(458) Sickle Cell’s Starry Night: A Case of Acute Chest Complicated by Fat Embolism Syndrome

Introduction: Fat embolism syndrome, often seen in the setting of traumatic long bone fracture, is a rare but potentially fatal complication of sickle cell disease caused by bone marrow necrosis. Patients usually present with respiratory distress, neurological changes, and petechiae. We present a case of fat embolism syndrome in a patient admitted for sickle cell crisis and acute chest syndrome progressing to acute encephalopathy with septic shock and multiorgan failure.

Description: A 22-year-old male with a history of frequent sickle cell crises and acute chest syndrome presented to the emergency department with acute chest pain. Vital signs were stable, and the physical exam and chest imaging on presentation were unremarkable. The patient was diagnosed with vaso-occlusive crisis and managed with IV fluids and analgesia. On hospital day 5, the patient developed sudden acute encephalopathy and acute respiratory failure requiring intubation. Chest radiograph demonstrated a new left lung consolidation. CTA chest showed left lung nodular opacities with interlobular septal thickening and no pulmonary embolism. The patient was diagnosed with acute chest syndrome and started on plasma exchange transfusion. Lab work was notable for elevated INR and transaminases concerning for acute liver injury, as well as acute renal failure requiring renal replacement therapy. Given continued encephalopathy, a non-contrast MRI of the brain showed a “starfield” pattern of subcortical ischemic strokes consistent with fat emboli. The patient was managed with supportive care. He was ultimately weaned off vasopressors, hemodialysis, and extubated. On hospital day 31, he was discharged to an inpatient acute rehab unit. After two weeks in inpatient rehab, his mental status improved back to baseline, and he was discharged home with minimal deficits.

Discussion: Fat embolism causing acute chest in sickle cell crisis is a rare but reported phenomenon. Cases typically carry high morbidity and mortality. Post-mortem diagnosis is usual, but antemortem diagnosis can be made in the setting of characteristic imaging findings, as seen in our case. We highlight the need for a high index of suspicion and the importance of early exchange transfusion.