Acute lymphoblastic leukemia relapse presenting with optic nerve infiltration

Abstract

Acute lymphoblastic leukemia is the most common childhood malignancy. Despite many advances in therapy, about 15%-20% of children with acute lymphoblastic leukemia experience a disease relapse. Isolated ocular relapse is relatively rare. A 14-year-old male with T-cell acute lymphoblastic leukemia in remission presented with sudden onset of right eye pain and visual acuity impairment. Fundoscopic examination of the eye and magnetic resonance imaging of the orbits were consistent with optic nerve infiltration. The patient was treated with salvage chemotherapy, orbital radiation and eventual bone marrow transplantation, with notable improvement in vision and regression of retinal and optic nerve findings. Optic nerve infiltration represents an ophthalmic emergency and requires urgent management. The use of radiation therapy is a helpful adjunct with systemic chemotherapy in obtaining disease remission.

Keywords: Acute lymphoblastic leukemia; optic nerve infiltration; radiation therapy; salvage treatment.

Figure 1.

(a) MRI T1-weighted brain and orbits. Surrounding the right optic nerve, pronounced gadolinium uptake can be seen. (b) MRI orbit scan repeated after completion of therapy. The right optic nerve is no longer thickened.

Figure 2.

(a) Fundus photographs at initial presentation. Right eye and left eye, respectively. The left eye was normal, while the right showed significant disk edema, tortuosity of the retinal vessels and hemorrhages. (b) Fundus photographs of the right and left eye, 3 months following bone marrow transplant. Resolution of disk edema can be noted.