Fat Embolization Syndrome Secondary to Steroid Treatment in a Case of Sickle Cell Vaso-Occlusive Crisis

Table 1

The following keywords were used for a literature search in PubMed: “fat embolism in sickle cell,” “neurological sequelae of fat embolism in sickle cell,” “presentation of fat embolism in sickle cell patients,” and “treatment of fat embolism in sickle cell.”

Article titles	Age (in years) and sex of the patient	Initial symptoms	Presentation on imaging	Procedure	Treatment and complications	Ref. no
Fat embolism in sickle-cell disease: A case report with literature review	37 females	Lower back pain and bilateral shoulder pain	CT/CTA reports were normal	Lumbar puncture	Red cell exchange transfusion	[7]
PMID: 36741476			Brain MRI showed a “starfield appearance” and incidental aneurysm in the supraclinoid portion of the right ICA		Psychomotor slowness in long-term follow-up
Bone marrow necrosis and fat embolism syndrome in sickle cell disease during COVID-19 infection treated successfully with sequential red cell and plasma exchange	35 males	Generalized body aches, altered mental status, and fever	Brain MRI showed multiple areas of restricted diffusion and microhemorrhages in a so‐called “starfield pattern”	Lumbar puncture and bone marrow biopsy	Red cell exchange transfusion and plasma cell exchange transfusion	[8]
PMID: 36718354					No complications
A suspected case of cerebral fat embolism triggering a drug-resistant status epilepticus in a HbS/β+-thalassaemia patient	61 females	Severe precordial chest pain and dyspnea	Brain MRI showed widespread, nonconfluent areas of ischemia in multiple anterior and posterior vascular distributions with microhemorrhages consistent with a “starfield appearance” with a “walnut kernel microbleed” pattern	Lumbar puncture	Red cell exchange transfusion therapy	[9]
PMID: 35444771					No complications
Complete neurological recovery from fat embolism syndrome in sickle cell disease after sequential red cell exchange transfusion and therapeutic plasma exchange	27 males	Altered mental status	Brain MRI showed multiple widespread microhemorrhages showing not only the characteristic “starfield” pattern but also a cytotoxic lesion of the corpus callosum, known to be the result of direct neurotoxicity by proinflammatory cytokines	Nil	Red cell exchange transfusion	[10]
PMID: 34489185					No complications
Bone marrow necrosis and fat embolism syndrome: a near-fatal complication in previously undiagnosed sickle beta + thalassemia	37 males	Fever, altered mental status, low back pain, unsteady gait, and urinary incontinence	Brain MRI showed evidence of subacute ischemia and chronic microhemorrhages	Lumbar puncture and bone marrow biopsy	Red cell exchange transfusion	[11]
PMID: 33408108			CT scan of the abdomen and pelvis showed moderate splenomegaly, hepatomegaly, cholelithiasis, and diffuse patchy sclerotic osseous abnormalities		No complications
Fat embolism syndrome in sickle cell β-thalassemia patient with osteonecrosis: an uncommon presentation in a young adult	34 males	Fever, hypoxia, encephalopathy, and generalized body aches	Brain MRI with and without contrast revealed extensive punctate multifocal areas of diffusion restriction throughout the basal ganglia, thalami, and white matter of both hemispheres consistent with multifocal areas of punctate ischemic infarction in the acute to subacute stages	BM biopsy	Red cell exchange transfusion	[12]
PMID: 34008428				Quinton catheter was placed for lifelong exchange transfusions every 4 to 6 weeks to keep hemoglobin S level to a goal of <30%	No complications
Neurologic recovery in systemic nontraumatic fat embolism syndrome in an elderly patient with hemoglobin SC disease: a case report	75 females	Chest pain and diffuse body aches	Brain MRI showed extensive multiple microhemorrhages across the neuroparenchyma in a “starfield” pattern classic for cerebral FES	Nil	Red cell exchange transfusion	[13]
PMID: 32983503					She remained profoundly paretic and required ventilation and assisted feeding via tracheostomy and gastrostomy