Sickle cell disease (SCD) is a common hereditary hemoglobinopathy in Bahrain and is associated with a broad spectrum of acute and chronic multisystem complications. Fat embolization syndrome (FES) is a rare but life-threatening complication of SCD that results from the systemic release of fat globules, typically originating from ischemic bone marrow, leading to pulmonary, neurological, and multiorgan dysfunction. Early diagnosis is often challenging due to its non-specific clinical presentation and overlap with other sickle cell complications. We report the case of a 22-year-old male with SCD who presented with a vaso-occlusive crisis (VOC) and subsequently developed acute chest syndrome and pulmonary embolism during hospitalization. The patient later experienced acute neurological deterioration, and brain magnetic resonance imaging (MRI) demonstrated the characteristic “starfield pattern,” supporting the diagnosis of cerebral fat embolization. His clinical course was further complicated by acute kidney injury, sepsis-induced cardiomyopathy, and tension pneumothorax, requiring prolonged intensive care management. Treatment included repeated red blood cell exchange transfusions alongside comprehensive supportive care. The patient demonstrated gradual neurological and cardiopulmonary recovery following an extended intensive care unit stay. This case highlights the diagnostic complexity of FES in SCD and emphasizes the importance of early clinical suspicion in patients presenting with respiratory failure or neurological deterioration following VOC. MRI, particularly the identification of the starfield pattern, serves as a key diagnostic tool. Prompt recognition and initiation of exchange transfusion remain critical in improving clinical outcomes and reducing mortality.
AlQassab et al. (Sat,) studied this question.