Abstract
Sickle cell disease is a hemoglobinopathy that results in paroxysmal vascular occlusion and tissue infarction that can manifest in a plurality of tissues. Vasoocclusive crises in sickle cell disease commonly involve bone marrow of the long bones and vertebrae. Involvement of bones with less marrow space, including the bones of the orbit, is reported rarely in the literature and can closely mimic orbital cellulitis, both clinically and radiologically. The present case is a 3 years old boy, a known case of sickle cell disease, who presented with what was thought to be orbital cellulitis and was treated accordingly. Subtle radiologic features of sickle cell orbitopathy were initially overlooked, resulting in an incorrect diagnosis and a treatment delay for the patient. Correctly treated most cases resolve with no adverse effects. This case highlights the importance of maintaining a high index of suspicion in patients with known sickle cell disease, even when the presentation is not classic.
Highlights
Sickle cell disease is a hemoglobinopathy that results in paroxysmal vascular occlusion and tissue infarction that can manifest in a plurality of tissues
Sickle cell disease (SCD) is a hemoglobinopathy characterized by chronic hemolytic anemia and vaso-occlusive crises
The disorder is inherited in autosomal fashion, and the patients that typically experience clinical manifestations are homozygous for the sickle cell β globin mutation (HbSS) or compound heterozygotes with 1 sickle cell allele and an abnormality in the other allele that disallows adequate transcription of normal β chain
Summary
Sickle cell disease (SCD) is a hemoglobinopathy characterized by chronic hemolytic anemia and vaso-occlusive crises. The most frequent complications requiring hospital admissions for patients with sickle cell disease are painful vaso-occlusive crises (Table 1) [2]. The pathogenesis of the microvascular occlusion, the hallmark of the painful sickle cell crisis, is complex involving activation and adhesion of leucocytes, platelets and endothelial cells as well as hemoglobin S-containing erythrocytes. While this process can occur in virtually any organ, it is common in the bone marrow, resulting in bone marrow infarction typically in the medullary cavity or epiphyses. Authors are presenting such a case of orbital bone infarction and osteomyelitis in a 3-year-old boy with sickle cell disease, managed successfully with blood transfusion and intravenous antibiotics only, without steroid or surgical intervention
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