Coats' Disease and Neovascular Glaucoma in a Child With Neurofibromatosis

Abstract

This report describes an extremely rare case of Coats' disease and neovascular glaucoma in a child with neurofibromatosis 1. Intraocular pressure control and vision restoration were achieved with intravitreal injection with bevacizumab, hemiretinal laser photocoagulation, peripheral retinal cryotherapy, and trabeculectomy with mitomycin C.

Role of intravitreal bevacizumab in adult onset Coats’ disease

“…Cryotherapy is preferred in lesions in the far periphery or with massive exudation [2]. IVTA [4,5] and anti-VEGF agents have been used alone [6,7] or with laser [8][9][10][11][12][13][14] primarily in children with Coats' disease to improve resorption of subretinal fluid and lipid exudates.…”

“…A review of the literature revealed that laser therapy with adjunctive intravitreal injection has been performed at varying time periods ranging from the time of injection [9,14] to 6 weeks following injection [8,10,12,15]. The optimal time period between intravitreal injection and laser treatment is unclear, but we feel that this should be judged clinically on an individual basis by the response to anti-VEGF treatment [11].…”

“…In addition, intravitreal triamcinolone acetonide (IVTA) has been used to reduce macular edema and subretinal exudation in Coats' disease [4,5]. Recently, it has been shown that intravitreal antivascular endothelial growth factor (VEGF) agents namely bevacizumab (Avastin Ò ; Genentech, San Francisco, CA, USA), a humanized monoclonal antibody to all isoforms of VEGF, effectively improved resorption of subretinal fluid and macular exudates when given to children with Coats' disease and may be employed alone [6,7] or with IVTA [8,9] or as adjunctive treatment to laser photocoagulation [8][9][10][11][12][13][14]. Only a single case report describes the use of intravitreal bevacizumab (IVBe) in adult-onset Coats' disease [15].…”

Iris Neovascularization and Neovascular Glaucoma in Neurofibromatosis Type 1

“…Although a variety of posterior segment vascular abnormalities including a variety of tumors have been reported in the literature in patients with NF1, mostly as single case reports, [8][9][10][11][12] the association of these abnormalities with neovascularization of the iris and neovascular glaucoma has only been described in a handful of cases. [3][4][5][6][7] Neovascular glaucoma in children is rare but NF1 needs to be considered among the possible causes in the pediatric age group as pointed out by case 1 in this study and the other cases reported in the literature. [3][4][5][6][7] Neovascular glaucoma in children is rare but NF1 needs to be considered among the possible causes in the pediatric age group as pointed out by case 1 in this study and the other cases reported in the literature.…”

“…It results from a combination of factors that include (1) infiltration of the angle by diffuse neurofibromas, (2) mechanical closure of the angle due to neurofibromatous thickening of the ciliary body, (3) secondary fibrovascular and synechial closure of the angle, (4) congenital angle abnormalities, 1 and (5) endothelialization of the angle attributed to secondary chronic angle closure. [3][4][5][6][7] We report the clinical findings and outcomes in 3 young patients with NF1 who developed iris neovascularization and neovascular glaucoma secondary to retinal vascular abnormalities and describe the histopathologic abnormalities in the enucleated specimen of 1 case. [3][4][5][6][7] We report the clinical findings and outcomes in 3 young patients with NF1 who developed iris neovascularization and neovascular glaucoma secondary to retinal vascular abnormalities and describe the histopathologic abnormalities in the enucleated specimen of 1 case.…”