Orbital Compression Syndrome Mimicking Severe Malaria in a Child with Sickle Cell Disease in a Ugandan Refugee Settlement: A Case Report.

Abstract

Background: Orbital compression syndrome (OCS) is a rare, sight-threatening complication of sickle cell disease (SCD), caused by vaso-occlusive infarction of orbital bone marrow with subperiosteal hematoma formation. Its presentation overlaps with orbital cellulitis and severe malaria, creating diagnostic uncertainty in resource-limited humanitarian settings.

Case presentation: A 9-year-old boy with homozygous SCD (HbSS) presented to a primary health facility in Palabek Kal refugee settlement, Lamwo District, northern Uganda, with a 1-day history of sudden severe bilateral frontal headache radiating to the eyes, high-grade fever, and rapidly progressive bilateral periorbital edema, epiphora, and photophobia, without altered consciousness, seizures, vomiting, or respiratory distress. He was febrile (39.3°C) and unwell, with bilateral periorbital swelling, mild scleral icterus, and hepatomegaly (approximately 6 cm below the right costal margin); visual acuity, pupillary responses, extraocular movements, and proptosis assessment were normal. Laboratory evaluation showed severe anemia (hemoglobin 6.5 g/dL), leukocytosis (14.81 × 10³/µL), and 3+ malaria parasitemia. Orbital imaging and cultures were unavailable. The clinical picture was most consistent with OCS secondary to vaso-occlusive crisis, with severe malaria, orbital cellulitis, and septicemia considered as concurrent or alternative diagnoses.

Management and outcome: The child received intravenous artesunate, empirical broad-spectrum antibiotics, intravenous fluids, analgesics, and one unit of blood transfusion for severe anemia, with close ophthalmic monitoring. Fever resolved, periorbital swelling subsided, and visual function was preserved without surgical intervention.

Conclusion: This case illustrates the diagnostic challenge of OCS in a child with SCD in a malaria-endemic humanitarian setting. OCS should be considered in any child with SCD presenting with acute bilateral periorbital swelling, headache, and fever, even when malaria is confirmed and orbital imaging is unavailable. Early recognition, parallel management of competing diagnoses, close clinical monitoring, and expanded access to orbital imaging in humanitarian settings are critical to prevent avoidable visual morbidity.