Тактика ведения и хирургического лечения задней формы iii и iv степени синдрома первичного персистирующего гиперпластического стекловидного тела у детей

Abstract

Persistent hyperplastic primary vitreous syndrome (PHPV) is a rare congenital anomaly associated with impaired regression of the primary embryonic vitreous. There is still no unequivocal opinion on the advisability and terms of surgical treatment of the posterior form of severe III and IV stages of PHPV. Objective. To determine the optimal tactics for the treatment of the posterior form III and IV stages of PHPV syndrome in children. Material and methods. During 2017-2021 we examined and performed surgical intervention in 18 children with PHPV syndrome (18 eyes). The first group – children under 2 years with III stage of PHPV, the second group – children under 1 year with IV stage of PHPV, the third group – children from 3 to 6 years with III and IV stage of PHPV not operated and operated at an early age with late complications of PHPV. All children underwent microinvasive lens extraction or extraction of the complicated cataract, separation of the fibrovascular membrane, removal of the retrolental membrane, if presents, vitrectomy (transciliary 3-port surgery 23-25 G). According to indications, reconstruction of the anterior chamber, intraocular lens implantation, scleral buckling, and antiglaucomatous surgery were carried out. Results. In children with III stage of PHPV during the surgery at an early age in 91.0 % of cases the process is stabilized, the traction component is completely eliminated, the retina is fully attached and visual functions are preserved. In children with IV stage of PHPV the restoration of the anterior chamber, complete elimination of the traction component, decrease in the height and area of retinal detachment and increase in the anterior-posterior size of the eyeball were achieved in all cases. While late surgical treatment of PHPV and secondary complications, it was not possible to achieve a positive result in retinal reattachment. Conclusions. 1. In III and IV stages of PHPV it is necessary to perform surgical treatment up to 1 year with an organ-preserving purpose as the disease may progress with the development of complications, which, if left untreated, can lead to complete loss of the eye as an organ. The terms, volume and stages of surgical treatment depend on the degree and clinical picture of the disease. 2. Children with PHPV syndrome should be constantly under the supervision of an ophthalmologist due to high risk of late complications. Keywords: persistent hyperplastic primary vitreous syndrome, fibrovascular membrane, intraocular lens implantation, vitrectomy.