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Washington University Experience | VASCULAR | Sickle cell anemia | 3A0 Case 3 History
Case 3 History ---- The patient was a 39-year-old African-American woman with a history of sickle cell disease, frequent sickle cell pain crises, chronic pain, iron overload disease, methicillin-resistant Staphylococcal aureus endocarditis of the tricuspid valve in 2006, prior upper extremity deep venous thrombosis, asthma, and a recent diagnosis of uveitis, on high dose steroids. She presented with increasing pain in her joints and chest for one day, concerning for sickle cell crisis. One day prior to admission, she developed sudden onset of severe sharp pain in her ankles, knees, hips, lower back, elbows, and chest, exacerbated by movement. She reported compliance with her medications, including chronic narcotics (methadone and oxycodone) which did not relieve her pain. On the morning of 1/30 she was noted to be confused, and had a non-focal neurological exam, attributed to opioids. At approximately noon, the medicine floor nurse witnessed the patient become acutely non-responsive. There were no obvious focal deficits on exam at that time. On arrival to the ICU she was noted to have a newly “blown pupil” on the right side. CT was notable for a left-sided intraparenchymal hemorrhage, intraventricular hemorrhage, rightward midline shift, subfalcine herniation, and uncal herniation. Neurosurgery was consulted; based on the location, amount, and type of hemorrhage, assessment suggested no mortality benefit from hematoma evacuation. Therefore, medical management was employed. However, the patient's heart rate became increasingly bradycardic with a widened QRS on cardiac monitors. The patient's family was informed of the changes in clinical status and was present at the bedside. The family decision was made not to perform cardiopulmonary resuscitation or intensify cardiac support. The patient was pronounced dead on 1/30. ---- At autopsy the weight of the unfixed brain was 1420g. The pathologic findings included a left cerebral intraparenchymal bleed with intraventricular, subarachnoid and subdural extension. The hemorrhages resulted in uncal, subfalcine and brainstem herniation. The microvasculature contained numerous sickled cells and acute/subacute microinfarcts and modest focal perivascular hemosiderin deposits.
