AIRP Best Cases in Radiologic-Pathologic Correlation: Cerebral Fat Embolism Syndrome in Sickle Cell β-Thalassemia

Abstract

A 57-year-old African American woman with a history of sickle cell b-thalassemia presented to the hospital with a 3-day history of chest and back pain unresponsive to oxygen and analgesic therapy. Her medical history included multiple painful crises due to sickle cell-induced vaso-occlusion, multiple blood transfusions, hypertension, and diabetes mellitus. The evening of her admission, she developed a fever and hypotension, and a sepsis evaluation was initiated. She was treated with empirical antimicrobial agents and received oxygen, a red blood cell transfusion, and medications for pain. Two nights later, she became mildly confused. Unenhanced computed tomography (CT) of the head was performed. The following morning, magnetic resonance (MR) imaging of the brain and MR angiography of the head and neck were performed. Later that day, the patient's hypotension worsened, and she became difficult to arouse. Intubation was performed to protect her airway, and she was transferred to the intensive care unit. Thrombocytopenia was diagnosed, and the prophylactic antithrombotic therapy initiated at admission was temporarily withheld. Multiple studies for infectious agents returned negative results. The patient's status continued to deteriorate with the development of respiratory failure, renal failure, and ischemic hepatitis ("shock liver"). Sedation was discontinued, and 48 hours later, with the patient still unresponsive, comfort measures were instituted. The patient died on the 10th day of her hospitalization.

Acute and Evolving MRI of High-Altitude Cerebral Edema: Microbleeds, Edema, and Pathophysiology

“…Whether these MBs in nonfatal HACE relate to microhemorrhages reported in postmortem examinations 7 is unknown, though similar-sized microhemorrhages in other conditions were clearly seen on gross pathology. 8 As expected, MBs were more easily detected with higher magnetic strength and SWI. 9 The distribution and extent of microbleeds we describe may be distinct for severe HACE.…”

Neuroimaging in Sickle Cell Disease: A Review

“…free fatty acids, among other proinflammatory cytokines, causes endothelial damage, disruption of the blood-brain barrier, and ultimately microhemorrhages. [92][93][94] Clinical presentation is usually with respiratory distress, focal neurological deficits, altered mental status and, most specifically, a petechial rash.…”

Cerebral Fat Embolism in Hemoglobin SC Disease

“…3,4,14 Diffusion-weighted MRI (showing hypersignal) and susceptibility-weighted MRI enhance the specificity and sensitivity. 1,3,15 Gibbs et al 16 performed a necropsy on a similar case showing diffuse microhemorrhages and microinfarcts involving both grayand white-matter structures of the brain, most likely corresponding to the "starfield" pattern. 16…”

“…1,3,15 Gibbs et al 16 performed a necropsy on a similar case showing diffuse microhemorrhages and microinfarcts involving both grayand white-matter structures of the brain, most likely corresponding to the "starfield" pattern. 16…”