Classification and treatment follow-up of a juxtapapillary retinal hemangioblastoma with optical coherence tomography angiography

Abstract

Purpose Only an endophytic growth pattern in juxtapapillary retinal hemangioblastoma (JRH) is an indication for surgical treatment, but classification of growth types is difficult using conventional imaging techniques. This case report describes the use of optical coherence tomography angiography (OCT-A) features for classification and treatment follow-up in a case with JRH. Observations The JRH of this patient was easily detected with two different OCT-A methods in both en-face and cross-sectional B-scan images, and was classified as a sessile growth type. This growth type excluded the treatment option of vitreoretinal surgery with excision of the lesion or ligation of the feeder vessels. The patient was treated multiple times with intravitreal bevacizumab. Treatment follow-up with OCT-A initially revealed a stable extent of the JRH, with some slight flow deviations in en-face visualization, followed by a period of progressive growth of the lesion. Conclusions OCT-A revealed the depth localization of the JRH and seems to be a valuable tool for JRH classification. Detailed classification may be useful when surgery is considered as a treatment strategy. Furthermore, treatment follow-up is possible with OCT-A, although imaging artifacts should be taken into account.

Comparison of Current Optical Coherence Tomography Angiography Methods in Imaging Retinal Hemangioblastomas

“…The surgical treatment option is mainly considered for juxtapapillary RCHs with an endophytic growth pattern due to the accessibility of these lesions, 23 whereas intravitreal bevacizumab (with very limited effectiveness) or verteporfin photodynamic therapy can be considered for sessile and exophytic tumors. 24 As shown in a case report by Smid et al, 8 OCTA seems to be a valuable tool for juxtapapillary RCH classification. Smid et al 8 reported OCTA as a possible tool for follow-up after therapy with intravitreal bevacizumab.…”

“…24 As shown in a case report by Smid et al, 8 OCTA seems to be a valuable tool for juxtapapillary RCH classification. Smid et al 8 reported OCTA as a possible tool for follow-up after therapy with intravitreal bevacizumab. OCTA can also be useful for follow-up after laser photocoagulation, as described in a case series by Lang et al 7 and in a case report by Chou et al 5 Taken together, OCTA can be a helpful diagnostic tool in the diagnosis and follow-up of RCHs.…”

“…Optical coherence tomography angiography (OCTA) is a new noninvasive technique for imaging the microvasculature of the retina and choroid 1,2 and has rapidly gained clinical acceptance. 2,3 Currently, only few reports exist that have analyzed the retinal and choroidal vasculature of retinal capillary hemangioblastomas (RCHs) via OCTA, [4][5][6][7][8] primarily addressing treatment follow-up [4][5][6][7] and more rare classification of the growth type. 8 Most of these studies were conducted in patients with von Hippel-Lindau disease (VHLD).…”

“…2,3 Currently, only few reports exist that have analyzed the retinal and choroidal vasculature of retinal capillary hemangioblastomas (RCHs) via OCTA, [4][5][6][7][8] primarily addressing treatment follow-up [4][5][6][7] and more rare classification of the growth type. 8 Most of these studies were conducted in patients with von Hippel-Lindau disease (VHLD). 4,[6][7][8] VHLD is an autosomal dominantly inherited familial phacomatosis that predisposes for different tumors and cysts in multiple organs.…”

“…8 Most of these studies were conducted in patients with von Hippel-Lindau disease (VHLD). 4,[6][7][8] VHLD is an autosomal dominantly inherited familial phacomatosis that predisposes for different tumors and cysts in multiple organs. Hemangioblastomas in the central nervous system and RCHs are the most common tumors in patients with VHLD [9][10][11] and are often the onset of VHLD.…”