目的: 分析甲醇中毒性视神经病变的临床特征、影像学动态演变及治疗反应,探讨中毒程度、治疗时机与视功能预后的相关性,为优化临床诊疗路径提供循证依据。方法: 采用病例系列研究与文献回顾方法,回顾性纳入2017—2024年在中山大学中山眼科中心确诊的9例(18只眼)甲醇中毒性视神经病变患者,收集其临床资料包括瞳孔变化、眼底照相、光学相干断层成像(optical coherence tomography; OCT)、视野、视觉诱发电位(visual evoked potential; VEP)等,结合文献整合病例数据。纳入标准包括明确甲醇暴露史、急性视力下降伴视盘水肿或视网膜神经纤维层(retinal nerve fiber layer; RNFL)增厚、血/尿甲醇或甲酸浓度升高。排除既往视力不佳或视网膜病变者。通过多模态影像学评估视神经损伤特征,分析治疗反应及预后相关因素。结果: 9例(18只眼)患者年龄为23~51岁(男性7例,女性2例),中毒后就诊时间为3 d~3个月。最佳矫正视力为无光感(11眼)至1.0,66.7%(12/18眼)出现瞳孔变化,眼底表现为视盘水肿,VEP提示振幅下降伴视野缺损,OCT显示急性期视盘区域RNFL增厚,慢性期进行性萎缩、杯盘比显著增大。经治疗后,12只眼视力提高(随访2个月,4只眼中毒后视力为1.0,未纳入其中),持续随访(4个月~3年)的患者中,多数患者急性期干预后进入视力平台期,但少数患者仍存在迟发性神经退行风险。因此,部分患者通过早期干预联合全身激素冲击、血液透析、营养神经等治疗可能有助于改善视功能,而部分延迟治疗患者可出现不可逆视力丧失。瞳孔对光反射保留者预后更佳。结论: 甲醇中毒性视神经病变的预后与中毒程度、干预时机及瞳孔反射状态密切相关。早期给予激素冲击联合营养神经治疗可能改善部分患者的预后,但中毒时间长者易遗留不可逆视神经损伤,个性化分期治疗及长期视功能监测对保护残余视力至关重要。
Objective: To analyze the clinical characteristics, dynamic imaging changes, and treatment responses of methanol-induced toxic neuropathy. Additionally, it sought to explore the correlation between poisoning severity, intervention timing, and visual functional outcomes, so as to evidence-based insights for optimizing clinical diagnosis and treatment strategies. Methods: A case-series study combined with a literature review was conducted. Nine patients (18 eyes) diagnosed with methanol-induced toxic neuropathy at Zhongshan Ophthalmic Center, Sun Yat-sen University from 2017 to 2024, were retrospectively included. Clinical data, including pupillary changes, fundus photography, optical coherence tomography (OCT), visual field tests, and visual evoked potentials (VEP), were collected and integrated with findings from the literature. The inclusion criteria were a confirmed history of methanol exposure, acute vision loss accompanied by optic disc edema or retinal nerve fiber layer (RNFL) thickening, and elevated blood/urine methanol or formic acid levels. Patients with pre-existing poor vision or retinal diseases were excluded. Multimodal imaging was employed to evaluate the characteristics of optic nerve injury, and treatment responses and prognostic factors were analyzed. Results: The nine patients (18 eyes) ranged in age from 23–51 years (7 males, 2 females), with the time from poisoning to consultation spanning from 3 days to 3 months. The best-corrected visual acuity (BCVA) ranged from no light perception (NLP, 11 eyes) to 1.0. Pupillary abnormalities were observed in 66.7% (12/18 eyes) of the cases. Fundus examination showed optic disc edema, while VEP revealed reduced amplitude and visual field defects. OCT demonstrated RNFL thickening in the acute phase and progressive atrophy with an increased cup-to-disc ratio (C/D) in the chronic phase. After treatment, 12 eyes showed visual improvement (followed at 2 months; 4 eyes with post-poisoning BCVA of 1.0 were excluded from this analysis). Among patients with extended follow-up (4 months to 3 years), most reached a visual acuity plateau after acute-phase intervention, although a minority remained at risk of delayed neurodegenerative decline. Early intervention with systemic high-dose steroid therapy, hemodialysis, and neuroprotective treatment might improve visual function in some patients, whereas delayed treatment often led to irreversible vision loss. Patients with preserved pupillary light reflexes had more favorable prognoses. Conclusions: The prognosis of methanol-induced optic neuropathy is closely related to poisoning severity, intervention timing, and pupillary reflex status. Early high-dose steroid therapy combined with neuroprotective treatment may enhance outcomes in some patients; however, prolonged poisoning typically results in irreversible neurovisual damage. Personalized staged treatment and long-term visual function monitoring are essential for preserving residual vision.
全眼球移植(Whole eye transplantation, WET)是治疗因外伤、肿瘤等导致眼球不可逆损伤及永久性失明的潜在终极手段。其核心不仅在于实现眼球解剖结构的完整移植,更关键的是实现功能性视觉恢复,关键挑战包括优化手术策略重建眼球血液循环,实现供体视神经与受体视觉中枢的神经连接,建立精准有效的免疫调控策略以平衡移植耐受与感染风险。本文系统梳理了WET领域的研究进展与发展挑战。早期研究以变温动物模型为主,这些模型在一定程度上证实了视觉通路在眼球移植后仍具有一定的可塑性,但由于其眼球结构与人类存在显著解剖差异,且实验设计中未涉及精细的血管与神经吻合,因而临床转化价值有限。随着显微血管技术的发展,哺乳动物模型及人眼球移植的初步探索在显微外科血管吻合技术方面取得重要突破,实现了移植眼球视网膜血液灌注的短期重建,但仍面临视神经再生障碍和功能维持难题。当前的研究瓶颈主要集中在三个方面:血液循环重建技术优化、神经轴突精准再生及免疫微环境调控。建立与人类接近的标准化非人灵长类动物模型,并开展系统性的实验研究,将为克服现有技术障碍、推动WET走向临床提供至关重要的实验依据和新思路。
Whole eye transplantation (WET) is a potential definitive treatment for irreversible ocular damage and permanent blindness caused by conditions like trauma or tumors. The success of WET depends not only on the complete anatomical restoration of the transplanted eye but, more critically, on the recovery of functional vision. Key challenges in this field include optimizing surgical strategies to re-establish ocular blood circulation, achieving functional neural integration between the donor optic nerve and the recipient's visual centers, and developing precise and effective immunomodulation strategies to strike a balance transplant tolerance and infection risk. This review provides a systematic review of the research progress and developmental challenges in the field of WET. Early studies primarily utilized poikilothermic animal models, which demonstrated a certain degree of plasticity in the visual pathway following transplantation. However, due to significant anatomical differences compared to humans and the lack of sophisticated vascular and neural anastomosis in experimental designs, their clinical translatability of these studies remained constrained. With the progress in microvascular techniques, preliminary explorations in mammalian models and human eye transplantation have achieved significant breakthroughs in microsurgical vascular anastomosis, enabling the short-term reestablishment of retinal blood perfusion in transplanted eyes. Nonetheless, perisitent obstacles include inadequate optic nerve regeneration and poor long-term functional maintenance. Currently, research bottlenecks mainly focus on three aspects: optimizing surgical techniques for blood circulation reconstruction, precisely regenerating nerve axons, and regulating the immune microenvironment. Establishing standardized non-human primate models that closely mimic humans and conducting systematic experimental studies will furnish crucial evidence and novel insights to overcome existing technical hurdles and propel WET toward clinical application.