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“ IMA PRAYER ” May happiness come to all May all be free from disease May everyone of us ensure that no one suffers from pain or sorrow Neither do I desire the crown, nor heaven, nor rebirth I only desire to alleviate the sufferings of the creatures burning in the fire of pain or sorrow All " Members" IMA Pathankot Fat Embolism Syndrome (FES) (in medical science till today) Dr. Puneet Sharma M.B.B.S., D.Ortho., DNB(Ortho.) Kalika hospital, Pathankot Fat Embolism, an entity first described as early as 1862, has remained a mystery to the medical science till date The abstruseness on diverse aspects of its patho-physiology and management has been well-acknowledged Fat embolism in humans was first recognized in 1861 by Zenker who described fat droplets in the lung of a railroad worker who had suffered a fatal thoraco-abdominal crush injury. Zenker FA. Beitrage zur Anatomie und Physiologie der Lunge. J Braundorf 1861. Twelve years later, Von Bergmann in 1873 clinically diagnosed fat embolism syndrome (FES) in a patient with a fractured femur. Von Bergmann EB. Ein fall todlicher fettembolie. Klan Wochenschr 1873; 10: 385–7. Fat Emboli Fat droplets that travel through the circulation Fat droplets “Fat Embolism” Fat Embolism: A process by which fat emboli passes into the bloodstream and travel within a blood vessel. "Fat Embolic Syndrome" (FES) The "Fat Embolic Syndrome" (FES) is defined as the occurrence of injury and dysfunction of one or more organs, caused by the occurrence of a Mechanical blockage or Biochemical injury to vascular capillaries caused by circulating droplets of fat with diameters about 8 to 10 microns, usually getting trapped in the capillary meshwork. This is a complication or a non-typical evolution of FE • It is usually asymptomatic, but a few patients will develop signs and symptoms of multi-organ dysfunction, particularly involving the triad of lungs, brain, and skin. • A small percentage of these patients develop picture similar to ARDS, this was recognized as FES long before ARDS entered into medical literature Incidence Surprisingly, the incidence was 0.9% when only clinical criteria were used to diagnose FES Whereas with the aid of postmortem examination the incidence was as high as 20%. The incidence of FES ranges from < 1 to 29% in different studies. Bulger et al. in their retrospective study, reported an incidence of < 1% Fabian et al. in their prospective study, reported an incidence of 11–29% Mortality from FES has been reported to be as high as 20 percent Mainstays of Treatment in “FES” Early diagnosis Low tidal volume with PEEP Supportive treatment Causes of “Fat Embolism” Severe traumas of the subcutaneous tissue Hiss et al. Beaten to death: why do they die? J Trauma 1996; 40:27-30. Liposuction and perinasal autologous fat injection Scroggins C et al. Md Med J 1999; 48:116-8. Ross AP et al. Surgery 2009; 65:271-3. Trauma Non -Traumatic Causes Acute pancreatitis, Fatty liver, Corticosteroid therapy, Lymphography, Fat emulsion infusion Haemoglobinopathies. Saldeen et al. J Trauma 1970; 10:273-86. Masson et al. Chest 1985; 88:908-14. Capan et al. Anesthesiol Clin North Am 1993 Mudd et al. J.Trauma 2000; 48:711-715. PATHOPHYSIOLOGY 2 Theories Mechanical…… (Baker et al) Biochemical….. (Lehman et al) Mechanical Theory (Baker et al) Systems affected include Lungs, Brain and Any Organ via circulation. Fat droplets are deposited in the pulmonary capillary beds and travel through arteriovenous shunts to the brain. Mechanical Theory…………. Micro-vascular lodging of droplets Local ischemia and inflammation Release of inflammatory mediators, platelet aggregation, and vasoactive amines. Mechanical Block Biochemical Theory (Lehman et al) Hormonal changes caused by trauma and/or sepsis induce systemic release of free fatty acids (FFA) as chylomicrons which cause the systemic FES. FES is dependent upon degradation of the embolized fat free fatty acids. Biochemical Theory………. The FFAs acts locally to produce an increase in the permeability of the capillary bed, a destruction of the alveolar architecture, and damage to lung surfactant. Onset of symptoms may coincide with Agglutination. The delay in onset of symptoms may be due to the time required for the production of toxic metabolites. CRP (acute phase reactant), which is elevated in trauma patients, appears to be responsible in lipid agglutination for both traumatic and non-traumatic FES. RISK FACTORS Young age, Closed fractures, Multiple fractures, and conservative therapy for long-bone fractures. Risk is especially high with femoral shaft fracture and concomitant head injury; Clinical Presentation Diagnosis is made “Clinically NOT Chemically” It does not matter how much fat globules are in the circulation, it just matters if pt have their side effects. FES typically manifests 24 to 72 hours after the initial insult. Rarely <12 hrs or >72 hrs. Classic triad: Hypoxemia Neurologic abnormalities Petechial Rash Clinical Presentation………. The number of clinically evident cases of respiratory distress represents the tip of the iceberg, with a large number of lung injury remaining clinically inapparent Pulmonary dysfunction is the earliest to manifest Seen in 75% of patients Progresses to respiratory failure in 10% of the cases. Respiratory dysfunction is major cause of mortality, which is about 10-20%. Clinical Presentation & Investigations of “FES” Count…. LUNGS Respiratory Insufficiency pH .. PaCO2.. ABG HCO3… PaO2… Lactate… ABG analysis showing an unexplained increase in pulmonary shunt fraction and an alveolar-to-arterial oxygen tension difference, especially within 24–48 h of a sentinel event associated with FES, is strongly suggestive of the diagnosis. Clinical Presentation & Investigations of “FES” Count…. LUNGS X-ray Chest A diffuse bilateral infiltrate i.e. “Snow Storm Appearance”, predominant on basal and per-hillar regions, and usually appears only about 24 to 48 h after trauma . This radiological aspect, although 'typical' of FES, is found in only 30 to 50% of the cases and cannot be considered as pathognomonic of this syndrome Capan LM, Miller SM, Patel KP. Fat embolism. Anesthesiol Clin North Am 1993; 11:25-54. Clinical Presentation & Investigations of “FES” Count…. LUNGS CT-Chest Ground glass opacification with interlobular septal thickening “FES” affects the lung in a Heterogeneous fashion Clinical Presentation & Investigations of “FES” Count…. LUNGS Pulmonary Perfusion Scintiscan Normal perfusion lung scan Pulmonary embolism. (a) Perfusion scan shows multiple areas of ischemia. (b) Ventilation image using Xe, shows normal aeration of both lungs. This set of findings is considered pathognomonic of pulmonary embolism. Clinical Presentation & Investigations of “FES” Count…. Brain Anxiety, Confusion, Convulsions, irritability…Coma 70 to 80% cases MRI - Brain “star field pattern” MRI showing hyper intense areas foci of ischemia suggestive of fat embolism syndrome Clinical Presentation & Investigations of “FES” Count…. Autopsy finding of Brain showing foci of Petechial Hemorrhages suggestive of fat embolism syndrome Autopsy finding of Brain Clinical Presentation & Investigations of “FES” Count…. SKIN Incidence between 20% and 60% in FES This is embolization of small dermal capillaries leading to extravasations of erythrocytes. It has no relation to platelets. This produces Petechial rash ( 1mm to 2mm) in the conjunctiva, oral mucosal membranes, Skin folds of upper body parts as neck, axilla, chest. It is Self Limiting Resolve in 36 h to 7 days Fat globules float and therefore distributed to branches of the aorta that arise from the top of the aortic arch that are uppermost Clinical Presentation & Investigations of “FES” Count…. The retina 50% cases Clinical Presentation & Investigations of “FES” Count…. LABORATORY CHANGES ANEMIA ---The reduction of hematocrit, (70% of patients) without apparent hemorrhage, within the first or second day after trauma THROMBOCYTOPENIA: -- Decrease Platelet (< 1.5 Lacks)occurring only in about 30% to 50% of the cases FAT DROPLETS :------ in Blood, Urine, Broncho-alveolar wash SERUM FREE FATTY ACID:----- increases SERUM LIPASE:----- increases ESR :----- increases These changes are "typical" of FES But they are not unique or diagnostic of this syndrome Since FES is not predictable preoperatively, one of the study proposed that intraoperative guidelines can be used to reduce risk of this complication. How we detect that really “Emboli” are generated during IM Fracture Fixation Trans- Esophegeal Echo Cardiography (TEEC) Atrial Blood Sampling Diagnostic Criteria's FES is a “Clinical diagnosis” There are Three imp. Criteria in literature:1….. Gurd and Wilson's criteria 2…… Schonfeld’s Scoring criteria 3….. Lindeque’s Criteria Gurd and Wilson's criteria ( 2 Major or 1 Major + 4 Minor ) required to diagnose FES. Schonfeld’s Scoring criteria A score of more than 5 is required to diagnose FES. Lindeque’s Criteria Early, reliable, utilitarian predictive factors for fat embolism syndrome in polytrauma patients -Nirmal raj gopinath et al; Indian J Crit Care Med. 2013 Jan-Feb; 17(1): 38–42. Concludes Patients presenting with a triad: 1) polytrauma (NISS >17) 2) raised initial serum lactate and 3) even a transient episode of hypoxia These poly-trauma Pt. are at a higher risk for developing FES/post-traumatic hypoxia Fat embolic syndrome may present with a wide spectrum of acute lung injury severity, with oxygenation impairment ranging from mild asymptomatic reduction in oxygen saturation to ARDS with severe shunt physiology Bosse MJ, et.al. J Bone Joint Surg Am 1997;79:799 - 809. Current concepts of respiratory insufficiency syndromes after fracture. J Bone Joint Surg Br 2001;83:781 - 791. TREATMENT Hypertonic Glucose:---- reduces the concentration of circulating fatty acids Human Albumin:----- chelating free fatty acids Heparin Dextran-40 Ethylic Alcohol:----- Alcohol had the ability of reducing serum lipase activity and, consequently, of reducing the release of fatty acids. Nitric Oxide Therapy - in Fat Embolism Syndrome to Prevent Right Heart Failure; (E. Brotfain et al Case Rep Crit Care. 2014; 2014) DHEA (Dehydroepiandrosterone) - modulates the inflammatory response in a bilateral femoral shaft fracture model (P. lichte Eur J Med Res. 2014; 19(1): 27.) No prospective and randomized studies were found in literature NOT Accepted as Treatment Corticosteroids ( Controversial) Methylprednisolone is the study drug Methylprednisolone …30mg/kg initial dose repeated @ 4 Hours, 1gm dose repeated @ 8 Hours ( Total 3 Doses ) Key is to initiate treatment early and for a short period of time being watchful of side effects Its efficiency, however, has never been proved by studies and its use is not considered in many studies. Capan LM, et.al.Anesthesiol Clin North Am 1993; 11:25-54. Robinson CM.et.al. J Bone Joint Surg Br 2001; 83: 781-91 "Disease with an undetermined Pathophysiology causes a non-specific treatment" SUPPORTIVE TREATMENT Oxygen therapy Bronchodilator agents to overcome bronchospasm Conservative fluid management Endotracheal intubation with mechanical ventilation at high inspired oxygen levels, using continuous PEEP Fracture Fixation Technique -Controversial• IM Nail - Reamed v/s Un-Reamed – Decreased with Unreamed Technique • Pape et al – No Difference • Keating et al • Canadian OTS • IM Nail Reamed v/s Plate Osteosynthesis – No Difference In Pulmonary Dysfunction i.e. FES • Bosse et al Fat embolism syndrome after IM nailing an isolated open tibial fracture in a stable patient: a case report by Gustavo Aparicio* BMC Research Notes 2014, 7:237 TIPS to Prevent “FES” during “IM Nailing” Drive the Nail slowly…Give pause after few blows…Give time for marrow to drain out Enlarge the Entry point….give space to the marrow to drain out Use the reamer in which Reamer Head size is short and with deep flutes During reaming longitudinal velocity of reamer should be less Using smaller diameter unreamed nails have also been shown to produce lesser lung injury Regarding the two most common kinds of rods - cylindrical and fluted - the cylindrical type causes a much higher increase of IMP, and, thus, cause much more FE than fluted rods TIPS to Prevent “FES” during “Cementing” in Arthroplasty VENTING: Make 4 - 6 mm-diameter hole in the diaphysis portion located at few centimeters from the prosthesis end RETROGRADE FILLING:-------- Filling from the distal to proximal VISCOSITY:------------ use of low-viscosity cements PROXIMAL VACUUM :- Applied a vacuum of 600mmHg through a metal cannula or intramedullary catheter introduced at the intertrochanteric line during prosthesis cementation. PREVENTION of “FES” Avoid hypovolemia and hypoxia Close monitoring of the blood pressure and the PaO2 Adequate analgesia is important to limit the sympathomimetic response to injury. Concept of “Early Fixation" of fractures in “Clinically Stable” Patients With supportive care and early fixation “FES” has a favorable outcome. Thanks