Purpose

To report the case of a pediatric patient with isolated intraocular relapse of B-Cell ALL (Acute lymphoblastic leukemia) initially presenting as bilateral ciliary body masses.

Methods

A 7-year-old female with underlying B-Cell ALL in remission presented with a three-week history of right eye redness and photophobia. Complete ophthalmologic & systemic examination were performed.

Results

The right eye had decreased visual acuity of 20/167 and slit-lamp biomicroscopy revealed a bedewed cornea, hyphema, shallow anterior chamber, anterior bowing of the iris, and increased intraocular pressure at 38 mmHg. The left eye was noted to have fine, non-pigmented keratic precipitates, anterior chamber cells 1+, and anterior bowing of the temporal iris after one month. Ultrasound biomicroscopy revealed large ciliary body masses on all clock hours of both eyes. Bone marrow and cerebrospinal fluid were negative for leukemic cells which strongly suggested the clinical diagnosis of isolated ocular relapse. Remission reinduction chemotherapy was initiated resulting in clinical improvement. On repeat ultrasound biomicroscopy after chemotherapy, the ciliary body masses previously seen were no longer appreciated.

Conclusions

Ocular relapse in ALL is uncommon, comprising 0.5%-2.5% of leukemic relapse. Presence of ophthalmologic manifestations is considered a marker of poor prognosis due to a higher incidence of central nervous system leukemia. Ocular infiltration may be the only manifestation of relapse hence the importance of having a high index of suspicion when a known case of ALL presents with ocular symptoms. Prompt diagnosis and initiation of treatment produce more favorable ophthalmologic and systemic outcomes, which have an impact on the prognosis of patients with ALL relapse.