VITREORETINAL SURGERY FOR PATIENTS WITH SEVERE EXUDATIVE AND PROLIFERATIVE MANIFESTATIONS OF RETINAL CAPILLARY HEMANGIOBLASTOMA BECAUSE OF VON HIPPEL-LINDAU DISEASE

Purpose: The evaluation of long-term results of vitreoretinal surgery and retinal capillary hemangioblastoma (RCH) resection in patients with complicated retinal detachment (RD) secondary to RCHs. Methods: Twelve eyes of 12 patients were operated on with vitreoretinal surgery, including occlusion of afferent and efferent feeder vessels with endodiathermy and endolaser photocoagulation, and subsequent resection of RCHs. The long-term anatomical and visual outcomes were retrospectively evaluated. Results: Total exudative RD was detected in six eyes and subtotal exudative RD was found in the remaining six eyes. A tractional component was present in eight eyes, and four eyes had pure exudative RD. Laser treatment had been previously applied to four eyes but vitreoretinal surgery was the primary treatment in eight eyes. A total of 19 RCHs were resected. The quadrant location of RCHs was superior temporal in six, superior nasal in five, inferior nasal in five, and inferior temporal in three. The dimensions of the RCHs ranged between 1 and 4 disk diameters (DD) (1-DD in 1 eye, 1.5-DD in 3, 2-DD in 10, 2.5-DD in 3, 3-DD in 1, and 4-DD in 1). Preoperative mean logarithm of the minimum angle of resolution visual acuity was 1.9 +/- 1.0 (3.0-0.7) (20/1,588 +/- 20/200 [20/20,000-20/100]). Silicone oil and C3F8 was applied to four and eight eyes, respectively. The median postoperative follow-up was 30.5 (18-48) months. Single operation and final anatomical success was obtained in 9 and 11 eyes, respectively. Mean logarithm of the minimum angle of resolution visual acuity in the postoperative 18th month and at the final visit were 1.05 +/- 0.8 (3.0-0.2) (20/224 +/- 20/125 [20/20,000-20/32]) and 0.96 +/- 0.8 (3.0-0.2) (20/182 +/- 20/125 [20/20,000-20/32]), respectively. New RCHs occurred in eight eyes. Rubeosis iridis was not present in any of the eyes. Conclusion: Promising anatomical and visual outcomes were obtained after vitreoretinal surgery and resection of RCHs in eyes with complicated RD secondary to RCH. However, life-long follow-up is needed to recognize recurrent RD seen even in the early period or recurrences of RCHs seen in the long-term period.