Diagnostic perplexities in acute critical illness related to sickle cell crisis complications
Rezik M, Elbanna A, and Jayaprakash N. Diagnostic perplexities in acute critical illness related to sickle cell crisis complications. Crit Care Med 2019; 47(1).
Crit Care Med
Learning Objectives: Sickle cell disease is commonly associated with pain crisis which in rare instances can result in bone necrosis and fat embolism syndrome. Herein, we report a case of multi-organ failure in a 19-year-old male with HbSS disease and how he survived his critical illness. Methods: A 19-year-old male with sickle cell disease presented following a seizure at home following a typical bone pain crisis. He was admitted to the intensive care unit with altered mental status and hyponatremia and given antimicrobials for suspected meningitis. On day 2, he rapidly deteriorated with shock, acute RV failure, acute bilateral subdural hemorrhage (despite negative CT head and CT angiography on admission), multi-organ failure, pancytopenia, and rhabdomyolysis. His LDH rapidly rose and he even developed a spontaneous pneumothorax. Multidisciplinary discussions involving critical care, hematology and transfusion medicine included discussions regarding sickle crisis with multiorgan failure and indications for plasma exchange. The risk of coagulopathy without clear benefit in multi-organ failure led the team to continue supportive critical care management. Eventually his mental status improved and a repeat MRI later in his hospital stay following extubation revealed findings consistent with fat emboli syndrome. In hindsight, the diagnosis of bone marrow necrosis and fat emboli syndrome resulting in multi-organ failure was clear. Three months following his critical illness our patient has returned to work, has complete functional recovery and is enrolling in college. Results: Bone marrow necrosis and fat embolism syndrome is an exceptionally rare but potentially deadly complication of sickle cell disease. A 2014 systematic review of 58 cases identified a 65% mortality rate with most diagnoses being made at autopsy. Our case highlights the diagnostic perplexity and complexities of managing such an acute critical illness. Fat embolism syndrome should be considered in patients who present with bone crisis and acutely deteriorate with multi-organ failure. Support intensive care including a multi-disciplinary team fortunately resulted in a positive outcome in this young man.