title: Retinal detachment in a child as the first sign of leukemic relapse: histopathology, MRI findings, treatment, and tumor-free follow up.
abstract: 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.
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002675:Child, Preschool; D015353:Eye Enucleation; D005500:Follow-Up Studies; D005901:Glaucoma / Q000209:etiology; D006801:Humans; D007278:Injections, Spinal; D017254:Leukemic Infiltration / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D009900:Optic Nerve / Q000473:pathology; D010146:Pain / Q000209:etiology; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D018714:Radiotherapy, Adjuvant; D012008:Recurrence; D012160:Retina / Q000473:pathology; D012163:Retinal Detachment / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy