Treatment Modalities of Ocular Lesions in von Hippel-Lindau Syndrome

Introduction
Von Hippel-Lindau (vH-L) syndrome is a rare autosomal dominant inherited disease, in which mutation of the VHL gene leads to formation of tumors in various different parts of the body, as well as hemangioblastomas in the retina.

Purpose
The aim of the study is to present the various methods of treatment of retinal hemangioblastomas associated with vH-L syndrome and the results of these therapies, their effects on anatomical status as well as the function of the treated eyes.

Material and methods
Twelve patients (18 eyes) with retinal hemangionblastomas were treated in years 1990-2015 in Ophthalmology and Ocular Oncology Clinic in Cracow.The following therapies were applied: laserocoagulation, cryoaplication, pars plana vitrectomy (ppV), transpupillary thermotherapy, photodynamic therapy, brachytherapy and intravitreal injections of anti-VEGF.

Results
Among our patients in 11 cases (16 eyes) multiple treatment modalities were performed, one eye underwent only laserocoagulation and one patient (one eye) underwent only cryoaplication of a single retinal hemangioblastoma. Another patient, after insufficient cryoaplication and TTT, underwent additional 106Ru brachytherapy. Despite of multiple therapies, five patients developed tractional and/or rhegmatogenous retinal detachment and pars plana vitrectomy with silicone oil endotamponade was performed. In one eye after application of intravitreal injections of anti-VEGF factor, the additional therapy, including TTT, cryotherapy, laserocagulation and ppV were performed. In a follow-up period in spite of application of many different and complementary treatments, the progression of ocular lesions and development of serious local complications were observed.

Conclusions
Our results show that treatment efficacy of vH-L hemangiomas is uncertain and depends on localization, number, size of the retinal lesions and dynamics of the disease. The development of posttreatment complications, deteriorate the prognosis of the visual acuity of the treated eye.