Volume 1, Issue 1, Pages 59-67 (January 2017) Laser Photocoagulation for Peripheral Retinal Capillary Hemangioblastoma in von Hippel-Lindau Disease  Valerie Krivosic, MD, Cynthia Kamami-Levy, MD, Julie Jacob, MD, Stephane Richard, PhD, Ramin Tadayoni, MD, PhD, Alain Gaudric, MD  Ophthalmology Retina   Volume 1, Issue 1, Pages 59-67 (January 2017) DOI: 10.1016/j.oret.2016.08.004 Copyright © 2016 American Academy of Ophthalmology Terms and Conditions

Figure 1 Flow chart of the studied population of patients with retinal capillary hemangioblastomas (RCHs) of von Hippel-Lindau disease. Initially, 304 peripheral RCH in 100 eyes of 74 patients were treated with laser photocoagulation in our department. Ophthalmology Retina  2017 1, 59-67DOI: (10.1016/j.oret.2016.08.004) Copyright © 2016 American Academy of Ophthalmology Terms and Conditions

Figure 2 Two mid-size peripheral retinal capillary hemangioblastomas (RCH), <1 disc diameter (DD), before and after laser photocoagulation. A, Each RCH measures about two-thirds of a DD. They are red and fed and drained by 2 dilated arteries and 1 dilated vein. B, Six months after 2 laser sessions on each RCH, they are inactivated and fibrotic, surrounded by an atrophic scar and the afferent and efferent arteries and veins have recovered their normal course and diameter. Ophthalmology Retina  2017 1, 59-67DOI: (10.1016/j.oret.2016.08.004) Copyright © 2016 American Academy of Ophthalmology Terms and Conditions

Figure 3 Large retinal capillary hemangioblastomas (RCH), measuring 1.25 disc diameter, before and after several laser photocoagulation sessions. A, Color photo and fluorescein angiography (FA) before treatment. The RCH is surrounded by subretinal fluid and causes the accumulation of hard exudates in the half-superior part of the macula. Inset, Mid-phase FA showing the pooling of dye in the RCH. B, Three hours after laser photocoagulation, the RCH is entirely white. Inset, Mid-phase FA showing that the RCH is excluded from the retinal circulation. C, Two weeks later, the RCH periphery has become red again. Inset, Mid-phase FA showing the reperfusion of the RCH periphery. D, One year after 3 consecutive laser sessions, the RCH is inactivated, fibrotic, and pigmented. The feeder and draining vessels have recovered their normal diameter, and the hard exudates have disappeared completely. Ophthalmology Retina  2017 1, 59-67DOI: (10.1016/j.oret.2016.08.004) Copyright © 2016 American Academy of Ophthalmology Terms and Conditions

Figure 4 Large retinal capillary hemangioblastoma (RCH) before and after several sessions of laser photocoagulation. A, Color photo and fluorescein angiography (FA) before treatment. Left: Subretinal fluid coming from the RCH detaches the macula in which hard exudates accumulate. Right: The RCH, measuring about 2 disc diameter, is fed by the dilated superotemporal artery and drained by the dilated inferotemporal vein. B, Color photo and FA 3 hours after a first laser session. Left, The RCH is white, surrounded by intraretinal hemorrhages. A prominent exudative retinal detachment is now present around the RCH, and the subretinal fluid has increased in the macula. Right, The RCH core is occluded on FA, but there is a leakage from the RCH periphery with dye pooling in the exudative retinal detachment. C, Color photo and FA 2 hours after retreatment by laser photocoagulation. Left, The photocoagulation covers the whole surface of the RCH. Right, The RCH is completely excluded from the retinal circulation on FA. D, Color photo and FA 3 months after 3 laser sessions. Right, The retina has completely flattened but the hard exudates are not yet resorbed; the RCH is narrowed and is now fibrotic. Left, There remains some filling of the RCH core surrounded by some intraretinal leakage on FA. Additional cryocoagulation was performed. Ophthalmology Retina  2017 1, 59-67DOI: (10.1016/j.oret.2016.08.004) Copyright © 2016 American Academy of Ophthalmology Terms and Conditions

Figure 5 Large retinal capillary hemangioblastoma (RCH) before and after several sessions of laser photocoagulation and cryocoagulation. A, Color photo, optical coherence tomography (OCT) B-scan and fluorescein angiography (FA) before treatment. Top, Two-disc diameter RCH with dilated afferent and efferent retinal vessels and massive intraretinal accumulation of hard exudates, reaching the macula. Bottom, Vertical OCT B-scan showing the intraretinal accumulation of hard exudates and subretinal fluid in the macula. Inset, Intense filling of the RCH by fluorescein. B, At 1 week after 3 consecutive laser sessions, FA shows complete exclusion of the RCH. C, Color photo 1 month after photocoagulation. Subretinal bleeding had occurred. The RCH is partially reperfused and lipid exudation has increased. Inset, Mid-phase of FA showing that the RCH is still active. D, Two years after additional cryocoagulation on the RCH. Color photo shows the atrophic chorioretinal scar around the shrunken and fibrotic RCH; feeder vessels have returned to normal and hard exudates have completely resorbed. Inset, vertical B-scan OCT shows that the macular profile returned to normal. Ophthalmology Retina  2017 1, 59-67DOI: (10.1016/j.oret.2016.08.004) Copyright © 2016 American Academy of Ophthalmology Terms and Conditions

Figure 6 Rate of retinal capillary hemangioblastoma (RCH) inactivation according to size. There were 271 RCHs ≤1 disc diameter (DD) inactivated by laser photocoagulation alone in 100% of cases, after a mean of 1.3 laser sessions. Of the 33 RCH >1 DD, 24 (73%) were inactivated by laser therapy alone after a mean number of 3.5 sessions. Additional cryocoagulation in 7 cases increased the inactivation rate to 94%, of large RCH, and 99% of all the RCH treated. Two cases (6% of large RCH) required additional vitreoretinal surgery. Ophthalmology Retina  2017 1, 59-67DOI: (10.1016/j.oret.2016.08.004) Copyright © 2016 American Academy of Ophthalmology Terms and Conditions