PDF(Pubmed)
Abstract：
Primary congenital glaucoma (PCG), a developmental glaucoma occurring due to angle anomaly, earns growing concerns among ophthalmologists for its vision-damaging attribute. The incidence of PCG varies among races and geographic regions and is mostly genetically associated. Theories have been posed in attempt to address the etiology of this congenital maldevelopment and in the meanwhile providing evidence for feasibility of PCG surgeries. In regard to the clinical aspects of this entity, both the clinical characteristics and general principals of management are introduced, with angle surgeries highlighted for clarifying details including their success rates, key points for a successful surgical intervention, postoperative management, and follow-up strategies. Taking patients\' vision-associated quality of life into consideration, we stressed that further perceptual learning and low vision rehabilitation are momentous. However, much has yet to be elucidated in respect of the truly comprehensive pathogenesis underneath as well as means by which clinical outcomes of PCG can be further improved. We are now looking forward to innovative therapeutic approaches like gene therapy in specific genes in the future, with the hope of improving their life-long visual quality in those young patients.
摘要：
原发性先天性青光眼（PCG），由于角度异常而发生的发育性青光眼，眼科医生越来越担心它的视力损害属性。PCG的发病率因种族和地理区域而异，并且大多与遗传相关。已经提出了一些理论来试图解决这种先天性发育不良的病因，同时为PCG手术的可行性提供了证据。关于这个实体的临床方面，介绍了临床特点和管理的一般原则，突出显示角度手术以澄清细节，包括成功率，成功手术干预的关键点，术后管理，以及后续策略。考虑到患者与视力相关的生活质量，我们强调，进一步的感性学习和低视力康复至关重要。然而,关于真正全面的发病机制以及可以进一步改善PCG临床结局的方法，还有很多有待阐明。我们现在期待着创新的治疗方法，比如未来特定基因的基因治疗，希望改善这些年轻患者的终身视觉质量。
