Wang Nan, Liu Rui, Li Jing, Wang Jinjin, Xu Liangyuan, Guo Qihan, Zhang Xuan, Ma Jianmin
Beijing Ophthalmology and Visual Sciences Key Laboratory, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
Tai'an City Central Hospital, Qingdao University, Tai'an, China.
Front Neurol. 2022 Oct 10;13:1013523. doi: 10.3389/fneur.2022.1013523. eCollection 2022.
This study aimed to observe the long-term effect of enucleation combined with primary orbital implantation in children with histopathologic optic nerve invasive retinoblastoma (RB).
We retrospectively analyzed the clinical data and outcomes of children with RB optic nerve invasion confirmed by histopathology who underwent enucleation combined with primary orbital implantation between March 2010 and April 2014 in Beijing Tongren Hospital. The follow-up time ranged from 81 to 129 months, with a mean follow-up of 96 ± 14 months.
A total of 59 children were included in this study. There were 32 males and 27 females; 52 children were affected in one eye and seven children in both eyes. The time from onset of symptoms to visit was between 3 days and 16 months, with a mean of 2.2 ± 2.8 months. The age at the of surgery was between 2 and 65 months, with an average of 24 ± 13 months. Patients were classified based on the degree of optic nerve invasion into four grades: grade 1 (invasion of prelaminar) in 28 cases, grade 2 (invasion of laminar) in 14 cases, grade 3 (invasion of retrolaminar but not reaching the optic nerve transection) in 16 cases, and grade 4 (invasion of the optic nerve transection) in 1 case. Post-operatively, all children were treated with 0-9 cycles of intravenous chemotherapy based on histopathology results from the removed eye. Of the 59 children, 13 had postoperative complications, and one died from recurrence. The survival rate was 98% (58/59). There was one case of orbital implant exposure, one of orbital cellulitis, six of enophthalmos and superior sulcus deformity, two of blepharoptosis, one of granuloma complicated with blepharoptosis, and one with a subconjunctival cyst.
For children with RB histopathologic invasion of the optic nerve, enucleation combined with primary orbital implantation reveals future potential treatment options when combined with a full course of intravenous chemotherapy.
本研究旨在观察眼球摘除联合一期眼眶植入术对组织病理学确诊视神经受侵的儿童视网膜母细胞瘤（RB）的长期疗效。
回顾性分析2010年3月至2014年4月在北京同仁医院接受眼球摘除联合一期眼眶植入术且经组织病理学确诊视神经受侵的RB患儿的临床资料及治疗结果。随访时间为81至129个月，平均随访时间为96±14个月。
本研究共纳入59例患儿。其中男性32例，女性27例；单眼受累52例，双眼受累7例。症状出现至就诊时间为3天至16个月，平均为2.2±2.8个月。手术年龄为2至65个月，平均为24±13个月。根据视神经受侵程度将患者分为四级：1级（筛板前受侵）28例，2级（筛板受侵）14例，3级（筛板后受侵但未达视神经横断处）16例，4级（视神经横断处受侵）1例。术后，所有患儿根据摘除眼球的组织病理学结果接受0至9个周期的静脉化疗。59例患儿中，13例出现术后并发症，1例因复发死亡。生存率为98%（58/59）。出现1例眼眶植入物暴露、1例眼眶蜂窝织炎、6例眼球内陷及眶上裂畸形、2例上睑下垂、1例肉芽肿合并上睑下垂、1例结膜下囊肿。
对于组织病理学确诊视神经受侵的RB患儿，眼球摘除联合一期眼眶植入术联合全程静脉化疗显示出未来潜在的治疗选择。
