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1.
Methodist Debakey Cardiovasc J ; 20(1): 70-73, 2024.
Article in English | MEDLINE | ID: mdl-39220350

ABSTRACT

Fat emboli may occur in patients after traumatic fractures or orthopedic procedures; however, their clinical detection is a very rare finding. Here, we describe a 77-year-old female who was admitted to the emergency department with a fracture of the right humerus. We diagnosed fat embolism after an ultrasound of the right subclavian vein. The embolism was detected by high-intensity transient signals present on the spectral Doppler. While these signals are well known for microembolization in transcranial Doppler, to our knowledge this is the first case report in the medical literature to observe and describe high-intensity transient signals seen in the upper extremities by spectral Doppler. Although it is unusual to detect a fat embolism in transit, we believe clinicians should be aware of this finding, particularly when evaluating high-risk patients.


Subject(s)
Embolism, Fat , Predictive Value of Tests , Subclavian Vein , Humans , Female , Aged , Embolism, Fat/diagnostic imaging , Embolism, Fat/etiology , Embolism, Fat/therapy , Subclavian Vein/diagnostic imaging , Humeral Fractures/diagnostic imaging , Humeral Fractures/complications , Humeral Fractures/therapy
2.
Ann Med Surg (Lond) ; 86(8): 4767-4771, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39118753

ABSTRACT

Introduction and importance: Fat embolism syndrome (FES) arises from the systemic effects of fat emboli in microcirculation. While sepsis is characterized by pathological, physiological, and metabolic abnormalities caused by infection. Septic shock is identified by elevated blood lactate (>2 mmol/l) and the need for vasopressors to maintain a mean arterial pressure of 65 mmHg or higher in the absence of hypovolemia. Case presentation: This case report discusses the clinical course and treatment of a 50-year-old male involved in a road traffic accident resulting in polytrauma. The patient presented with multiple fractures, hemopneumothorax, lung contusions, and rib fractures. He was then stabilized following which fractures were reduced and managed operatively. Postoperatively, the patient developed FES with septic shock, manifested by altered consciousness, petechial rashes, and respiratory distress. He was managed with intubation, chest drainage, and a combination of antibiotics, anticoagulants, and vasoactive agents. A tracheostomy was performed due to respiratory insufficiency. Following 29 days in the SICU, the patient's condition was stabilized and shifted to the general ward for further management. He was discharged after 48 days, with a complete recovery and a 2-week follow-up. This case report depicts the challenges in the management of FES with septic shock following polytrauma. Conclusion: This case report is a comprehensive overview of FES complicated with septic shock. It highlights the importance of supportive care as the primary treatment modality, incorporating various medical interventions. The successful outcome and complete recovery of the patient underline the significance of prolonged monitoring, wound care, and physiotherapy.

3.
Rev Med Liege ; 79(7-8): 507-510, 2024 Jul.
Article in French | MEDLINE | ID: mdl-39129549

ABSTRACT

Fat embolism syndrome mainly occurs following trauma to the long bones or pelvis. Non-traumatic causes are rarer. Its incidence varies greatly and depends on the number of fractures involved. Two physiopathological theories, one mechanical and the other biochemical, attempt to explain this still poorly understood phenomenon. The complete form of the syndrome results in a combination of pulmonary involvement, neurological disorders and a petechial rash. Given the polymorphism of signs and symptoms, Fat embolism syndrome remains a diagnosis of exclusion. Regarding treatment, the therapeutic strategy combines treatment of the causative process with conservative measures.


Le syndrome d'embolie graisseuse se manifeste majoritairement dans les suites d'un traumatisme des os longs ou du bassin. Les causes non traumatiques sont plus rares. Son incidence varie fortement et dépend du nombre de fractures impliquées. Deux théories physiopathologiques, l'une mécanique et l'autre biochimique, tentent d'expliquer ce phénomène encore mal compris. La forme complète du syndrome se traduit par la combinaison d'une atteinte pulmonaire, de troubles neurologiques et d'une éruption pétéchiale. Étant donné le polymorphisme des signes et des symptômes, le syndrome d'embolie graisseuse reste un diagnostic d'exclusion. Concernant le traitement, la stratégie thérapeutique associe la prise en charge du processus causal combinée à des mesures conservatrices.


Subject(s)
Embolism, Fat , Humans , Embolism, Fat/diagnosis , Embolism, Fat/etiology , Embolism, Fat/therapy , Stroke/etiology , Stroke/diagnosis , Male
4.
Cureus ; 16(7): e64819, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39156416

ABSTRACT

Cerebral fat embolism syndrome (CFES) is a rare but certainly devastating impediment following long bone fractures. The diagnosis of CFES primarily depends on identifying clinical manifestations like respiratory distress, petechial rash, and neurological symptoms. However, in rare instances, CFES can manifest with atypical or absent clinical features, posing diagnostic challenges. Here, we present a rare case report of a woman in her 20s who developed CFES after suffering a femur shaft fracture devoid of conventional clinical features. The diagnosis of CFES was built upon clinical suspicion and a typical MRI brain finding of a starfield pattern. Our case highlights the importance of including CFES in the differential diagnosis of neurological deterioration, especially after long bone fractures. We suggest early plate osteosynthesis to stop more emboli from forming in people with FES, as well as continuous neuromonitoring and a reminder that CFES can show up without any other signs or symptoms in the body.

5.
Front Immunol ; 15: 1396800, 2024.
Article in English | MEDLINE | ID: mdl-39100680

ABSTRACT

Introduction: Bone marrow embolization may complicate orthopedic surgery, potentially causing fat embolism syndrome. The inflammatory potential of bone marrow emboli is unclear. We aimed to investigate the inflammatory response to femoral intramedullary nailing, specifically the systemic inflammatory effects in plasma, and local tissue responses. Additionally, the plasma response was compared to that following intravenous injection of autologous bone marrow. Methods: Twelve pigs underwent femoral nailing (previously shown to have fat emboli in lung and heart), four received intravenous bone marrow, and four served as sham controls. Blood samples were collected hourly and tissue samples postmortem. Additionally, we incubated bone marrow and blood, separately and in combination, from six pigs in vitro. Complement activation was detected by C3a and the terminal C5b-9 complement complex (TCC), and the cytokines TNF, IL-1ß, IL-6 and IL-10 as well as the thrombin-antithrombin complexes (TAT) were all measured using enzyme-immunoassays. Results: After nailing, plasma IL-6 rose 21-fold, compared to a 4-fold rise in sham (p=0.0004). No plasma differences in the rest of the inflammatory markers were noted across groups. However, nailing yielded 2-3-times higher C3a, TCC, TNF, IL-1ß and IL-10 in lung tissue compared to sham (p<0.0001-0.03). Similarly, heart tissue exhibited 2-times higher TCC and IL-1ß compared to sham (p<0.0001-0.03). Intravenous bone marrow yielded 8-times higher TAT than sham at 30 minutes (p<0.0001). In vitro, incubation of bone marrow for four hours resulted in 95-times higher IL-6 compared to whole blood (p=0.03). Discussion: A selective increase in plasma IL-6 was observed following femoral nailing, whereas lung and heart tissues revealed a broad local inflammatory response not reflected systemically. In vitro experiments may imply bone marrow to be the primary IL-6 source.


Subject(s)
Embolism, Fat , Interleukin-6 , Lung , Animals , Swine , Interleukin-6/blood , Embolism, Fat/etiology , Embolism, Fat/blood , Embolism, Fat/immunology , Lung/immunology , Lung/pathology , Lung/metabolism , Bone Marrow/metabolism , Fracture Fixation, Intramedullary/adverse effects , Myocardium/metabolism , Myocardium/pathology , Myocardium/immunology , Inflammation/blood , Inflammation/immunology , Female , Cytokines/blood , Cytokines/metabolism , Bone Nails , Complement Activation , Femur/metabolism , Disease Models, Animal
6.
Cureus ; 16(7): e64376, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39130986

ABSTRACT

Gluteal augmentation surgery, commonly known as the Brazilian Butt Lift (BBL), has become increasingly popular and is offered at numerous surgical centers. Typically performed on an outpatient basis, the procedure takes less than four hours, making it an appealing option for many patients. However, BBL is associated with multiple complications, some of which can be severe, resulting in high mortality rates. Most such post-operative adverse events necessitate urgent transfer to hospitals for optimal care, with post-operative respiratory distress being one such critical sign. Fat embolism syndrome (FES) is a notable complication of BBL. The diagnosis of FES is primarily clinical, supported by imaging studies such as chest X-rays and CT scans. FES often goes underdiagnosed due to the lack of definitive diagnostic criteria and its clinical and radiological similarities to other conditions. Despite its underdiagnosis, FES is reported in approximately 0.06% of patients undergoing BBL. Failure to diagnose it early can lead to complications from empiric treatment of other suspected conditions, potentially worsening the prognosis. Our patient developed respiratory failure within an hour after undergoing BBL. The time to symptom onset and the patient's agitation before the respiratory episode broadened the differential for her condition. This case report highlights the importance of recognizing FES and exploring potential preventive measures, including advancements in surgical techniques and prophylactic strategies.

7.
Brain Inj ; 38(11): 938-940, 2024 Sep 18.
Article in English | MEDLINE | ID: mdl-38722041

ABSTRACT

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.


Subject(s)
Embolism, Fat , Intracranial Embolism , Humans , Male , Embolism, Fat/diagnosis , Embolism, Fat/etiology , Aged , Intracranial Embolism/diagnosis , Intracranial Embolism/etiology , Femoral Fractures/diagnosis , Magnetic Resonance Imaging , Accidents, Traffic
8.
Trauma Case Rep ; 52: 101040, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38784218

ABSTRACT

A 28-year-old man involved in a serious motorcycle accident was admitted to our hospital with comminuted fractures of the ipsilateral femoral shaft and tibial shaft, as well as multiple fractures of the right lower limb, including the proximal fibula, medial malleolus, and the third and fourth distal metatarsals. In addition, the patient suffered a skin contusion and laceration of the right foot. On the first day of admission, this patient suddenly developed tachycardia, pyrexia, and tachypnoea, and was immediately transferred to the ICU for further treatment due to a CT-diagnosed pulmonary fat embolism (FE). As a symptomatic treatment, he received a prophylactic dose of low-molecular-weight heparin for 10 days, after which his condition improved. A Doppler ultrasound of the lower leg and a follow-up chest CT angiography were performed, which excluded any remaining thrombus and verified that the pulmonary FE had improved without deterioration. Closed-reduction and retrograde intramedullary nailing were performed for the femoral shaft fractures, while antegrade intramedullary nailing was performed for the tibial shaft fractures under general anaesthesia. In the three-year follow-up, the patient had recovered with good function of the right limb, without any respiratory discomfort. Both the femoral and tibial shaft fractures finally resolved without any further treatment. Ipsilateral femoral and tibial shaft fractures should undergo surgical stabilisation as early as possible to avoid pulmonary FEs. It is still controversial whether intramedullary nailing is suitable for floating knee injuries complicated by pulmonary FEs. However, if patients with pulmonary FEs require intramedullary nailing, we suggest that surgery should be performed after at least one week of anticoagulant use, when patient vital signs are stable and there is no sign of dyspnoea. In addition, patients should try to avoid reaming during the operation to prevent and decrease "second hit" for the lung.

9.
Clin Case Rep ; 12(4): e8681, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38560285

ABSTRACT

Diagnosing FES is difficult and time-consuming, and identify FES as an etiology of right ventricular volume overload for early diagnosis. Because FES is a reversible condition, even severe cases can bse treated if the patient survives the acute phase.

10.
J Spine Surg ; 10(1): 144-151, 2024 Mar 20.
Article in English | MEDLINE | ID: mdl-38567009

ABSTRACT

Background: For patients undergoing long-construct fusion surgeries, simultaneous sacroiliac joint (SIJ) fusion is a growing trend in spine surgery. Some options for posterior SIJ fusion include 3D-printed triangular titanium implants or self-harvesting SIJ screws. Both implants require fixation within the sacrum and ileum. Fat embolism syndrome is a rare but known complication of lumbar pedicle instrumentation but has never been reported in association with SIJ fusion, regardless of implant type. We report the first two known cases of fat embolism associated with placement of SIJ fusion devices during long construct posterior spine fusion. Case Description: Case 1-a 50-year-old female with multiple previous spine surgeries complicated by osteomyelitis/diskitis that was successfully eradicated, underwent T10-pelvis posterior spinal fusion (PSF), L4 pedicle-subtracting-osteotomy, and bilateral SIJ fusion. During implantation of each SIJ fusion device, the patient's hemodynamic status deteriorated necessitating vasopressor support, intravenous fluid bolus, and hyperventilation, but quickly resolved. The case was completed without further issue, and she had an uneventful post-operative course. Case 2-a 71-year-old female with a past medical history of ankylosing spondylitis, previous L2-L5 PSF, rheumatoid arthritis on chronic steroids, underwent a T9-pelvis PSF, bilateral SIJ fusion, L4 pedicle subtraction osteotomy, T10-L1 Smith Peterson osteotomies. After implantation of the second SIJ fusion device, she became hypotensive and tachycardic, pulses were absent, and cardiopulmonary resuscitation was initiated. Pulses returned quickly, the index surgery was terminated, and she was transferred to the intensive care unit (ICU). In the ICU she was quickly weaned off the ventilator on post-operative day 1. On post-operative day 4, the patient returned to the operating room for completion of the surgery and had an extended, but uneventful, recovery afterwards. Conclusions: We report on the first two known cases of fat embolism syndrome occurring immediately after implantation of SIJ fusion devices. Spine surgeons should be aware of this rare, but potentially fatal, complication. Collaboration with the anesthesia team and optimization of the patient's hemodynamic status prior to implantation may help prevent catastrophic complications.

11.
Trauma Case Rep ; 51: 101028, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38633377

ABSTRACT

Fat embolism syndrome (FES) is a rare complication of long bone fractures, with fulminant FES developing within 12 h of injury and often proving fatal (Shaikh, 2009 [1]). Here, we present a case of fulminant FES in a patient who developed sudden right heart failure after undergoing external fixation of a lower leg fracture and required veno-arterial extracorporeal membrane oxygenation (VA-ECMO). A 79-year-old woman injured in a traffic accident was transferred to our emergency department. Upon arrival, her level of consciousness deteriorated, and she developed circulatory failure. We promptly performed transcatheter arterial embolization for the pelvic fracture and external fixation of the tibiofibular fracture. Within four hours of the injury, she was admitted to our intensive care unit (ICU). Two hours after ICU admission, her hemodynamic status worsened, necessitating the administration of maximum catecholamine dose. Echocardiography revealed petechial hemorrhage of the palpebral conjunctiva and enlargement of the right ventricle. Despite maximal supportive care, the patient remained cardiovascularly unstable. Therefore, VA-ECMO was initiated to stabilize her hemodynamic status. Thereafter, her hemodynamics stabilized, and ECMO support was weaned off and removed on day 3. Subsequent magnetic resonance imaging revealed evidence of cerebral fat embolism. On day 9, she underwent open reduction of the left lower leg with internal fixation and was transferred to another hospital on day 29. This report documents the successful management of fulminant FES during the acute phase of multiple traumas. Clinicians should consider VA-ECMO when suspecting uncontrolled circulatory failure due to fulminant FES, even in the acute phase of multiple trauma.

12.
Zhongguo Gu Shang ; 37(3): 306-10, 2024 Mar 25.
Article in Chinese | MEDLINE | ID: mdl-38515420

ABSTRACT

OBJECTIVE: To explore characteristics of clinical and imaging findings in patients with fat embolism syndrome. METHODS: From January 2021 to October 2022,clinical manifestations of 13 patients with fat embolism due to fracture or orthopaedic surgery were retrospectively analyzed,including 11 males and 2 females,aged from 17 to 60 years old. Mental and respiratory abnormalities and changes in vital signs occurred after admission or after surgery,and patient's chest and brain imaging results were abnormal. The patient's mental and respiratory abnormalities,vital signs,chest and brain imaging results were continuously monitored. RESULTS: The main clinical manifestations of fat embolism syndrome were abnormal pulmonary respiration in 13 patients,abnormal central nervous function in 7 patients,and spotted rash in 2 patients. Chest CT showed diffuse distribution of ground glass shadows in 13 patients,and severe symptoms were "snowstorm". Nine patients with ground glass fusion consolidation,5 patients with multiple nodules and 4 patients accompanied by bilateral pleural effusion. Head CT findings of 5 patients were negative,and head MRI findings of 1 patient showed multiple T1WI low signal,T2WI high signal shadow,DWI high signal shadow,and "starry sky sign" in basal ganglia,radiative crown,hemioval center,thalamus,frontal parietal cortex and subcortex. CONCLUSION: Fat embolism syndrome has a high mortality rate. Clinical manifestations of respiratory system and nervous system are not specific,and the skin spot rash has a characteristic manifestation. The "blizzard" sign is the specific manifestation of chest X-ray and CT examination of fat embolism,and the "starry sky" sign is the typical manifestation of diffusion-weighted sequence of brain MRI examination of fat embolism.


Subject(s)
Embolism, Fat , Exanthema , Male , Female , Humans , Adolescent , Young Adult , Adult , Middle Aged , Retrospective Studies , Magnetic Resonance Imaging , Brain , Embolism, Fat/diagnostic imaging , Embolism, Fat/etiology
13.
J Surg Case Rep ; 2024(2): rjae042, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38344138

ABSTRACT

A 14-year-old male patient was successfully treated with the reamer irrigator aspirator for femur intramedullary rod preparation after sustaining right and left closed femur fractures because of an all-terrain vehicle accident. In patients already categorized as high risk for fat embolism syndrome, such as those with bilateral femur fractures, reaming both femora greatly increases the likelihood of this complication. The reamer irrigator aspirator provides an effective tool that potentially mitigates the risk of fat embolism syndrome in pediatric patients with this type of orthopedic trauma.

14.
J Forensic Sci ; 69(2): 718-724, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38317612

ABSTRACT

Fat embolism syndrome (FES) is a potentially life-threatening condition that develops when fat embolism leads to clinical symptoms and multisystem dysfunction. The classic triad of respiratory distress, neurologic symptoms, and petechial rash are non-specific, and the lack of specific laboratory tests makes the diagnosis of FES difficult. Although FES is most common after long bone fractures, multiple conditions some of which are atraumatic have been associated with the development of FES. We report a case of FES that occurred in the setting of a non-traumatic compartment syndrome of the upper extremities. The pathologic and clinical findings, pathophysiology, diagnostic challenges, and pathologic methods to properly diagnose FES are discussed with a review of the relevant literature. This case highlights the importance of the autopsy in making a diagnosis of FES in cases where death could otherwise be incorrectly attributed to multi-organ system failure, shock, or sepsis.


Subject(s)
Compartment Syndromes , Embolism, Fat , Fractures, Bone , Humans , Fractures, Bone/complications , Compartment Syndromes/complications , Embolism, Fat/etiology , Multiple Organ Failure/etiology , Autopsy
15.
Cureus ; 15(9): e45551, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37868491

ABSTRACT

Fat embolism syndrome (FES) is a rare but potentially life-threatening complication that can occur following orthopedic procedures, such as long bone fracture repairs. FES is caused by the release of fat globules into the bloodstream, leading to the obstruction of blood vessels and subsequent tissue damage. Pulmonary embolism (PE), a condition in which a blood clot travels to the lungs, is another potential complication of orthopedic procedures due to the mobilization of blood clots during surgery. We report the case of a 56-year-old female who presented to the emergency department with a left femur fracture following a mechanical fall and underwent open reduction internal fixation (ORIF) surgery for the fracture. The procedure was complicated by the development of FES and multiple small pulmonary emboli. The patient was managed postoperatively in the ICU, requiring support with multiple vasopressors and mechanical ventilation. She remained in the ICU for three days postoperatively and was discharged on postoperative day six to an inpatient rehabilitation facility.

16.
Cureus ; 15(9): e45450, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37859880

ABSTRACT

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.

17.
Respirol Case Rep ; 11(10): e01214, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37692762

ABSTRACT

Fat embolism syndrome (FES) is a rare but potentially fatal complication of trauma or orthopaedic surgery, which presents predominantly with pulmonary symptoms. The rapid worsening respiratory failure in a previously normal orthopaedic surgery or trauma patients usually get evaluated for pulmonary embolism, fat-embolism-related acute respiratory distress or transfusion related acute lung injury. Orthopaedic surgeons and clinicians need to be aware of related entity termed 'Fat Embolism related Diffuse Alveolar Haemorrhage' (FEDAH). The clinical presentation in an orthopaedic surgery of trauma patient with FEDAH are haemoptysis, worsening type 1 respiratory failure and oxygen requirement, drop in haemoglobin levels with chest x-ray/computed tomography suggestive of Diffuse alveolar haemorrhage (DAH). Early bronchoscopy and bronchoalveolar lavage (BAL) confirmation of DAH, presence of BAL haemosiderophages and lipid-laden macrophages are the pointers in the early diagnosis of FEDAH. It needs a high clinical suspicion and interdepartmental collaborative measures. Timely referral from orthopaedic surgeons, early bronchoscopy and treatment with steroids is key in diagnosis and management.

18.
Cureus ; 15(9): e45936, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37766778

ABSTRACT

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.

19.
Transfus Med Hemother ; 50(4): 360-364, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37767282

ABSTRACT

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.

20.
Clin Case Rep ; 11(6): e7496, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37305886

ABSTRACT

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).

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