永存胚胎血管综合征的手术治疗及研究进展

永存胚胎血管综合征(PFVS)是一种临床少见的玻璃体先天发育异常,按累及眼球部位分为前部型、后部型及混合型三种类型。其临床表现多样,早期的手术干预十分重要。手术以解除纤维血管膜对视网膜及晶状体的前后牵引、重建清晰视轴为主要目标。常见的手术方式有瞳孔成形手术、晶状体切除手术伴或不伴人工晶体植入手术、前入路或后入路玻璃体切割手术等。在新技术上,粘弹剂与飞秒激光的应用均有较为理想的结果。除了注重提高手术成功率,还需系统全面地评估患儿的手术前整体情况及手术后视功能。PFVS患儿手术后视力提高有限,这与病变累及范围及并发症的发生有关。伴有视网膜黄斑牵拉脱离和发育不良以及睫状突拉长者,其手术后视力预后不佳。如何从纤维血管蒂与视网膜的显微解剖关系入手,提高累及眼后节PFVS患儿的手术后视力值得进一步研究。另一方面,优化手术步骤、减少手术创伤以降低手术中和手术后并发症也是今后PFVS治疗的重点研究方向。

Surgical treatment and current advances of persistent fetal vasculature syndrome

Persistent fetal vasculature syndrome(PFVS)is a rare congenital vitreous dysplasia,which is classified as anterior,posterior and combined types according to the location of the vascular abnormalities.The clinical manifestations of PFVS are diverse,and early surgical intervention is very important.The main objective of surgical treatment is to remove the anterior and posterior traction between fibrovascular membranes and retina as well as lens,and to reconstruct clear visual axis.Surgical treatments include pupilloplasty,lensectomy with or without intraocular lens implantation and vitrectomy via limbal or scleral approach.For new technologies,the applications of ophthalmic viscosurgical device and femtosecond lasers have desirable results.In addition to focusing on improving the success rate of surgery,it is also necessary to systematically and comprehensively assess the overall preoperative condition and postoperative visual function of the patients.PFVS eyes have limited improvement in postoperative vision,which is related to the extent of lesion involvement and the occurrence of complications.Eyes with macular dysplasia and tractional retinal detachment,as well as elongated ciliary process,have a poor prognosis of vision after surgery.How to improve postoperative vision in the eye affecting the posterior segment of the eye with PFVS from the microscopic anatomical relationship between the fibrous vascular pedicle and the retina is worth further study.On the other hand,reducing surgical trauma and optimizing surgical procedures in order to improve postoperative visual acuity and reduce postoperative complications are also the key research directions of future PFVS treatments.