scholarly journals Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Danielle M. Graff ◽  
Erin Owen ◽  
Robert Bendon ◽  
Salvatore Bertolone ◽  
Ashok Raj
Keyword(s):  
Correct Diagnosis ◽  
Brain Lesion ◽  
Signs And Symptoms ◽  
Fat Embolism ◽  
Sickle Cells ◽  
Embolism Syndrome ◽  
Pain Crisis ◽  
High Index Of Suspicion ◽  
The Brain ◽  
Hemoglobin Sc Disease

An adolescent with mild hemoglobin SC disease presented with pelvic pain with subsequent respiratory and neurologic deterioration, which led to ultimately death. The autopsy demonstrated acellular fat emboli particularly in the lung and brain. There was marrow necrosis in the lumbar spine with aggregated sickle cells and positive parvovirus immunostaining. The brain lesion both grossly and microscopically presented a distinct pathology of acellular fat emboli that led to the correct diagnosis of this increasingly recognized association of sickle hemoglobinopathies with fat embolism syndrome (FES). A clinical diagnosis of FES is difficult to confirm in many patients with sickle hemoglobinopathy presenting with pain crisis because of concurrent illness. However, this case report highlights the need for a thorough knowledge of the signs and symptoms of the syndrome and a high index of suspicion for the diagnosis to be made premortem.

scholarly journals P.006 Keeping neurosarcoidosis on the differential

2016 ◽  
Vol 43 (S2) ◽  
pp. S22-S23
Author(s):  
E Spinelli ◽  
G Blevins ◽  
J Mailo ◽  
L Atilano ◽  
H Leonard ◽  
...  
Keyword(s):  
Correct Diagnosis ◽  
Signs And Symptoms ◽  
Lymphocytic Infiltration ◽  
Current Treatment ◽  
Cns Involvement ◽  
Normal Limits ◽  
Pontine Lesion ◽  
Initial Imaging ◽  
High Index Of Suspicion ◽  
The Brain

Background: Sarcoidosis is a multiorgan autoimmune disease characterized by the presence of non-caseating granulomas. The diagnosis can be difficult, particularly with central nervous system (CNS) involvement, and pathology outside of the CNS has to be carefully evaluated. Early and correct diagnosis is crucial for appropriate management particularly in children where sarcoidosis and neurosarcoidosis are rare. Methods: We describe a 16 year old previously healthy boy who presented with progressive pyramidal neurological signs and symptoms localizable primarily to the brain stem. Results: Initial imaging revealed striking brainstem, as well as cerebral, cerebellar and spinal cord perivascular enhancement. Lung involvement was subclinical with a miliary pattern on chest imaging and needle biopsy revealed an interstitial lymphocytic infiltration. Extensive serum and CSF rheumatological, autoimmune and infectious investigations were noncontributory. Serum ACE levels were at first within normal limits. Steroid treatment stabilized symptoms and perhaps coincidentally, separate rituximab treatments were followed within days by vertigo (with a new pontine lesion) or a respiratory decompensation. A wedge lung biopsy revealed granulomatosis. Current treatment consists of mycophenolate, methotrexate with a prednisone wean. Conclusions: This case report reinforces the varied manifestations and mimics of sarcoidosis (including CLIPPERS) and highlights the need for a high index of suspicion despite apparently negative investigations.

Bone marrow necrosis and fat embolism syndrome: a near fatal complication in previously undiagnosed sickle beta + thalassaemia

2021 ◽  
Vol 14 (1) ◽  
pp. e238317
Author(s):  
Nibash Budhathoki ◽  
Sunita Timilsina ◽  
Bebu Ram ◽  
Douglas Marks
Keyword(s):  
Bone Marrow ◽  
Multiorgan Failure ◽  
Rare Condition ◽  
Altered Mental Status ◽  
Fat Embolism ◽  
Bone Marrow Necrosis ◽  
Embolism Syndrome ◽  
Hb S ◽  
High Index Of Suspicion ◽  
Specific Symptoms

Prevalence of haemoglobin sickle-β+ thalassaemia (Hb S/β+thal) is variable with geography ranging from 0.2% to 10% among sickle cell patients. Clinical presentation of Hb S/β+thal patients depends on HbA level, with milder disease often going undiagnosed. However, rarely these patients can present with a fulminant vaso-occlusive crisis (VOC). Given VOC can present with non-specific symptoms, the diagnosis and treatment is often delayed. Here, we present a patient who initially developed altered mental status, pancytopenia and multiorgan failure due a critical VOC resulting in bone marrow necrosis and fat embolism. Subsequent workup confirmed that our patient had Sickle-β+ thalassaemia, which had gone undiagnosed, despite subclinical evidence of haemolysis on routine lab work for years. Following diagnosis and initiation of RBC exchange, he improved significantly and was discharged home. High index of suspicion and bone marrow biopsy is vital for early diagnosis and management of this rare condition.

scholarly journals A rare case of fat embolism syndrome secondary to abdominal liposuction and gluteal fat infiltration

2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Salvador Recinos ◽  
Sabrina Barillas ◽  
Alejandra Rodas ◽  
Javier Ardebol
Keyword(s):  
Right Ventricular Dysfunction ◽  
Signs And Symptoms ◽  
Fat Embolism ◽  
Fat Infiltration ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome ◽  
Threatening Condition ◽  
Life Threatening ◽  
Bone Marrow Harvesting ◽  
Dermatologic Manifestations

Abstract Fat embolism syndrome (FES) is a rare, life-threatening condition habitually associated with traumatic events such as fractures and, less commonly, burns, liposuction and bone marrow harvesting and transplant [ 1]. The biochemical theory for this condition suggests that fat droplets embolize and convert into fatty acids, eventually leading to toxic injury and inflammation, which results in increased vascular permeability, edema and hemorrhage [ 2]. FES may have an asymptomatic interval lasting 12–72 hours after the insult; however, in some cases, signs have also been seen intraoperatively. Pulmonary signs and symptoms are customarily the earliest and manifest in 75% of patients. Nevertheless, neurologic and dermatologic manifestations are also characteristic, and most severe cases could perhaps present with disseminated intravascular coagulation, right ventricular dysfunction, shock or death. The following case consists of a 37-year-old patient that presented with fat embolism syndrome during liposuction and gluteal fat infiltration.

scholarly journals FAT EMBOLISM AND FAT EMBOLISM SYNDROME;

2008 ◽  
Vol 15 (04) ◽  
pp. 407-413
Author(s):  
ANSAR LATIF ◽  
ANILA BASHIR ◽  
AURANGZEB , ◽  
Umar Ghani
Keyword(s):  
Fat Embolism ◽  
Long Bones ◽  
Neurological Manifestations ◽  
Cell Disease ◽  
Fat Embolism Syndrome ◽  
Severe Burns ◽  
Embolism Syndrome ◽  
Threatening Condition ◽  
Life Threatening ◽  
High Index Of Suspicion

Fat Embolism and the associated Fat Embolism Syndrome is a serious and potentially life threatening condition. It tends tooccur usually after fractures or intramedullary instrumentation of long bones. Non-traumatic conditions such as Diabetes Mellitus severe Burns,SLE, sickle cell disease and Pancreatitis can also lead to Fat Embolic syndrome. Young adults are commonly affected. Presentation consistsof an asymptomatic interval followed by pulmonary and neurological manifestations combined with petechial haemorrhages. The diagnosislargely depends on high index of suspicion and exclusion of other conditions. Treatment of this condition remains supportive. Mortalityassociated with this condition is significant, ranging from 10-20% .

MR imaging of the brain in fat embolism syndrome

2007 ◽  
Vol 15 (3) ◽  
pp. 187-192 ◽  
Author(s):  
Joseph Jen-Sho Chen ◽  
James C. Ha ◽  
Stuart E. Mirvis
Keyword(s):  
Mr Imaging ◽  
Fat Embolism ◽  
Fat Embolism Syndrome ◽  
Embolism Syndrome ◽  
The Brain

scholarly journals Liposuction-Induced Fat Embolism Syndrome: A Brief Review and Postmortem Diagnostic Approach

2018 ◽  
Vol 142 (7) ◽  
pp. 871-875 ◽  
Author(s):  
Colby A. Cantu ◽  
Elizabeth N. Pavlisko
Keyword(s):  
Supportive Therapy ◽  
Signs And Symptoms ◽  
The United States ◽  
Fat Embolism ◽  
Specific Therapy ◽  
Fat Embolism Syndrome ◽  
Neurologic Impairment ◽  
Embolism Syndrome ◽  
Life Threatening ◽  
Elective Procedure

Lipoplasty, or liposuction, the surgical process of removing excess fat, is an elective procedure with rising frequency in the United States. Fat embolism syndrome is a clinical diagnosis and is defined as fat in the circulation with an identifiable clinical pattern of signs and symptoms (eg, hypoxemia, respiratory insufficiency, neurologic impairment, and petechial rash) that occur in the appropriate clinical context. Fat embolism syndrome following liposuction is a life-threatening complication, although its incidence is low. Currently, there is no specific therapy for fat embolism syndrome, so prevention, early detection, and supportive therapy are critical. Many cases of fat embolism syndrome are undiagnosed or misdiagnosed; however, postmortem examination can provide the means for appropriate diagnosis. Therefore, a pathologist must keep a keen eye, as microscopic fat emboli are difficult to appreciate with routine tissue processing and staining.

scholarly journals Adolescent Fat Embolism Syndrome after Closed Tibial Shaft Fracture: Treatment with Emergent External Fixation

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Dillon C. O’Neill ◽  
Graham J. Dekeyser ◽  
Alexander J. Mortensen ◽  
Christopher A. Makarewich
Keyword(s):  
External Fixation ◽  
Shaft Fracture ◽  
Fat Embolism ◽  
Adolescent Male ◽  
Tibial Shaft Fracture ◽  
Tibial Shaft ◽  
Fat Embolism Syndrome ◽  
Football Game ◽  
Embolism Syndrome ◽  
High Index Of Suspicion

Case. An adolescent male developed fat embolism syndrome 24 hours after sustaining a closed right tibial shaft fracture in a football game. The patient was treated with emergent external fixator application due to declining respiratory and mental status and experienced swift recovery after stabilization. He was treated with an intramedullary nail within 1 week of injury. Conclusion. Pediatric fat embolism syndrome is uncommon, and a high index of suspicion is required to facilitate appropriate orthopaedic involvement. External fixation can be performed emergently with minimal fracture manipulation. Rapid provisional fixation appears to have facilitated recovery in this example.

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