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  • Title: Strabismus as a Presenting Sign in Retinoblastoma.
    Author: Kiernan M, Fabian ID, Smith V, Sagoo MS, Reddy MA.
    Journal: J Pediatr Ophthalmol Strabismus; 2021; 58(5):324-330. PubMed ID: 34592118.
    Abstract:
    PURPOSE: To report the presenting signs of retinoblastoma in a large cohort of patients who underwent orthoptic assessment at presentation. METHODS: A retrospective medical chart review was conducted on 131 patients with retinoblastoma who presented consecutively to a single institution during a 6-year period. The main outcome measure was the presenting sign(s) of the disease. RESULTS: Of 131 patients with retinoblastoma, 88 presented with unilateral disease and 43 presented with bilateral disease (mean ages: 22.7 and 14.8 months, respectively). Leukocoria was the presenting sign in 56% of patients, leukocoria and strabismus in 18%, strabismus in 13%, inflammation in 8%, and "other" signs in 5%. The fovea was affected by the retinoblastoma tumor or its sequelae in 75% of patients. Patients who presented with strabismus were significantly more likely to have foveal involvement than patients who presented with leukocoria alone (P = .001). Thirty-one percent 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 increased to 66% when small angle and variable strabismus were also considered. Patients with inflammation had worse ocular survival (P < .05). CONCLUSIONS: This study assessed the combination of leukocoria and strabismus as presenting features of retinoblastoma. Foveal involvement is common in patients who have strabismus and may influence decision-making regarding globe salvage. The authors confirmed that exotropia is more common than esotropia in retinoblastoma in the largest cohort to have undergone an orthoptic assessment. [J Pediatr Ophthalmol Strabismus. 2021;58(5):324-330.].
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