Retinal detachment in a child as the first sign of leukemic relapse: Histopathology, MRI findings, treatment, and tumor-free follow up

Improved leukemia therapies in children have brought about prolonged remissions with extramedullary relapses being reported in sites other than the most common (bone marrow, testes, brain, and spinal cord). A 3- 1/2 year- old boy with a history of acute lymphocytic leukemia presented with total retinal detachment in one eye. Painful glaucoma unresponsive to medical therapy necessitated enucleation. Histopathologic examination documented the presence of a dense leukemic cellular infiltrate replacing a totally detached, necrotic retina. Tumor cells also were present in the optic nerve. The child had remained free of leukemia for 3 years after systemic and intrathecal chemotherapy, supplemented by craniospinal radiation. This represents the first case of relapse of acute lymphocytic leukemia presenting solely as a retinal detachment. Our case also underscores the point that the treatment of leukemia after an isolated ocular relapse can be associated with a favorable outcome.