To report the presenting signs of Retinoblastoma (Rb) in a large cohort of patients who underwent orthoptic assessment at presentation.
METHODSA retrospective medical chart review of 131 patients with retinoblastoma who presented consecutively to a single institution over a 6-year period. The main outcome measure was the presenting sign(s) of the disease.
RESULTSOf 131 Rb patients, 88 presented with unilateral disease and 43 bilateral disease (mean age; 22.7 and 14.8 months respectively). Leukocoria (L) was the presenting sign in 56% of patients, leukocoria and strabismus (LS) in 18%, strabismus (S) in 13%, inflammation (I) in 8%, and 'other' signs in 5%. The fovea was affected by the Rb tumor or its sequelae in 75% of cases. Patients who presented with strabismus were significantly more likely to have foveal involvement than patients who presented with leukocoria alone (P = 0.001). 31% of patients had strabismus as a component of their presentation; 63% had exotropia, 23% had esotropia, and 14% had variable strabismus. The percentage of patients with strabismus rises to 66% if small angle and variable strabismus is also considered. Patients with inflammation had worse ocular survival (P <0.05).
CONCLUSIONSThe combination of leukocoria and strabismus as presenting features of Rb has been assessed. Foveal involvement is common in patients who have strabismus and may influence decision-making regarding globe salvage. We have confirmed that exotropia is more common than esotropia in Rb in the largest cohort to have undergone an orthoptic assessment.