双眼异时性视网膜母细胞瘤21例临床分析

Clinical analysis of 21 cases with metachronous bilateral retinoblastomas

目的通过分析双眼异时性视网膜母细胞瘤(RB)患儿的临床资料,确定高危因素,制订合理的随访计划,从而使肿瘤得以早期发现、早期治疗。方法回顾性病例分析。收集2014年12月至2019年3月北京儿童医院收治的第一眼确诊为RB后,第二眼发现新发RB的病例共21例,分析双眼确诊间隔时间、发病年龄、首发症状,以及第二眼的确诊方法与肿瘤分期(IIRC)关系等。眼底检查以全身麻醉下间接眼底镜联合RetCam作为常规检查。结果第一眼发病时患儿的年龄为1~36个月,平均12.2个月,13例年龄≤12个月;第二眼发病年龄为3~84个月,平均25.7个月,19例年龄≤36个月;双眼发病间隔时间为1~48个月,平均13.7个月。第二眼有首发症状者4例,17例(81.0%)在随诊例行检查时发现,无临床症状。第二眼肿瘤分期A期8例,B期4例,C期1例,D期6例,E期2例。第二眼肿瘤以全身麻醉下间接眼底镜联合RetCam检查发现为主,患者多为早期。结论第一眼RB发病年龄≤12个月是第二眼发生RB的高危因素,双眼确诊的间隔时间多≤36个月;定期、规律随诊和全身麻醉下间接眼底镜联合RetCam检查是早期发现第二眼RB的有效方法。

Objective To identify the risk factors for the development of metachronous bilateral retinoblastoma(MBRB)and make a reasonable follow-up schedule for early detection and treatment by collecting clinical data of MBRB children.Methods In the retrospective analysis of clinical records,21 cases diagnosed with retinoblastoma in one eye and new RB in fellow eye were included at the Department of Ophthalmology in Beijing Children’s Hospital from December 2014 to March 2019.Interval between definite diagnosis,age of onset and initial symptoms of both eyes were analyzed;correlation of diagnosis means with neoplasm staging was analyzed.Results The ages at diagnosis of retinoblastoma in the first eye ranged from 1 to 36 months with an average of 12.2 months,and 13 cases were under 12 months.The ages at later diagnosis of MBRB ranged from 3 to 84 months with an average of 25.7 months,and 19 cases were under 36 months.The latency periods ranged from 1 to 48 months with an average of 13.7 months.There were 4 cases developed initial manifestation in fellow eye,and 17(81.0%)cases without clinical presentations were diagnosed in routing fundus examination.Neoplasm staging in the fellow eye 8 cases at stage A,4 cases at stage B,1 case at stage C,6 cases at stage D and 2 cases at stage E.Indirect fundoscopy under general anesthesia with RetCam was the regular ophthalmological follow-up methods for the fellow eye,and the patient was mainly at early stage.Conclusion Those patients who were diagnosed with unilateral retinoblastoma within 12 months are at great risk of developing MBRB and the most latency periods are within 36 months after the initial diagnosis.Regular follow-up,indirect fundoscopy under general anesthesia with RetCam are effective methods for early diagnosis of retinoblastoma in the fellow eye.