Fat embolism syndrome in DMD: Report on a novel case and systematic literature review

Fat embolisms can occur after a long bone fracture where bone marrow content accesses the circulation. This can lead to respiratory distress and neurological problems when the fragments get stuck in lung and brain capillaries.

Usually symptoms occur 1-3 days after a fracture. They can be mild and transient or lead to death. Duchenne patients are at risk for fractures and thus also for fat embolisms. Authors here present a case report of a 13 year old patient who underwent orthopedic surgery.

The patient had never been on steroids and was wheelchair dependent since 9 years. After surgery he first recovered well and then went into respiratory distress requiring ventilation after 24 hours. 9 days post surgery he was weaned off ventilation. Analysis revealed the patient had experienced fractures of both femoral during surgery. This resulted in fat embolisms in the lung but also the brain. Authors detected white matter injury. The patient has minimal consciousness 5 months post surgery.

Authors performed a literature review finding 11 cases of fat embolism syndrome in Duchenne patients after a fracture. This was usually due to a fall from the wheelchair or during transfer and usually of the femur. Most of the cases were on steroids.

Respiratory problems and tachycardia were most common symptoms and most patients needed ventilation. Some patients also experienced confusion, lethargy and seizures. 29% of patients died due to the embolism and 54% fully recovered.