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儿童颅咽管瘤的长期视觉预后。

Long-term visual outcomes of craniopharyngioma in children.

机构信息

Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children and University of Toronto, 555 University Ave., Toronto, ON, M5G 1X8, Canada.

Division of Haematology/Oncology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada.

出版信息

J Neurooncol. 2018 May;137(3):645-651. doi: 10.1007/s11060-018-2762-3. Epub 2018 Jan 17.

Abstract

Visual function is a critical factor in the diagnosis, monitoring, and prognosis of craniopharyngiomas in children. The aim of this study was to report the long-term visual outcomes in a cohort of pediatric patients with craniopharyngioma. The study design is a retrospective chart review of craniopharyngioma patients from a single tertiary-care pediatric hospital. 59 patients were included in the study. Mean age at presentation was 9.4 years old (range 0.7-18.0 years old). The most common presenting features were headache (76%), nausea/vomiting (32%), and vision loss (31%). Median follow-up was 5.2 years (range 1.0-17.2 years). During follow-up, visual decline occurred in 17 patients (29%). On Kaplan Meier survival analysis, 47% of the cases of visual decline occurred within 4 months of diagnosis, with the remaining cases occurring sporadically during follow-up (up to 8 years after diagnosis). In terms of risk factors, younger age at diagnosis, optic nerve edema at presentation, and tumor recurrence were found to have statistically significant associations with visual decline. At final follow-up, 58% of the patients had visual impairment in at least one eye but only 10% were legally blind in both eyes (visual acuity 20/200 or worse or < 20° of visual field). Vision loss is a common presenting symptom of craniopharyngiomas in children. After diagnosis, monitoring vision is important as about 30% of patients will experience significant visual decline. Long-term vision loss occurs in the majority of patients, but severe binocular visual impairment is uncommon.

摘要

视觉功能是儿童颅咽管瘤诊断、监测和预后的关键因素。本研究旨在报告一组颅咽管瘤患儿的长期视觉结果。研究设计是对一家三级儿科医院的颅咽管瘤患者进行回顾性图表审查。本研究纳入了 59 名患者。发病时的平均年龄为 9.4 岁(范围 0.7-18.0 岁)。最常见的表现为头痛(76%)、恶心/呕吐(32%)和视力下降(31%)。中位随访时间为 5.2 年(范围 1.0-17.2 年)。在随访期间,17 名患者(29%)出现视力下降。在 Kaplan-Meier 生存分析中,47%的视力下降病例发生在诊断后 4 个月内,其余病例在随访期间零星发生(诊断后 8 年)。就危险因素而言,诊断时年龄较小、发病时视神经水肿以及肿瘤复发与视力下降有统计学显著关联。在最终随访时,58%的患者至少一只眼视力受损,但只有 10%的患者双眼视力严重受损(视力 20/200 或更差或视野<20°)。视力丧失是儿童颅咽管瘤的常见表现症状。诊断后,监测视力很重要,因为约 30%的患者会出现明显的视力下降。大多数患者会出现长期视力丧失,但严重的双眼视力障碍并不常见。

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