A 19-year-old black man with a three-year history of sickle cell disease was admitted to our hospital with shortness of breath and severe abdominal and joint pain consistent with a sickle cell crisis. Figure 1 shows the acute and chronic effects of this disease. Three days after admission, a space-occupying epidural hematoma developed, most likely caused by expansion of hematopoietic tissue and disruption of the bony margins with bleeding into the epidural space. Despite an emergency craniotomy with surgical removal of the hematoma, his postoperative course was complicated, including cerebral edema, a midline shift, and supra- and infratentorial herniation. Because of the hemolytic crisis, a full blood-exchange transfusion was done, and therapy with norepinephrine, cortisol and terlipressin was started. Six days later, renal insufficiency with hyperkalemia developed, and the patient died.
Sickle cell disease is an autosomal recessive blood disorder that affects about 0.2% of African Americans.1 The average life expectancy is 42 years for men with sickle cell disease and 48 years for women. The term “sickle cell crisis” is used to describe several acute conditions, including vaso-occlusion, aplastic crisis, splenic sequestration and hemolysis. Most crises last between five and seven days and can be managed with fluids and analgesia.2,3
Clinical images are chosen because they are particularly intriguing, classic or dramatic. Submissions of clear, appropriately labelled high-resolution images must be accompanied by a figure caption and the patient’s written consent for publication. A brief explanation (250 words maximum) of the educational significance of the images with minimal references is required.
Footnotes
-
Competing interests: None declared.
-
This article has been peer reviewed.