Orbital compression syndrome in sickle cell disease

Orbital complications in sickle cell disease are uncommon, but can be severe enough to result in significant morbidity. We report a 10-year-old boy with sickle cell disease who presented with fever, bilateral eyelid edema, proptosis, and diminished vision with left eye involvement more than the right eye. Investigations revealed anemia, thrombocytopenia, and derangement of coagulation profile consistent with disseminated intravascular coagulopathy, and salmonella species was recovered from blood culture. MRI of the orbits showed bilateral large subperiosteal hematomas. The treatment included intravenous antibiotics, pulse methylprednisolone and bilateral canthotomy with surgical drainage of the hematomas. Postoperative visual assessment revealed complete loss of vision in the left eye with normal vision in right eye. This case highlights the importance of the early evaluation and consideration of surgical intervention in sickle cell disease with this rare complication.