Bilateral orbital infarction and retinal detachment in a previously undiagnosed sickle cell hemoglobinopathy African child
An 11-year-old girl presented with 4 days history of fever, headache, generalized body weakness, and 1 day history of bilateral painful proptosis associated with sudden loss of vision and purulent discharge. She had no nasal discharge, blockage, dental pain or dental procedure. There was no history of trauma, previous bone pains or frequent illness. Her genotype was not known; she is the fourth of seven children and a 3-year-old sibling died following fever and severe anemia. She had lived all her life in a rural community with both parents who are farmers and genotypes unknown.\nShe was conscious but lethargic, pale, afebrile, mildly dehydrated with bilateral sub-mandibular, and sub-mental tender lymph node enlargement. She had tender hepatomegaly and splenomegaly. Visual acuity was no light perception in both eyes with marked tender periorbital swelling, axial, non-pulsatile proptosis of 23.5 mm and 23 mm on the right and left respectively []. There were no extraocular muscle movements and inferior prolapsing chemosis; the right corneal was hazy with turbid anterior chamber, round and unreactive pupil, clear lens, and a retrolental greyish reflex. The cornea was clear on the left, with clear anterior chamber, small, unreactive pupil, clear lens, and a retrolental glow. Fundal view was precluded by the retrolental reflex in both eyes. Intraocular pressure was 16 mm Hg in each eye. Initial diagnosis of bilateral orbital cellulitis to rule out cavernous sinus thrombosis was made; she was investigated and commenced on broad spectrum intravenous and topical antibiotics empirically as in-patient. Ocular ultrasound of bilateral proptosis, bullous retinal detachment, and subretinal fluid collection with no orbital collection or mass [], Cranial computed tomographyscan exclusion of space occupying lesion, orbital collections or intracranial extension [] as well as Hemoglobin genotype result of HbSS lead to diagnosis of bilateral severe orbital infarction in a sickle cell disease. Management with systemic/topical steroid, intravenous fluids, analgesics as well as cycloplegics produced sustained progressive reduction in proptosis, chemosis and improved general health although, her vision remained no perception of light in both eyes. She was discharged on oral medications and tapering steroid dose after 2 weeks to continue out-patient management. Patients have since, defaulted preventing repeat ocular scan and follow-up evaluation.