Objective: To investigate the characteristics of initial ocular symptoms in children with optic pathway glioma (OPG) at different age stages. Methods: A retrospective case series study was conducted. Clinical data of 16 children with OPG who were diagnosed and treated in the Department of Ophthalmology, Beijing Children's Hospital, Capital Medical University from April 2017 to July 2021 were collected. The initial ocular symptoms, clinical manifestations of the eyes and nervous system, imaging and histopathological features were analyzed, and the differences in initial ocular symptoms between infants and young children aged≤36 months and older children aged>36 months were compared. Results: Of all 16 children included, 9 were male and 7 were female. The onset age was 15.0 (6.3, 56.5) months, and the diagnosis age was 48.0 (11.3, 78.0) months. There were 11 infants and young children, and 5 older children. Eye symptoms were the initial manifestation in 11 cases, including 8 cases of infants and young children (5 cases presented with irregular nystagmus, 2 cases with strabismus, and 1 case with failure to chase visual target), and 3 cases of older children (2 cases presented with decreased vision and 1 case with optic disc edema). The imaging findings showed that optic chiasm-involved OPG accounted for the highest proportion, with 3 cases in older children and 8 cases in infants and young children. Among the 8 children with optic chiasm-involved OPG who underwent surgical treatment, 5 were diagnosed with pilocytic astrocytoma according to histopathological results. Seven children had useful vision saved after treatment, and 1 child had visual loss accompanied by cognitive impairment due to surgery after 4 years of nystagmus. Conclusion: OPG in children often manifests as initial ocular symptoms, with irregular nystagmus being more common in infants and young children, and vision loss being the main symptom in older children.
目的： 探讨不同年龄阶段儿童视路胶质瘤眼部首发症状的特征。 方法： 回顾性病例系列研究。收集2017年4月至2021年7月就诊于首都医科大学附属北京儿童医院眼科的16例视路胶质瘤患儿的临床资料。分析首发症状、眼部及神经系统临床表现、影像学及组织病理学特点，并比较年龄≤36个月的婴幼儿和年龄>36个月的大龄儿童患儿眼部首发症状的区别。 结果： 16例患儿中男性9例，女性7例；发病年龄为15.0（6.3，56.5）个月，确诊年龄为48.0（11.3，78.0）个月。其中婴幼儿患儿11例，大龄儿童患儿5例。因眼部为首发症状而被确诊11例，其中婴幼儿患儿8例（5例表现为不规则的眼球震颤，2例表现为斜视，1例表现为不追视），大龄儿童患儿3例（2例表现为视力下降，1例表现为视盘水肿）。影像学表现以累及视交叉的视路胶质瘤占比最高，大龄儿童患儿和婴幼儿患儿分别为3例（3/5）和8例（8/11）。8例累及视交叉的视路胶质瘤患儿行手术治疗，其中5例组织病理结果提示毛细胞型星形细胞瘤。7例预后保存了有用视力；1例眼部出现震颤4年后，因出现头痛、呕吐等神经系统症状而行手术治疗，预后视力丧失伴有认知障碍。 结论： 儿童视路胶质瘤多以眼部异常为首发症状，婴幼儿以眼球不规则震颤多见，大龄儿童以视力下降为主。.