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.].

中文翻译：

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斜视作为视网膜母细胞瘤的一个呈现标志。

目的 报告一大群在就诊时接受矫形检查的患者中视网膜母细胞瘤的表现体征。方法 对 131 名视网膜母细胞瘤患者进行了回顾性病历审查，这些患者在 6 年内连续就诊于一个机构。主要结局指标是疾病的表现体征。结果 131 例视网膜母细胞瘤患者中，88 例单侧病变，43 例双侧病变（平均年龄分别为 22.7 和 14.8 个月）。白瞳是 56% 的患者的表现体征，白瞳和斜视占 18%，斜视占 13%，炎症占 8%，“其他”征象占 5%。75% 的患者中央凹受视网膜母细胞瘤肿瘤或其后遗症的影响。与仅出现白瞳症的患者相比，出现斜视的患者更容易出现中央凹受累（P = .001）。31% 的患者有斜视作为其表现的一个组成部分；63% 有外斜视，23% 有内斜视，14% 有可变斜视。当同时考虑小角度和可变斜视时，斜视患者的百分比增加到 66%。炎症患者的眼部存活率较差（P < .05）。结论 本研究评估了白瞳症和斜视的组合作为视网膜母细胞瘤的表现特征。中央凹受累在斜视患者中很常见，可能会影响眼球抢救的决策。作者证实，在接受过正视评估的最大队列的视网膜母细胞瘤中，外斜视比内斜视更常见。[J Pediatr Ophthalmol 斜视。2021;58(5):324-330.]。