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

• Published in: Journal of pediatric ophthalmology and strabismus Vol. 32; no. 4; p. 253
• Main Authors: Primack, J D, Smith, M E, Tychsen, L
• Format: Journal Article
• Language: English
• Published: United States 01-07-1995
• Subjects: Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Child, Preschool; Eye Enucleation; Follow-Up Studies; Glaucoma - etiology; Humans; Injections, Spinal; Leukemic Infiltration - pathology; Magnetic Resonance Imaging; Male; Optic Nerve - pathology; Pain - etiology; Precursor Cell Lymphoblastic Leukemia-Lymphoma - complications; Precursor Cell Lymphoblastic Leukemia-Lymphoma - diagnosis; Precursor Cell Lymphoblastic Leukemia-Lymphoma - therapy; Radiotherapy, Adjuvant; Recurrence; Retina - pathology; Retinal Detachment - diagnosis; Retinal Detachment - etiology; Retinal Detachment - therapy
• ISSN: 0191-3913
• DOI: 10.3928/0191-3913-19950701-13

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.