误诊行玻璃体切割手术的视网膜母细胞瘤临床处理

Management of patients with retinoblastoma treated with vitrectomy due to misdiagnosis

目的初步探讨视网膜母细胞瘤(RB)因误诊行玻璃体切割手术(PPV)治疗后临床处理方法。方法回顾性病例研究。2015年7月至2018年7月于北京同仁医院眼科中心病理检查确诊的RB患儿5例5只眼纳入研究。其中,男性3例3只眼,女性2例2只眼;均为单眼患病。平均年龄(4.8±1.7)岁。首次就诊诊断为眼内炎2只眼(40%,2/5);玻璃体积血3只眼(60%,3/5)。均行PPV治疗。患儿均行裂隙灯显微镜、眼眶核磁共振成像和CT、眼彩色多普勒超声血流成像检查。无明确眼外蔓延者行眼球摘除联合义眼台植入手术;有明确眼外蔓延者行保留部分眼睑的改良眶内容物剜除手术。手术后平均随访时间(34.6±7.9)个月。结果5只眼中,行眼球摘除联合Ⅰ期义眼台植入手术2只眼(40%,2/5);改良眶内容物剜除手术3只眼(60%,3/5)。内生型2只眼(40%,2/5);弥漫浸润型1只眼(20%,1/5);混合型2只眼(40%,2/5)。眶内蔓延3只眼,肿瘤侵犯视神经1只眼,区域淋巴结转移2只眼。所有患儿均行全身化学药物治疗(化疗)。随访期间中无新发转移性疾病发生,无死亡病例。结论RB误诊行PPV后应尽早手术治疗,无明确眼外蔓延行眼球摘除或联合Ⅰ期义眼台植入术,有明确眼外蔓延行需要行眶内容物剜除手术;手术后均应联合化疗。

Objective To explore the clinical treatment of retinoblastoma(RB)after being treated with vitrectomy(PPV)due to misdiagnosis.Methods A retrospective case study.From July 2015 to July 2018,5 cases and 5 eyes of RB children diagnosed by pathological examination at the Eye Center of Beijing Tongren Hospital were included in the study.Among them,there were 3 males with 3 eyes and 2 females with 2 eyes;all of them had monocular disease.The average age was 4.8±1.7 years old.At the first visit,the diagnosis was endophthalmitis in 2 eyes(40%,2/5);vitreous hemorrhage in 3 eyes(60%,3/5).All were treated with PPV.All children underwent slit lamp microscopy,orbital MRI and CT,and eye color Doppler ultrasound blood flow imaging.If there was no clear extraocular spread,the eyeball removal combined with artificial orbital implantation was performed;if there was clear extraocular spread,the modified orbital content enucleation operation was performed with part of the eyelid preserved.The average follow-up time after surgery was 34.6±7.9 months.Results Among the 5 eyes,2 eyes(40%,2/5)underwent eyeball enucleation combined with stage I artificial orbital implantation,and 3 eyes(60%,3/5)with modified orbital content enucleation.There were 2 eyes of endogenous type(40%,2/5),1 eye of diffuse infiltration type(20%,1/5),and 2 eyes of mixed type(40%,2/5).The orbit spread in 3 eyes,the tumor invaded the optic nerve in 1 eye,and regional lymph node metastasis in 2 eyes.All children received systemic chemical therapy(chemotherapy).During the follow-up period,there were no new metastatic diseases and no deaths.Conclusions After RB misdiagnosis and PPV,surgical treatment should be performed as soon as possible.If there is no clear extraocular spread,eyeball removal or combined stage I orbital implantation should be performed.If there is clear extraocular spread,the orbital contents should be enucleated;Chemotherapy should be combined after surgery.