Novel Surgical Treatment of an Intraretinal Juxtapapillary Hemangioblastoma Using Intraocular Diathermy Forceps: A Case Report

Abstract

The surgical treatment of intraretinal juxtapapillary retinal hemangioblastomas (JRHs) was previously contraindicated because of the significant risk of collateral damage to the macula and optic nerve. This case report discusses the effectiveness and safety of a novel surgical technique using intraocular bipolar diathermy forceps to coagulate feeder and draining blood vessels of an intraretinal JRH. The patient suffered from bilateral retinal hemangioblastomas with loss of visual function in one eye and the development of an intraretinal JRH in the other eye. Despite intensive treatment with intravitreal bevacizumab and subconjunctival triamcinolone acetonide, growth of the intraretinal JRH continued, macular exudation worsened, and visual acuity decreased. Surgical treatment was undertaken in which, first, the feeder and draining vessels of the JRH were identified by comparing the retinal imaging of the JRH with the imaging before the emergence of the JRH 4 years earlier. Then, retinal incisions were made above the blood vessels and parallel to the nerve fibers during a pars plana vitrectomy. Lastly, these vessels were lifted above the retinal surface and coagulated using intraocular diathermy forceps. Postoperatively, macular edema reduced, and visual acuity increased and remained stable for about 6 months. Using intraocular diathermy forceps, this case report demonstrates effective and safe intraretinal JRH blood vessel coagulation above the retinal surface. This novel surgical approach was able to delay the deterioration of visual acuity due to tumor growth and exudation in this patient. This suggests that coagulation with intraocular diathermy forceps can be considered an additional surgical treatment option for JRHs, especially those with an intraretinal growth pattern.

Keywords: Case report; Intraocular diathermy forceps; Juxtapapillary retinal hemangioblastoma; Novel surgical treatment; Sessile growth pattern; Von Hippel-Lindau disease.

Fig. 1.

Overview of treatment (upper part) and outcome of treatment (lower part) of the peripheral retinal hemangioblastoma (PRH) and juxtapapillary retinal hemangioblastoma (JRH) between 2016 and 2022. As the outcome of treatment, the course of best corrected visual acuity (black dashed line) and central foveal thickness (black solid line) pre- and postoperatively are presented. The red dotted line indicates the timepoint of application of the novel surgical technique in which intraocular bipolar diathermy of the feeder and draining vessel of the JRH was performed.

Fig. 2.

Video still images illustrating the different steps of the novel surgical technique for feeder vessel (a–f) and draining vessel (g–l) coagulation of a sessile juxtapapillary retinal hemangioblastoma (JRH). Identification of the blood vessels (a, g); retinal incision above, parallel to, and as deep as the blood vessels using a bent 23-gauge stiletto tip (b, h); blood vessels pulled up just above the retinal surface with the bent stiletto tip (c, i); blood vessels visible between the open jaws of the diathermy forceps (d, j); blood vessels compressed and coagulated with closed forceps jaws above the retinal surface (e, k); closed blood vessel with limited visible retinal collateral damage after coagulation using the 23-gauge intraocular diathermy forceps (f, l).

Fig. 3.

Pre- and postoperative fundus photography (upper row), optical coherence tomography (OCT) thickness map (middle row), and OCT cross-section of the juxtapapillary hemangioblastoma (JRH). a 4 years preoperatively: decimal visual acuity (VA) 1.0, early stage juxtapapillary hemangioblastoma (JRH) with cystoid macular edema (CME) requiring regular treatment with intravitreal bevacizumab and subconjunctival triamcinolone acetonide. b 2 months preoperatively: VA was reduced to 0.15 with a larger JRH; some retinal hemorrhages, exudates, and more CME were retreated with bevacizumab and triamcinolone acetonide. c 1 month preoperatively: VA was 0.15 with less CME, more retinal hemorrhages and exudates, after which it was decided to perform the novel surgical technique. Briefly, intraocular bipolar diathermy forceps were used to grab, compress, and coagulate the feeding and draining blood vessels of the JRH during this surgery. d 3 months postoperatively: VA was 0.4 with much fewer retinal hemorrhages, exudates, and a whitish lesion, indicating reduced to no perfusion. e 6 months postoperatively: VA was 0.5 with no CME, retinal hemorrhage, and exudates 4 weeks after restarting bevacizumab and triamcinolone acetonide for CME reappearance. f 12 months postoperatively: VA was 0.4 with a new JRH temporal superior and a larger new or reperfused JRH temporal of the optic disc with increased CME and enlarged blood vessels temporal inferior of the optic disc.