Витрэктомия в сочетании с эндотермотерапией ангиом и питающих сосудов в хирургическом лечении отслойки сетчатки с пролиферативной витреоретинопатией при синдроме Гиппеля-Линдау

Abstract

Actuality . Von Hippel-Lindau disease is a rare autosomal dominant multi-systemic disease, characterized by the presence of vascular formations (angiomas) of different localization. Treatment of severe forms of ophthalmic manifestations of this disease involves the implementation of a pars plana vitrectomy to eliminate retinal detachment, however, the methods of impact on angiomatous nodes during vitrectomy may be different. Endo-thermotherapy (Endo-TT) can be a suitable method for «devitalizing» the nodes during vitrectomy in the treatment of severe forms of von Hippel-Lindau disease. Purpose . To study the efficacy of Endo-TT with angiomas and feeding vessels during vitrectomy in the treatment of retinal detachment complicated by proliferative vitreoretinopathy (PVR) in von Hippel-Lindau desease. Material and methods . Vitrectomy was performed in combination with Endo-TT in 5 eyes (5 patients) with retinal detachment and PVR in Hippel-Lindau desease. Results . A complete retinal attachment was observed in 4 cases in the early and late postoperative period. A relapse of the lower retinal detachment occurred during of a silicone tamponade in one case. Considering the stabilization of intraocular pressure, the calm state of the eye, the lack of prospects for improving vision due to the initially severe condition of the eye, it was decided to refuse a further surgical treatment. The second eye of this patient was preserved sighted, with high visual functions due to timely diagnosis and conduct of transpupillary thermotherapy. Conclusion . Vitrectomy in combination with Endo-TT is an effective method for treatment of retinal detachment and PVR in von Hippel-Lindau desease in severe forms. Early detection and treatment of mild forms of the disease is necessary to maintain high visual functions and a high quality of life.