May 31, 2019 Conference
Dr. Robert Janigian Jr., Janigian Retina Assoc
A 24 year-old-male with pre-B cell ALL (diagnosed 9 months prior) was referred to our clinic with new onset blurry vision OD. He had previously undergone standard pediatric four-drug induction and was on a maintenance chemotherapy regimen. On examination, vision was 20/30 OD and 20/20 OS with normal IOP and no APD. Slit-lamp biomicroscopy revealed no anterior chamber abnormality and no anterior vitreous cell. On DFE, the right optic nerve exhibited a white, elevated infiltrate with associated peripapillary hemorrhage. The left optic nerve was normal with an otherwise stable retinal exam (Figures 1). Fluorescein angiography revealed late disc leakage of the right eye; left eye was unremarkable (Figure 2). MRI orbits showed no retrobulbar enhancement; CSF and bone marrow studies were negative for blasts. Vitreous biopsy revealed no blasts or immunoglobulin gene rearrangements. Evaluation by ocular oncology at MEEI confirmed leukemic nerve infiltrate and the patient was treated with systemic (cytarabine, asparaginase) and intrathecal (methotrexate, cytarabine, hydrocortisone) chemotherapy as well as optic nerve irradiation OU (800cGy total). Two months after treatment, vision was 20/20 OU without APD or color vision loss. DFE revealed nearly complete regression of nerve infiltrate and peripapillary hemorrhages (Figure 3). The patient is scheduled for bone marrow transplant.
A multidisciplinary approach is crucial in management of ALL patients. Frequent DFEs should be considered standard of care in all ALL patients as many exhibit ocular manifestations of the disorder. In the setting of optic nerve involvement, emergent orbital radiation is warranted to decrease morbidity and mortality.