Fulminant cerebral fat embolism syndrome initially sparing the lungs after long-bone fractures: a case report of refractory status epilepticus.

BACKGROUND

Cerebral fat embolism syndrome (CFES) usually occurs within fat embolism syndrome, classically characterized by hypoxemia, neurological abnormalities, and petechial rash. Pulmonary-sparing CFES is uncommon and may delay diagnosis.

CASE PRESENTATION: A 23-year-old man sustained multiple lower-extremity long-bone fractures after a traffic accident. After emergency orthopedic surgery, he was fully conscious with preserved oxygenation. Approximately 12 h after injury, he developed lethargy; at that time, oxygen saturation was normal, and head CT, chest CT, and CT pulmonary angiography showed no intracranial, pulmonary parenchymal, or pulmonary embolic abnormality. Two hours later, generalized seizures with vomiting were followed by desaturation and intubation, indicating that the subsequent respiratory failure was temporally more consistent with aspiration than primary pulmonary fat embolism. After ICU admission, brain MRI demonstrated the characteristic "starfield" pattern. Day-1 resting right-heart contrast echocardiography showed no definite right-to-left shunt. Management was mainly supportive, including airway protection, mechanical ventilation, continuous EEG monitoring, antiseizure therapy, targeted temperature management, organ support, infection control, and staged orthopedic treatment.

OUTCOMES: Despite comprehensive treatment, the patient developed refractory status epilepticus, septic shock, rhabdomyolysis, acute kidney injury, hepatic dysfunction, and coagulopathy. Serial early neuron-specific enolase levels remained normal. He remained in an unresponsive wakefulness syndrome at 1 month and had a severe disorder of consciousness with a Glasgow Coma Scale score of 6 at 15 months.

CONCLUSION

Severe CFES may occur despite normal early pulmonary imaging and oxygenation. Early brain MRI is essential after unexplained neurological deterioration following long-bone fractures. In this case, normal early NSE did not exclude devastating neurological injury.