Orbital compression syndrome (OCS), also referred to in the sickle cell disease (SCD) literature as orbital infarction syndrome, is an uncommon but potentially sight-threatening complication of vaso-occlusive disease. During a vaso-occlusive crisis, sickling of red blood cells obstructs the microvasculature supplying the haematopoietically active marrow of the orbital bones; the resulting ischemic infarction disrupts adjacent small vessels and triggers hemorrhage into the subperiosteal space, producing a subperiosteal hematoma and periorbital edema.1,2 In severe cases, this process may result in optic nerve compression and permanent visual loss.
The condition is clinically important because its presentation can overlap with more common orbital and systemic emergencies, including orbital cellulitis, pre-septal cellulitis, and malaria-related illness.3,4 This overlap creates diagnostic uncertainty, particularly in children with SCD presenting with fever and acute periorbital swelling, and OCS remains rare enough that clinicians outside specialist centers may not readily consider it in the differential diagnosis.4
The diagnostic challenge is greater in malaria-endemic, resource-limited settings, where access to orbital imaging (computed tomography or magnetic resonance imaging) – usually required to confirm subperiosteal hematoma and exclude orbital abscess – and specialist ophthalmic assessment may be limited.4 In such contexts, clinicians must often rely on clinical evaluation while simultaneously considering infectious and non-infectious causes. Published reports have described OCS in children with SCD across various regions, including recent cases from Uganda;1,3,5–7 however, reports from humanitarian refugee settlements in sub-Saharan Africa remain limited.