PDF(Pubmed)
Abstract：
Primary congenital glaucoma (PCG) occurs worldwide and has a broad range of ocular manifestations. It poses a therapeutic challenge to the ophthalmologist. A proper diagnostic evaluation under anesthesia is advisable for all children who do not cooperate for an office examination. Medical therapy only serves as a supportive role, and surgical intervention remains the principal therapeutic modality. Angle incision surgery such as goniotomy or trabeculotomy ab externo is the preferred choice of surgery in the Caucasian population. Primary combined trabeculotomy-trabeculectomy with or without antifibrotic therapy is the preferred choice in certain regions such as India and the Middle East where the disease usually presents with severe forms of corneal edema along with megalocornea. In refractory cases, trabeculectomy with antifibrotic therapy or glaucoma drainage devices are available options in the armamentarium. Cycloablative procedures should be reserved for eyes with poor visual potential. Myopia is common among children with PCG, and appropriate optical refractive correction in the form of glasses or contact lenses should be provided. Amblyopia therapy should be instituted to ensure overall visual development in the early developmental years. Low-vision rehabilitation services should be provided to children with vision impairment. Long-term follow-up is mandatory and carers of children with PCG should be counseled and educated about this need. Regardless of the visual outcomes, clinicians should emphasize the need for education of these children during the clinic visit. The overall goal of the management should be to improve the overall quality of life of the children with PCG and their carers.
摘要：
原发性先天性青光眼（PCG）在世界范围内发生，并具有广泛的眼部表现。它对眼科医生提出了治疗挑战。对于所有不配合办公室检查的儿童，建议在麻醉下进行适当的诊断评估。药物治疗只能起到辅助作用,手术干预仍然是主要的治疗方式。在高加索人群中，角切开术或小梁切开术等角度切口手术是首选手术。在某些地区，例如印度和中东，有或没有抗纤维化治疗的原发性联合小梁切开术-小梁切除术是首选，该疾病通常伴有严重的角膜水肿和巨角膜。在难治性病例中,具有抗纤维化治疗或青光眼引流装置的小梁切除术是兵工厂的可用选项。对于视觉潜能差的眼睛，应保留旋光手术。近视在PCG儿童中很常见，应提供适当的眼镜或隐形眼镜形式的光学屈光矫正。应建立弱视治疗，以确保早期发育的整体视觉发育。应向视力障碍儿童提供低视力康复服务。长期随访是强制性的，应就这一需求向PCG儿童的看护者提供咨询和教育。不管视觉结果如何，临床医生应强调在就诊期间对这些儿童进行教育的必要性.管理的总体目标应该是提高PCG儿童及其照顾者的整体生活质量。
