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Strabismus as a Presenting Sign in Retinoblastoma

Kiernan, M; Fabian, ID; Smith, V; Sagoo, MS; Reddy, MA; (2021) Strabismus as a Presenting Sign in Retinoblastoma. Journal of Pediatric Ophthalmology and Strabismus , 58 (5) pp. 324-330. 10.3928/01913913-20210614-03. Green open access

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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.

Type: Article
Title: Strabismus as a Presenting Sign in Retinoblastoma
Open access status: An open access version is available from UCL Discovery
DOI: 10.3928/01913913-20210614-03
Publisher version: https://doi.org/10.3928/01913913-20210614-03
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/10137862
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