DMD from traumatic injury of large bones and also during elective surgery with a high risk of causing fat embolism with severe sequelae.

Occurrence of Numerous Cerebral White Matter Hyperintensities in Trauma Patients With Cerebral Fat Embolism: A Systematic Review and Report of Two Cases.

There has been little effort to identify an overall occurrence of numerous cerebral white matter hyperintensities (NCWMH) on relevant brain magnetic resonance imaging (MRI) sequences in postinjury cerebral fat embolism syndrome (CFES) patients. Also, quantification of pre-CFES cognitive status, degree of neurologic deterioration, and presence of a skeletal fracture with CFES is nominal. The authors performed a PubMed search and identified 24 relevant manuscripts. Two case reports from the authors' institution were also used. The presence of NCWMH was assessed by reviewing T2-weighted image (T2WI), diffusion-weighted image (DWI), fluid-attenuated inversion recovery (FLAIR) figures and captions, and by evaluating manuscript descriptions. When pre-CFES cognitive status was described, it was categorized as Glasgow Coma Scale (GCS) score = 14-15 (yes or no). When the degree of neurologic deterioration was noted with CFES, it was classified as coma or GCS ≤ 8 (yes or no). When skeletal fractures were itemized, they were categorized as yes or no. The total number of CFES patients was 133 (literature search was 131 and two author-described case reports). Of the 131 patients with manuscript MRI figures or descriptive statements, 120 (91. 6%) had NCWMH. Of 63 patients with a delineation of the MRI sequence, NCWMH appeared on DWI in 24, on T2WI in 57, and on FLAIR in 10 patients. Pre-CFES cognitive status was GCS 14-15 in 93.5% (58/62) of the patients. The CFES neurologic deterioration was coma or GCS ≤ 8 in 52.5% (62/118) of the patients. A skeletal fracture was present in 99.0% (101/102) of the CFES patients. The presence of NCWMH in trauma patients with hospital-acquired neurologic deterioration and the presence of a skeletal fracture is consistent with CFES.

A Rare Case of Progressive Encephalopathy in a Sickle Cell Trait Patient: A Case Report.

Fat embolism syndrome (FES) is one of the underdiagnosed and underrecognized complications that can happen in multiple medical and surgical conditions. FES can manifest in a broad spectrum of signs and symptoms and affect multiple organ systems in the human body. One of the most commonly involved is the central nervous system (CNS), mainly the brain, which can be involved in different ways, and the presenting symptoms can vary in type and severity. One of the most common causes of FES is trauma, mainly a long bone fracture or any orthopedic injury. However, one of the rare causes of FES is sickle cell disease (SCD) and thalassemia. Generalized and vague presenting symptoms, the rarity of FES, and the absence of well-defined diagnostic criteria make it a challenging diagnosis for healthcare practitioners. FES diagnosis is usually made after having a high index of suspicion in patients with underlying risk factors that can precipitate and contribute to the pathophysiology of FES. Moreover, the diagnosis is usually reached after excluding other more common and treatable conditions.

Do you know the diagnostic importance of susceptibility-weighted imaging on MRI for patients with cerebral fat embolism?

Key Clinical Message: If you suspect cerebral fat embolism (CFE) of the diagnosis for the patients who present with impaired consciousness and hypoxia following a fracture, susceptibility-weighted imaging should be included of head MRI imaging. Abstract: We report a case of cerebral fat embolism (CFE) that could be identified only by susceptibility-weighted imaging (SWI). Collection and analysis of previous case reports of CFE revealed utilization of SWI in less than one third of suspected cases, despite its known diagnostic ability for CFE.

Stars from the bones-An uncommon presentation of fat embolism syndrome.

Trauma or surgery to the lower limbs can cause fat from the marrow within the leg bones to enter the bloodstream and form an embolus. However, if there is cerebral involvement without any pulmonary or dermatological manifestations at diagnosis, it could delay identifying cerebral fat embolism (CFE).

Cerebral Fat Embolism Syndrome in a Patient With an Aortic Dissection and Orthopedic Injuries: A Case Report.

Traumatic brain injury is a significant cause of morbidity and mortality in adults and can be associated with severe secondary complications, including post-traumatic cerebral infarction. One potential cause of post-traumatic cerebral infarction is cerebral fat embolism syndrome (FES). We present a case in which a male in his twenties was involved in a motorcycle collision with a truck. He sustained numerous injuries, including bilateral femur fractures, left acetabular, open left tibial and fibular fractures, and a type A aortic dissection. Before orthopedic fixation, his Glasgow Coma Score (GCS) was 10. Following open reduction and internal fixation, his GCS was noted to be 4, with a stable computed tomography scan of the head. The differential included embolic strokes related to his dissection, an unrecognized cervical spine injury, and cerebral FES. Stat magnetic resonance imaging of the head demonstrated a starfield pattern of restricted diffusion consistent with cerebral FES. An intracranial pressure (ICP) monitor was placed, and his ICP acutely spiked to over 100 mmHg despite maximal medical management. This case highlights several key learning points, namely, that cerebral FES should remain in the mind of any physician treating high-energy multisystem traumas. While it is a rare syndrome, its effects can lead to significant morbidity and mortality as treatment is controversial and can conflict with the treatment of other systemic injuries. Further research into prevention and treatment is warranted to continue optimizing outcomes following cerebral FES.

Cerebral fat embolism syndrome in a patient with homozygous sickle cell disease in the setting of multisystem inflammatory syndrome in children.

Multisystem inflammatory syndrome in children (MIS-C) is a rare progressive inflammatory process temporally associated with exposure to SARS-CoV-2 (COVID-19) in patients 20 years of age and younger. At this time, much of MIS-C is not well understood, including the pathogenesis, long-term implications, and how each variant of the COVID-19 virus affects the progression and severity. We present the unusual case of a 19-year-old man with a history of homozygous sickle cell disease who developed a vaso-occlusive pain crisis and cerebral fat embolism syndrome as a complication of MIS-C secondary to the Omicron variant of COVID-19.

Prospective study examining the probability of cerebral fat embolism based on magnetic resonance imaging.

Purpose: Cerebral fat embolism (CFE) is a rare syndrome caused by the embolization of fat particles into the brain circulation. This prospective single-center observational study investigated the incidence of CFE in long-bone or pelvic fractures based on magnetic resonance imaging (MRI) findings. The purpose of this study was to investigate the incidence of CFE by MRI findings with or without symptoms suggestive of CFE. Methods: Eligible patients were consecutive, aged 15 years or older, with high-energy traumas, including pelvic or femur fractures. Excluded patients were those who died, could not undergo MRI resulting from medical conditions, or had insufficient mental capacity and no consultee to provide consent. The MRI was scheduled within 4 weeks of the injury, and the images were reviewed by one of the three neuroradiologists who were unaware of the patient's clinical information. Patient data regarding demographics, preceding trauma, injury severity score (ISS), presentation and examination timing of MRI, management including surgery, and outcome were collected. Results: Sixty-two patients were recruited, and three patients were excluded. All patients were injured by blunt trauma. The median patient age was 44 years. The median ISS was 13, and 53 patients needed surgical fixation. There were 22 patients with long-bone fractures, all of whom received external fixation or intramedullary nailing on admission day. MRI was performed after a median hospital day of 18 days. Using MRI imaging, three (5.0%) patients were diagnosed with CFE, and three patients were suspected of CFE. Conclusions: This is the first study to prospectively examine the probability of CFE based on MRI. Since fat embolism syndrome (FES) is confirmed in patients without clinical symptoms, CFE may be more common in patients with trauma than currently believed. Therefore, studies to determine the diagnostic criteria combined with symptoms, MRI, or other objective findings are required in the future.

Fat emboli and critical illness-associated cerebral microbleeds (CICMs) in a patient with sickle cell disease: Do these 2 entities coexist?

We report the case of a 30-year-old female patient with sickle cell disease presenting with an acute chest syndrome and neurological deterioration. Cerebral magnetic resonance imaging revealed a handful of foci of diffusion restriction and numerous microbleeds with marked involvement of corpus callosum and subcortical white matter, with relative sparing of the cortex and deep white matter. Corpus callosum-predominant and juxtacortical microbleeds have been typically documented in cerebral fat embolism syndrome, but also in the so-called "critical-illness-associated cerebral microbleeds", a recently described entity associated with respiratory failure. We discussed whether these 2 entities may coexist.

Fat embolism in sickle-cell disease: A case report with literature review.

Background: Sickle cell disease (SCD) is associated with an under-recognized and well-known complication of bone marrow necrosis known as cerebral fat embolism (CFE). Case Presentation: We report a case of successfully treated SCD patient suffering from non-traumatic cerebral fat embolism (NCFE) who came with initial manifestation of localized pain followed by loss of consciousness and absence of cardiac shunt. This was an unusual case of fat embolism in SCD patient with the absence of right-to-left cardiac shunt indicating the biochemical etiology. Patient initially showed localized pain followed by loss of consciousness, suggesting that any such initial manifestation in patient of SCD should be suspected for CFE. MRI served as an accurate diagnostic tool showing the characteristic neuroradiologic sign. Treatment with exchange-transfusion recovered the patient successfully. Conclusion: Overall, this case suggested that prompt diagnosis using MRI and treatment with RBC exchange could lead to better outcomes in SCD patients suffering from CFE.

Isolated Cerebral Fat Embolism Syndrome in a Polytrauma Patient with Complete Recovery - A Case Report.

Introduction: Isolated cerebral fat embolism syndrome (FES) is a rare complication that occurs within the first 3 days of the initial insult. We report a case of multiple long bone fractures with isolated cerebral FES, despite undergoing early total care with definitive fixation. Case Presentation: A 22-year-old female presented with type IIIA open femur shaft fracture on the right side (AO 32B2), closed femur shaft fracture (AO 32B2), comminuted patella fracture on the left side (AO 34C3), and undisplaced mandible fracture. She had a normal sensorium with a Glasgow Coma Scale (GCS) of E4V5M6. A whole body computed tomography (CT) scan was done to rule out other injuries. All initial scans were normal. After about 6 h in the ICU, she was noticed to have disconjugate gaze and was answering in monosyllables. A repeat CT scan of the brain was normal. The early total care and definitive fixation with titanium intramedullary nails for femur fractures and tension band wiring for patella was done under general anesthesia. On 1st post-operative day (POD), her GCS dropped to E1VTM1. On the 3rd POD, she developed decerebrate rigidity and generalized tonic clonic seizures. Fundoscopic examination showed multiple fat globules along the vessel in the entire field of both eyes. Since there were no other signs of FES in the lungs or on the skin, an MRI brain was done which revealed a hyperintensive starfield pattern on diffusion-weighted images, suggestive of cerebral fat embolism (CFE). At 4 weeks, her upper limb and lower limb muscle power improved. By 2 months, she was mobilized with support. Her Mini-Mental State Examination showed no cognitive impairment. At the latest follow-up at 1 year, her fractures are completely healed and she has no neurological or functional impairment. Conclusion: We must always suspect isolated cerebral FES as a diagnosis in polytrauma patients even when the classical findings are not present. MRI compatible implants have to be used as far as possible as MRI may be required to confirm the diagnosis of CFE. The early total care with definitive fixation and supportive treatment helped us in this patient's complete recovery without cognitive impairment.

Cerebral Fat Embolism After a Fall.

BACKGROUND: Cerebral fat embolism is a rare diagnosis that can occur after significant long bone trauma. Most patients have evidence of pulmonary involvement, but this case involved a patient with a pure neurologic manifestation of a fat embolism. CASE REPORT: An 89-year-old woman presented to the emergency department as a transfer from an outside hospital with a diagnosis of air embolism after an episode of altered mental status and expressive aphasia. A secondary review of the patient's computed tomography angiography head imaging uncovered a cerebral fat embolism as the cause of the patient's acute neurologic event. The cerebral fat embolism was likely from a remote sacral fracture 6 weeks prior. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: When a patient presents with a concern for a stroke-like symptoms and a cerebral fat embolism is diagnosed, a thorough examination of the patient must be performed to identify the primary fracture site. Geriatric long bone fractures have well-known significant morbidity and mortality. An associated cerebral fat embolism can increase that mortality and morbidity and prompt diagnosis is important.

Cerebral fat embolism syndrome at a single trauma center.

OBJECTIVES: Based on a 16-year case series, we sought lessons about diagnosis and treatment of cerebral fat embolism syndrome. MATERIALS AND METHODS: Using discharge codes at a Level 1 Trauma Center, we performed a retrospective chart review of clinical characteristics, diagnostic studies, treatments, and outcome in cerebral fat embolism syndrome. RESULTS: Thirty-nine (40%) of 97 patients with fat embolism syndrome were diagnosed with cerebral fat embolism syndrome, with 29 (74%) presenting with coma. All had abnormal brain magnetic resonance imaging, with scattered cytotoxic edema (starfield pattern) in 29 (74%). All but two of the 21 patients with dilated fundoscopy showed retinal embolism. Among 29 patients with transcranial Doppler, the presence of microembolic signals in 15 (52%) was associated with fever (p = 0.039), right-to-left intracardiac shunting (p = 0.046) and a trend towards initial coma. In 11 patients with serial transcranial Dopplers and treatment with high-intensity statin therapy, the frequency of microembolic signals tended to decrease after therapy was initiated. Of the 28 (72%) of the 39 patients discharged, 16 (57%) had mild to moderate disability at last follow up. CONCLUSIONS: The recognition of cerebral fat embolism syndrome may be improved with routine inclusion of brain magnetic resonance imaging, dilated fundoscopy, and transcranial Doppler. We share our empiric management algorithm for cerebral fat embolism syndrome using these studies and with consideration of experimental therapies in select patients to prevent ongoing cerebral injury.

Central Pontine Myelinolysis: A Case Report of Persistent Hyperglycemia With Normal Serum Sodium.

Rapid correction of hyponatremia is the most frequent predisposing factor for the development of central pontine myelinolysis (CPM). Alcoholism, cirrhosis, malnutrition, and severe burns are associated conditions that often present in combination with a rapid rise in serum sodium concentration. However, its association with hyperglycemia has not been as well established. There have been recent reports of acute to subacute presentation of CPM with hyperglycemia. We report an unusual case of a 48-year-old Caucasian male who presented with pseudobulbar palsy, ataxia, and quadriplegia with worsening pontine hyperintensities and was diagnosed with CPM in the setting of persistent hyperglycemia with normal serum sodium.

Stroke in sickle cell disease and the promise of recent disease modifying agents.

Sickle cell disease (SCD) is an inherited hemoglobinopathy affecting approximately 100,000 individuals in the United States. Cerebrovascular disease is among the most common and debilitating complications of SCA, with 53% experiencing silent cerebral infarct by age 30 and 3.8% experiencing overt stroke by age 40 years. This review highlights the burden of cerebrovascular disease in SCD, including both stroke and silent cerebral infarct (SCI). We then discuss the pathophysiology of stroke and cerebral fat embolism in the absence of a patent foramen ovale. This review also reveals that options for primary and secondary stroke prevention in SCD are still limited to hydroxyurea and blood transfusion, and that the role of aspirin and anticoagulation in SCD stroke has not been adequately studied. Limited data suggest that the novel disease-modifying agents for SCD management may improve renal dysfunction, leg ulcers, and lower the abnormally high TCD flow velocity. Further research is urgently needed to investigate their role in stroke prevention in SCD, as these novel agents target the main stroke contributors in SCD - hemolysis and vaso-occlusion. This literature review also explores the role of healthcare disparities in slowing progress in SCD management and research in the United States, highlighting the need for more investment in patient and clinician education, SCD management, and research.

Cerebral Fat Embolism: A Rare East African Conundrum.

Cerebral fat embolism (CFE) is a potentially fatal condition associated with displaced long bone fracture of the lower extremities. CFE, usually seen in young men, has an incidence ranging between 0.9% and 11% in patients with long bone fractures. CFE can present with various neurological symptoms, and a diffusion-weighted magnetic resonance imaging (MRI) (DWI) remains the definitive diagnostic study. Early treatment of the fracture is crucial in the management of CFE. To the best of our knowledge, we are the first to report a case of CFE in East Africa.

A Rare Case of Cerebral Fat Embolism With No Respiratory or Dermatologic Involvement.

Fat embolism syndrome is potentially lethal. It is frequently a complication of long bone fractures and/or orthopedic surgery. Cerebral fat embolism is an unusual condition characterized by purely cerebral involvement. Neurological signs can be variable and brain MRI has a pivotal role in the diagnosis. We report the case of a 69-year-old male who presented motor impairment and a disorder of consciousness in the early postoperative course of total hip arthroplasty for a left femoral neck fracture, which occurred 24 hours before surgery. He had no dermatologic or respiratory signs. No respiratory or dermatologic signs were found. Blood samples showed moderate thrombopenia and hemolytic anemia. Multiple lesions were found on brain MRI. Diagnosis of cerebral fat embolism was established after ruling out differentials.