DMD patient with respiratory distress, fever, mild coagulopathy following multiple long bone fractures. Was it Fat Embolism Syndrome?


Clinical Management

Poster Number: 16


Virginia Kaperick, MD, Medical College of Wisconsin, Children's Wisconsin

Our patient is a 13 year old non-ambulatory, obese male with Duchenne Muscular Dystrophy (DMD), on chronic glucocorticoids who presented with respiratory distress following multiple extremity fractures concerning for possible Fat Embolism Syndrome. He was diagnosed with DMD at age 6 ½ years and started steroids a short time later. At age 13 ½ years he fell during a transfer from his power wheelchair, which then fell on top of him, resulting in bilateral ankle fractures and distal femur fracture. In the ED he had a low grade fever, no respiratory symptoms or neurologic changes, and was admitted overnight for pain control. He was discharged home the following day with splints and knee immobilizer. He presented again two days later with dry cough, tachypnea, hypoxia, and fever. Labs were remarkable for mildly elevated WBC and abnormal clotting factors, with negative respiratory pathogen panel. CT scan was negative for pulmonary embolus, with nodular and interstitial opacities felt consistent with atelectasis and pneumonia. He received supportive respiratory care and antibiotics in ICU x 3 days, returned to baseline, and was discharged home with diagnosis of pneumonia.
Fat Embolism Syndrome (FES) after bone injury or fracture is a rare complication but carries a mortality rate up to 15-20 % in the general population. FES is more common in DMD patients, with mortality rates reported as high as 44%. There is no specific pharmacologic therapy for FES. Maintaining a high index of suspicion, with early recognition and aggressive supportive therapy, are paramount given the high mortality risk. Fat embolism should have remained in the differential for this patient.

Learning points to be included in poster:
1.Review of Fat Embolism Syndrome and why DMD patients are at higher risk.

2.Review specific exam and imaging findings, treatment, and specific precautions in the DMD population