Abstract
Purpose :
To describe the fundamental features of children who presented with strabismus as their initial sign of retinoblastoma.
Methods :
We performed a retrospective review and analysis of the medical records of consecutive retinoblastoma patients from multiple centers spanning the period between 2006 and 2023. We specifically analyzed the characteristics of patients whose initial presenting symptom was strabismus.
Results :
Among the 3457 children diagnosed with retinoblastoma, 416 patients (545 eyes) presented with strabismus as their initial symptom (12%), including 211 male (50.7%) and 205 female (49.3%). Out of the 416 patients, 287 (69%) presented with unilateral tumors, while 129 (31%) presented with bilateral tumors. By the end of the follow-up period, 312 patients (92.3%) had survived, 26 (7.7%) had died, and 78 were lost to follow-up. Based on the International Retinoblastoma Classification Committee (IRCC) criteria, the tumors were staged as follows: A (n = 9 [1.7%]), B (n = 32 [5.9%]), C (n = 36 [6.6%]), D (n = 295 [54.1%]), and E (n = 155 [28.4%]). Additionally, the stage of 18 patients (3.3%) was unknown. Out of the 545 eyes, 243 were salvaged (44.6%), while 230 (42.2%) underwent enucleation, with 112 (20.6%) undergoing primary enucleation and 118 (21.7%) undergoing secondary enucleation. The average lag time between the onset of strabismus and diagnosis was 5.2 months, with a median duration of 3.5 months (<1 – 45.5).
Conclusions :
Our study reaffirms that strabismus is the second most common initial symptom observed in retinoblastoma patients. A significant number of these patients were diagnosed at an advanced stage, primarily due to delays in diagnosis. Therefore, it is crucial to implement careful fundus screenings for children presenting with strabismus, as early detection plays a pivotal role in improving outcomes and treatment strategies for retinoblastoma.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.