Acute Orbitopathy Manifesting as Periorbital Cellulitis in Sickle Cell Disease

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.

Isolated Unilateral Orbital Compression Syndrome in A 19-Year-Old Male With Homozygous Sickle Cell Disease

“…Although orbital involvement in sickle cell disease is rare, when it does occur, it could even be complicated by orbital compression syndrome; this develops when infarction of the microvasculature in the sphenoid bone marrow leads to inflammation and necrosis, which in turn cause subperiosteal hemorrhage [ 9 ]. This is either managed medically or surgically whenever there is optic nerve dysfunction or large hematomas [ 10 ].…”