Original Article

Analyzing fat syndrome in trauma patients at AIIMS Apex , New Delhi, India

Babita Gupta1, Nita D’souza2, Chhavi Sawhney3, Kamran Farooque4, Ajeet Kumar5, Pramendra Agrawal6, M C Misra7 1-3,5,6Departments of Anesthesia, 4Orthopedics and 7Surgery, Jai Prakash Narayan Apex Trauma Centre, AIIMS, New Delhi, India

ABSTRACT Background: (FES) is a constellation of symptoms and signs subsequent to orthopedic trauma. Materials and Methods: The clinical profile of FES in the trauma population was studied over 2 years and 8 months. Results: The incidence of FES among all patients with long bone and pelvic fractures was 0.7% (12). The mean severity score was 10.37 (SD 1.69) (range 9-14). The diagnosis of FES was made by clinical and laboratory criteria. Hypoxia was the commonest presentation (92%). The average days of onset of symptoms were 3.5 (SD1.29) days. Management included ventilator support in 75%, average ventilator days being 7.8 (SD 4.08) days. The average ICU stay and hospital stay were 9.1 days and 29.7 days, respectively. A mortality of 8.3% (1) was observed. Conclusion: Fat embolism remains a diagnosis of exclusion and is a clinical dilemma. Clinically apparent FES is unusual and needs high index of suspicion, especially in long bone and pelvic fractures.

Key Words: Fat embolism, risk factors, trauma

INTRODUCTION define the syndrome by clinical abnormalities. Many of the clinical abnormalities are subtle, and must be diligently sought in Fat embolism is the presence of fat globules in the peripheral patients with high suspicion by careful prospective monitoring. circulation and lung parenchyma most often after a long Classically patients present with respiratory, neurological and or . The fat embolism syndrome cutaneous manifestations as described by Gurd.[1] Over the (FES) has more serious manifestation of a multisystem years various authors have adapted the Gurd’s criteria with involvement. Clinical onset of symptoms may occur within slight changes. A retrospective analysis was done to study the 12 hrs; but usually patients manifest symptoms 24-72 hrs incidence and clinical profile of FES in trauma population in later. Presentation is variable and no individual symptom is our level I trauma center. diagnostic of the syndrome. The criterion for diagnosis of FES remains controversial. Some would accept only histological MATERIALS AND METHODS demonstration of fat macroglobules in the organs, while others A retrospective case record analysis was done in our level I Address for correspondence: trauma center, after seeking ethical committee approval medical Dr. Nita D’souza, E-mail: [email protected] records of patients from April 2007 to November 2009 in whom FES was diagnosed as per Gurd’s criteria were reviewed. Access this article online To meet the clinical diagnosis of FES, patients needed to Quick Response Code: demonstrate at least 1 major and 3 minor or 2 major and 2 Website: www.onlinejets.org minor signs. Data collected included demographics, injury severity scores (ISS), clinical presentation, time of onset of

DOI: symptoms of FES, laboratory investigations, management and 10.4103/0974-2700.83859 outcomes. Comparison between various diagnostic criteria was studied.

Journal of Emergencies, Trauma, and Shock I 4:3 I Jul - Sep 2011 337 Gupta, et al.: Fat embolism syndrome

RESULTS T2 and flair hyperintense lesions were seen in bilateral cerebral hemisphere, basal ganglia, thalamus, pons and cerebellum. There were 1692 patents with long bone and pelvic fracture, admitted during the study period. Fracture femur formed the The management of these patients was primarily supportive. largest contributor 60.4% (1022), followed by fracture tibia-fibula Steroids were not administered in any of our patients. Early at 32.6% (552) and pelvic fractures at 7% (118). Twelve cases fixation within 12-48 hrs was done in three patients (25%). Nine were identified to have FES as per Gurd’s criteria (incidence of patients required ventilator support due to respiratory distress, 0.7%). Five of 12 patients had multiple bone fracture (42%) hypoxia and poor Glasgow Coma Score. The average ventilator and one patient had associated vertebral fracture. The male to days were 7.8 (SD 4.08) days and the average stay in the intensive female ratio was 3:1. All patients were victims of road traffic care unit was 9.1 days. The average hospital stay was 29.7 days. accidents with mean ISS of 10.377 (SD ±1.69) with a range of One patient died of ARDS and septicemia consequent to fracture 9-14 [Figure 1]. The average age of patients was 24 years (range femur and subsequent complications. 18-28 years). The mean days of onset of symptoms were 3.5 days (SD ±1.29). Four patients presented on the second day (24-48 DISCUSSION hrs), three patients each on the fourth (72-96 hrs) and fifth day (96-120 hrs). Unusually, one patient presented postoperatively Fat embolises in almost every patient of long bone fracture or [Figure 2]. major tissue trauma.[2] Gurd’s criteria were proposed in 1974 and since then there is no ‘universal criteria’ or ‘gold standard’ for the None of the patients presented with all three major signs of diagnosis of FES.[3] The incidence of post-traumatic FES has Gurd’s criteria. Distribution of major and minor signs as per been reported to be as low as 0.2-0.9% in retrospective studies Gurd’s criteria, have been tabulated [Table 1]. Hypocalcemia to as high as 35% in prospective studies [Table 2].[1,2,3-6] Clinically and hyperbilirubinemia was seen in six patients (50%) and apparent FES in our study population was 0.7%. Our study hypoalbuminemia was present in five patients (41.7%). population comprised only of young adults as this particular Computerized tomogram (CT) pulmonary angiography was group of patients is more prone for road traffic accidents. There available in six patients with the findings of three (50%) being were no pediatric patients diagnosed with FES in our study. consistent with the diagnosis of FES. Magnetic resonance imaging (MRI) brain was done in three patients with neurological status Major signs alteration and was diagnostic of cerebral fat embolism (CFE) Patients with FES present with a classic triad of respiratory in all three patients. Multiple punctuate, scattered nonconfluent manifestations (95%), cerebral effects (60%) and petechiae (33%). [2,6] Fifty percent of the patients with FES caused by 6 long bone fractures develop severe hypoxemia and respiratory No. of patients insufficiency and require mechanical ventilation.[7] Majority of our 5 patients (92%) had respiratory distress and hypoxic presentation. 4 Other differential diagnoses of hypoxia were excluded. Bulger [2] 3 et al noted acute hypoxia in 96% patients of FES.

2 Neurological signs due to cerebral emboli occur in up to 86% 1 of cases and often occur after the development of respiratory distress.[8] The more common presentation as in our cases 0 9 ISS10 11 12 13 14 (25%) was acute confusional state although focal neurological ISS ISS ISS ISS ISS deficits (hemiplegia, aphasia, apraxia, visual field disturbances,

Figure 1: Injury severity score (ISS) distribution for patients with fat embolism syndrome (‘x’ axis: Injury Severity Score, ‘y’ axis: Table 1: Clinical presentation as per Gurd’s criteria No. of patients) Diagnostic criteria for fat embolism based on Gurd’s criteria Criteria/ Signs No. of patients (%) Major signs

Hypoxia (PaO2 <60 mm Hg) 11 (91.67) 24-48 hours - 4 patients Mental state changes (CNS depression) 3 (25) Petechiae (cutaneous changes) 1 (8.3) 48-72 hours - 2 patients Minor signs 72-96 hours - 3 patients Tachycardia (heart rate >120 beats/min) 10 (83.3) Thrombocytopenia(<1.5 lakh) 9 (75) 96-120 hours - 3 patients Unexplained anemia (decline in hematocrit) 9 (75) Hyperpyrexia (>39°C) 6 (50) Fat globules in fundus 2 (16.7) Fat globules in urine 1 (8.3) Figure 2: Onset of symptoms after injury

338 Journal of Emergencies, Trauma, and Shock I 4:3 I Jul - Sep 2011 Gupta, et al.: Fat embolism syndrome

Table 2: Incidence and mortality of fat embolism syndrome in various studies Year Study design Incidence % (n) Mortality % (n) Study (reference) 1983 Prospective- Randomized trial of steroids in 62 trauma patients 15% (9) in pts not given steroids None Schonfeld[12] 1984 Prospective- 80 consecutive trauma patients 8.75% (7) 2.5% (2) Chan[19] 35% of multiply injured patients 1987 Prospective- trial of steroids in 55 trauma patients 13% (7 by Gurd’s) None Lindeque[11] 29% (16 by Lindeque’s) 1990 Prospective- 96 consecutive long bone fractures over 12 months 11% (10) 10% (1) Fabian[20] 1993 Retrospective- 25 years review 0.26% (20) 20% (4) Robert[21] 1997 Retrospective- 10 years review of trauma cases 0.9% (27) 7% (2) Bulger[2] 2009 Retrospective- 32 months review in trauma patients 0.7% (12) 8.3% (1) Our study

[8] anisocoria) have also been described. None of our patients Table 3: Lindeque criteria with neurological deterioration had any residual neurodeficit. Diagnosis based on respiratory status alone Sustained PaO <60 mHg (pO <8 kPa) Petechial rash is considered pathognomonic of FES and 2 2 Sustained PaCO2 >55 mmHg (pCO2 >7.3 kPa) reportedly present in upto 60% of patients, usually on the Sustained respiratory rate >35/ min, inspite of adequate sedation conjunctiva, oral mucous membranes and skin folds of the neck Increased work of breathing, use of accessory muscles, dyspnea, tachycardia, anxiety [9] and axillae. Despite cutaneous changes being a major criterion Patient showing at least one of the above criteria was judged to have FES of the Gurd’s criteria, only one of our patients was reported to have petechial rash with an incidence of 8.3%. This probably could be attributed to the difficulty and delay in identifying rash Table 4: Schonfeld’s criteria in the darker skin of the Asian population, its presence for a Schonfeld’s criteria Score short duration of time and retrospective nature of the study. Petechiae 5 X-ray chest diffuse infiltrates 4 Other than Gurd’s there have been attempts at developing other Hypoxemia 3 diagnostic criteria, such as those described by Lindeque and Fever 1 [1,10,11] Tachycardia 1 Schonfield. Lindeque based the diagnosis of fat embolism solely Tachypnoea 1 on the respiratory status of the patient stating that Gurd’s criteria Confusion 1 [10] may underdiagnose FES. According to the criteria suggested Cumulative score of >5 needed for diagnosis of FES by Lindeque in our study 11 of 12 patients qualified. Schonfeld quantified the diagnosis of FES [Table 3].[11] As per Schonfeld’s criteria 10 of our patients qualify by having a cumulative score of chest radiographs to aid diagnosis of FES, although it shows greater than 5 [Table 4]. Schonfeld gave the maximum score to earlier, minor infiltrates than ordinary X-ray.[16] However, CT is petechiae which was, however, seen in only one of our patients. an insensitive diagnostic modality in FES.[9,17]

Minor signs and laboratory tests CFE is an uncommon but potentially life-threatening Recovery of fat globules in blood and identification of fat complication of long bone fractures. In our study three patients droplets within cells recovered by bronchoalveolar lavage (BAL) had neurological deterioration (altered mentation, confusion, has been suggested as a rapid and specific method for establishing disorientation) and all had diagnostic MRI brain findings. the diagnosis of the FES, although of uncertain significance. [12,13] Although CT brain findings were not contributory to the Our patients were not tested for fat globules in sputum or BAL. diagnosis of CFE, it played a role in excluding other causes Anemia and thrombocytopenia unexplained by any other cause of neurological dysfunction. Thus when brain lesions are seen were observed in nine (75%) of our patients. The cause for on MRI without the possibility of CFE must be hypocalcemia is not well-understood but may result from affinity considered. MRI findings in the acute stage of CFE consist of of plasma free fatty acids (FFA) for calcium or elevated serum diffuse patchy lesions throughout the brain, most commonly lipase.[8,14] Hypoalbuminemia (present in five patients) has also in the white matter and sub cortical grey matter. The lesions [15] been suggested due to FFA binding to albumin. were demonstrated by low signal on T2-weighted images in the cerebellum, cerebrum and brain stem as seen in our patients.

Imaging studies T2-weighted gradient-echo provides useful information on The chest radiographic appearance of fat embolism is determining the clinical severity of patients with CFE [Figures 3a nonspecific. [14] Although chest imaging play miniscule role and b].[18] MRI is highly sensitive and specific in detecting in diagnosis and management of FES, recognition of the encephalic lesions in FES and can detect lesions as small as 2 temporal sequence of radiological abnormalities can be a mm and as early as 4 hrs after trauma.[19,20] All three patients with clue in distinguishing pulmonary FES from other pulmonary CFE completely recovered without any neurological deficit, over conditions.[14,15] Thoracic CT does not contribute to the regular a period of 3 weeks.

Journal of Emergencies, Trauma, and Shock I 4:3 I Jul - Sep 2011 339 Gupta, et al.: Fat embolism syndrome

deterioration of neurological status and X-ray chest findings. 3) CFE is self-limiting with no residual neurological deficit. 4) The management primarily remains supportive: modern ICU care, ventilator support and efficient nursing care. Clinical expertise with high degree of suspicion in susceptible trauma population enables diagnosis and appropriate management of this relatively rare but significant syndrome. a b ACKNOWLEDGMENT Figure 3: Axial T2-weighted images showing hyperintensities in the corpus callosum, bilateral thalami (a) and centrum semiovale (b) We would like to thank Mr. Chaman Singh, Mr. Narayan Singh, Treatment Mr. Naresh and Mr. Inderpal for their technical support and help. Consideration of the differential diagnosis is essential. Other pulmonary pathological conditions were excluded before REFERENCES confirming the diagnosis. Medical care includes adequate hydration, oxygenation, ventilation, stable hemodynamics and 1. Gurd AR, Wilson RI. The fat embolism syndrome. 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